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Tetralogy of Fallot - Wikipedia
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id="toc-Signs_and_symptoms-sublist" class="vector-toc-list"> <li id="toc-Tet_spells" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Tet_spells"> <div class="vector-toc-text"> <span class="vector-toc-numb">1.1</span> <span>Tet spells</span> </div> </a> <ul id="toc-Tet_spells-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Cause" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#Cause"> <div class="vector-toc-text"> <span class="vector-toc-numb">2</span> <span>Cause</span> </div> </a> <ul id="toc-Cause-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Pathophysiology" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#Pathophysiology"> <div class="vector-toc-text"> <span class="vector-toc-numb">3</span> <span>Pathophysiology</span> </div> </a> <button aria-controls="toc-Pathophysiology-sublist" class="cdx-button cdx-button--weight-quiet cdx-button--icon-only vector-toc-toggle"> <span class="vector-icon mw-ui-icon-wikimedia-expand"></span> <span>Toggle Pathophysiology subsection</span> </button> <ul id="toc-Pathophysiology-sublist" class="vector-toc-list"> <li id="toc-Four_malformations" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Four_malformations"> <div class="vector-toc-text"> <span class="vector-toc-numb">3.1</span> <span>Four malformations</span> </div> </a> <ul id="toc-Four_malformations-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Additional_anomalies" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Additional_anomalies"> <div class="vector-toc-text"> <span class="vector-toc-numb">3.2</span> <span>Additional anomalies</span> </div> </a> <ul id="toc-Additional_anomalies-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Diagnosis" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#Diagnosis"> <div class="vector-toc-text"> <span class="vector-toc-numb">4</span> <span>Diagnosis</span> </div> </a> <button aria-controls="toc-Diagnosis-sublist" class="cdx-button cdx-button--weight-quiet cdx-button--icon-only vector-toc-toggle"> <span class="vector-icon mw-ui-icon-wikimedia-expand"></span> <span>Toggle Diagnosis subsection</span> </button> <ul id="toc-Diagnosis-sublist" class="vector-toc-list"> <li id="toc-Chest_radiograph" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Chest_radiograph"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.1</span> <span>Chest radiograph</span> </div> </a> <ul id="toc-Chest_radiograph-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Electrocardiogram" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Electrocardiogram"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.2</span> <span>Electrocardiogram</span> </div> </a> <ul id="toc-Electrocardiogram-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Echocardiogram" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Echocardiogram"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.3</span> <span>Echocardiogram</span> </div> </a> <ul id="toc-Echocardiogram-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Genetics" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Genetics"> <div class="vector-toc-text"> <span class="vector-toc-numb">4.4</span> <span>Genetics</span> </div> </a> <ul id="toc-Genetics-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Treatment" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#Treatment"> <div class="vector-toc-text"> <span class="vector-toc-numb">5</span> <span>Treatment</span> </div> </a> <button aria-controls="toc-Treatment-sublist" class="cdx-button cdx-button--weight-quiet cdx-button--icon-only vector-toc-toggle"> <span class="vector-icon mw-ui-icon-wikimedia-expand"></span> <span>Toggle Treatment subsection</span> </button> <ul id="toc-Treatment-sublist" class="vector-toc-list"> <li id="toc-Tet_spells_2" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Tet_spells_2"> <div class="vector-toc-text"> <span class="vector-toc-numb">5.1</span> <span>Tet spells</span> </div> </a> <ul id="toc-Tet_spells_2-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Total_surgical_repair" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Total_surgical_repair"> <div class="vector-toc-text"> <span class="vector-toc-numb">5.2</span> <span>Total surgical repair</span> </div> </a> <ul id="toc-Total_surgical_repair-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Palliative_surgery" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Palliative_surgery"> <div class="vector-toc-text"> <span class="vector-toc-numb">5.3</span> <span>Palliative surgery</span> </div> </a> <ul id="toc-Palliative_surgery-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Approaches_to_surgical_repair" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Approaches_to_surgical_repair"> <div class="vector-toc-text"> <span class="vector-toc-numb">5.4</span> <span>Approaches to surgical repair</span> </div> </a> <ul id="toc-Approaches_to_surgical_repair-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Complications" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#Complications"> <div class="vector-toc-text"> <span class="vector-toc-numb">6</span> <span>Complications</span> </div> </a> <button aria-controls="toc-Complications-sublist" class="cdx-button cdx-button--weight-quiet cdx-button--icon-only vector-toc-toggle"> <span class="vector-icon mw-ui-icon-wikimedia-expand"></span> <span>Toggle Complications subsection</span> </button> <ul id="toc-Complications-sublist" class="vector-toc-list"> <li id="toc-Short-term" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Short-term"> <div class="vector-toc-text"> <span class="vector-toc-numb">6.1</span> <span>Short-term</span> </div> </a> <ul id="toc-Short-term-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Long-term" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Long-term"> <div class="vector-toc-text"> <span class="vector-toc-numb">6.2</span> <span>Long-term</span> </div> </a> <ul id="toc-Long-term-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Pregnancy" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Pregnancy"> <div class="vector-toc-text"> <span class="vector-toc-numb">6.3</span> <span>Pregnancy</span> </div> </a> <ul id="toc-Pregnancy-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Prognosis" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#Prognosis"> <div class="vector-toc-text"> <span class="vector-toc-numb">7</span> <span>Prognosis</span> </div> </a> <ul id="toc-Prognosis-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Comorbidities" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#Comorbidities"> <div class="vector-toc-text"> <span class="vector-toc-numb">8</span> <span>Comorbidities</span> </div> </a> <ul id="toc-Comorbidities-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Epidemiology" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#Epidemiology"> <div class="vector-toc-text"> <span class="vector-toc-numb">9</span> <span>Epidemiology</span> </div> </a> <ul id="toc-Epidemiology-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-History" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#History"> <div class="vector-toc-text"> <span class="vector-toc-numb">10</span> <span>History</span> </div> </a> <ul id="toc-History-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Related_disorders" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#Related_disorders"> <div class="vector-toc-text"> <span class="vector-toc-numb">11</span> <span>Related disorders</span> </div> </a> <button aria-controls="toc-Related_disorders-sublist" class="cdx-button cdx-button--weight-quiet cdx-button--icon-only vector-toc-toggle"> <span class="vector-icon mw-ui-icon-wikimedia-expand"></span> <span>Toggle Related disorders subsection</span> </button> <ul id="toc-Related_disorders-sublist" class="vector-toc-list"> <li id="toc-Atrial_septal_defects" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Atrial_septal_defects"> <div class="vector-toc-text"> <span class="vector-toc-numb">11.1</span> <span>Atrial septal defects</span> </div> </a> <ul id="toc-Atrial_septal_defects-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Ventricular_septal_defects" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Ventricular_septal_defects"> <div class="vector-toc-text"> <span class="vector-toc-numb">11.2</span> <span>Ventricular septal defects</span> </div> </a> <ul id="toc-Ventricular_septal_defects-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Atrioventricular_septal_defect" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Atrioventricular_septal_defect"> <div class="vector-toc-text"> <span class="vector-toc-numb">11.3</span> <span>Atrioventricular septal defect</span> </div> </a> <ul id="toc-Atrioventricular_septal_defect-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Mitral_valve_stenosis" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Mitral_valve_stenosis"> <div class="vector-toc-text"> <span class="vector-toc-numb">11.4</span> <span>Mitral valve stenosis</span> </div> </a> <ul id="toc-Mitral_valve_stenosis-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Notable_cases" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#Notable_cases"> <div class="vector-toc-text"> <span class="vector-toc-numb">12</span> <span>Notable cases</span> </div> </a> <ul id="toc-Notable_cases-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-See_also" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#See_also"> <div class="vector-toc-text"> <span class="vector-toc-numb">13</span> <span>See also</span> </div> </a> <ul id="toc-See_also-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-References" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#References"> <div class="vector-toc-text"> <span class="vector-toc-numb">14</span> <span>References</span> </div> </a> <ul id="toc-References-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-External_links" class="vector-toc-list-item vector-toc-level-1"> <a class="vector-toc-link" href="#External_links"> <div class="vector-toc-text"> <span class="vector-toc-numb">15</span> <span>External links</span> </div> </a> <ul id="toc-External_links-sublist" class="vector-toc-list"> </ul> </li> </ul> </div> </div> </nav> </div> </div> <div class="mw-content-container"> <main id="content" class="mw-body"> <header class="mw-body-header vector-page-titlebar"> <nav aria-label="Contents" class="vector-toc-landmark"> <div id="vector-page-titlebar-toc" class="vector-dropdown vector-page-titlebar-toc vector-button-flush-left" > <input type="checkbox" id="vector-page-titlebar-toc-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-vector-page-titlebar-toc" class="vector-dropdown-checkbox " aria-label="Toggle the table of contents" > <label id="vector-page-titlebar-toc-label" for="vector-page-titlebar-toc-checkbox" class="vector-dropdown-label cdx-button cdx-button--fake-button cdx-button--fake-button--enabled cdx-button--weight-quiet cdx-button--icon-only " aria-hidden="true" ><span class="vector-icon mw-ui-icon-listBullet mw-ui-icon-wikimedia-listBullet"></span> <span class="vector-dropdown-label-text">Toggle the table of contents</span> </label> <div class="vector-dropdown-content"> <div id="vector-page-titlebar-toc-unpinned-container" class="vector-unpinned-container"> </div> </div> </div> </nav> <h1 id="firstHeading" class="firstHeading mw-first-heading"><span class="mw-page-title-main">Tetralogy of Fallot</span></h1> <div id="p-lang-btn" class="vector-dropdown mw-portlet mw-portlet-lang" > <input type="checkbox" id="p-lang-btn-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-p-lang-btn" class="vector-dropdown-checkbox 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Available in 34 languages" > <label id="p-lang-btn-label" for="p-lang-btn-checkbox" class="vector-dropdown-label cdx-button cdx-button--fake-button cdx-button--fake-button--enabled cdx-button--weight-quiet cdx-button--action-progressive mw-portlet-lang-heading-34" aria-hidden="true" ><span class="vector-icon mw-ui-icon-language-progressive mw-ui-icon-wikimedia-language-progressive"></span> <span class="vector-dropdown-label-text">34 languages</span> </label> <div class="vector-dropdown-content"> <div class="vector-menu-content"> <ul class="vector-menu-content-list"> <li class="interlanguage-link interwiki-ar mw-list-item"><a href="https://ar.wikipedia.org/wiki/%D8%B1%D8%A8%D8%A7%D8%B9%D9%8A%D8%A9_%D9%81%D8%A7%D9%84%D9%88" title="رباعية فالو – Arabic" lang="ar" hreflang="ar" data-title="رباعية فالو" data-language-autonym="العربية" data-language-local-name="Arabic" class="interlanguage-link-target"><span>العربية</span></a></li><li class="interlanguage-link interwiki-az mw-list-item"><a href="https://az.wikipedia.org/wiki/Fallo_tetradas%C4%B1" title="Fallo tetradası – Azerbaijani" lang="az" hreflang="az" data-title="Fallo tetradası" data-language-autonym="Azərbaycanca" data-language-local-name="Azerbaijani" class="interlanguage-link-target"><span>Azərbaycanca</span></a></li><li class="interlanguage-link interwiki-bs mw-list-item"><a href="https://bs.wikipedia.org/wiki/Fallotova_tetralogija" title="Fallotova tetralogija – Bosnian" lang="bs" hreflang="bs" data-title="Fallotova tetralogija" data-language-autonym="Bosanski" data-language-local-name="Bosnian" class="interlanguage-link-target"><span>Bosanski</span></a></li><li class="interlanguage-link interwiki-ca mw-list-item"><a href="https://ca.wikipedia.org/wiki/Tetralogia_de_Fallot" title="Tetralogia de Fallot – Catalan" lang="ca" hreflang="ca" data-title="Tetralogia de Fallot" data-language-autonym="Català" data-language-local-name="Catalan" class="interlanguage-link-target"><span>Català</span></a></li><li class="interlanguage-link interwiki-cs mw-list-item"><a href="https://cs.wikipedia.org/wiki/Fallotova_tetralogie" title="Fallotova tetralogie – Czech" lang="cs" hreflang="cs" data-title="Fallotova tetralogie" data-language-autonym="Čeština" data-language-local-name="Czech" class="interlanguage-link-target"><span>Čeština</span></a></li><li class="interlanguage-link interwiki-de mw-list-item"><a href="https://de.wikipedia.org/wiki/Fallot-Tetralogie" title="Fallot-Tetralogie – German" lang="de" hreflang="de" data-title="Fallot-Tetralogie" data-language-autonym="Deutsch" data-language-local-name="German" class="interlanguage-link-target"><span>Deutsch</span></a></li><li class="interlanguage-link interwiki-es mw-list-item"><a href="https://es.wikipedia.org/wiki/Tetralog%C3%ADa_de_Fallot" title="Tetralogía de Fallot – Spanish" lang="es" hreflang="es" data-title="Tetralogía de Fallot" data-language-autonym="Español" data-language-local-name="Spanish" class="interlanguage-link-target"><span>Español</span></a></li><li class="interlanguage-link interwiki-fa mw-list-item"><a href="https://fa.wikipedia.org/wiki/%D8%AA%D8%AA%D8%B1%D8%A7%D9%84%D9%88%DA%98%DB%8C_%D9%81%D8%A7%D9%84%D9%88%D8%AA" title="تترالوژی فالوت – Persian" lang="fa" hreflang="fa" data-title="تترالوژی فالوت" data-language-autonym="فارسی" data-language-local-name="Persian" class="interlanguage-link-target"><span>فارسی</span></a></li><li class="interlanguage-link interwiki-fr mw-list-item"><a href="https://fr.wikipedia.org/wiki/T%C3%A9tralogie_de_Fallot" title="Tétralogie de Fallot – French" lang="fr" hreflang="fr" data-title="Tétralogie de Fallot" data-language-autonym="Français" data-language-local-name="French" class="interlanguage-link-target"><span>Français</span></a></li><li class="interlanguage-link interwiki-ko mw-list-item"><a href="https://ko.wikipedia.org/wiki/%ED%8C%94%EB%A1%9C%EC%82%AC%EC%A7%95%EC%A6%9D" title="팔로사징증 – Korean" lang="ko" hreflang="ko" data-title="팔로사징증" data-language-autonym="한국어" data-language-local-name="Korean" class="interlanguage-link-target"><span>한국어</span></a></li><li class="interlanguage-link interwiki-ha mw-list-item"><a href="https://ha.wikipedia.org/wiki/Tetralogy_na_Falot" title="Tetralogy na Falot – Hausa" lang="ha" hreflang="ha" data-title="Tetralogy na Falot" data-language-autonym="Hausa" data-language-local-name="Hausa" class="interlanguage-link-target"><span>Hausa</span></a></li><li class="interlanguage-link interwiki-hy mw-list-item"><a href="https://hy.wikipedia.org/wiki/%D5%96%D5%A1%D5%AC%D5%AC%D5%B8%D5%B5%D5%AB_%D5%BF%D5%A5%D5%BF%D6%80%D5%A1%D5%A4%D5%A1" title="Ֆալլոյի տետրադա – Armenian" lang="hy" hreflang="hy" data-title="Ֆալլոյի տետրադա" data-language-autonym="Հայերեն" data-language-local-name="Armenian" class="interlanguage-link-target"><span>Հայերեն</span></a></li><li class="interlanguage-link interwiki-id mw-list-item"><a href="https://id.wikipedia.org/wiki/Tetralogi_Fallot" title="Tetralogi Fallot – Indonesian" lang="id" hreflang="id" data-title="Tetralogi Fallot" data-language-autonym="Bahasa Indonesia" data-language-local-name="Indonesian" class="interlanguage-link-target"><span>Bahasa Indonesia</span></a></li><li class="interlanguage-link interwiki-it mw-list-item"><a href="https://it.wikipedia.org/wiki/Tetralogia_di_Fallot" title="Tetralogia di Fallot – Italian" lang="it" hreflang="it" data-title="Tetralogia di Fallot" data-language-autonym="Italiano" data-language-local-name="Italian" class="interlanguage-link-target"><span>Italiano</span></a></li><li class="interlanguage-link interwiki-he mw-list-item"><a href="https://he.wikipedia.org/wiki/%D7%98%D7%98%D7%A8%D7%9C%D7%95%D7%92%D7%99%D7%94_%D7%A2%D7%9C_%D7%A9%D7%9D_%D7%A4%D7%90%D7%9C%D7%95" title="טטרלוגיה על שם פאלו – Hebrew" lang="he" hreflang="he" data-title="טטרלוגיה על שם פאלו" data-language-autonym="עברית" data-language-local-name="Hebrew" class="interlanguage-link-target"><span>עברית</span></a></li><li class="interlanguage-link interwiki-mk mw-list-item"><a href="https://mk.wikipedia.org/wiki/%D0%A2%D0%B5%D1%82%D1%80%D0%B0%D0%BB%D0%BE%D0%B3%D0%B8%D1%98%D0%B0_%D0%BD%D0%B0_%D0%A4%D0%B0%D0%BB%D0%BE" title="Тетралогија на Фало – Macedonian" lang="mk" hreflang="mk" data-title="Тетралогија на Фало" data-language-autonym="Македонски" data-language-local-name="Macedonian" class="interlanguage-link-target"><span>Македонски</span></a></li><li class="interlanguage-link interwiki-ms mw-list-item"><a href="https://ms.wikipedia.org/wiki/Tetralogi_Fallot" title="Tetralogi Fallot – Malay" lang="ms" hreflang="ms" data-title="Tetralogi Fallot" data-language-autonym="Bahasa Melayu" data-language-local-name="Malay" class="interlanguage-link-target"><span>Bahasa Melayu</span></a></li><li class="interlanguage-link interwiki-nl mw-list-item"><a href="https://nl.wikipedia.org/wiki/Tetralogie_van_Fallot" title="Tetralogie van Fallot – Dutch" lang="nl" hreflang="nl" data-title="Tetralogie van Fallot" data-language-autonym="Nederlands" data-language-local-name="Dutch" class="interlanguage-link-target"><span>Nederlands</span></a></li><li class="interlanguage-link interwiki-ja mw-list-item"><a href="https://ja.wikipedia.org/wiki/%E3%83%95%E3%82%A1%E3%83%AD%E3%83%BC%E5%9B%9B%E5%BE%B4%E7%97%87" title="ファロー四徴症 – Japanese" lang="ja" hreflang="ja" data-title="ファロー四徴症" data-language-autonym="日本語" data-language-local-name="Japanese" class="interlanguage-link-target"><span>日本語</span></a></li><li class="interlanguage-link interwiki-nn mw-list-item"><a href="https://nn.wikipedia.org/wiki/Fallots_tetrade" title="Fallots tetrade – Norwegian Nynorsk" lang="nn" hreflang="nn" data-title="Fallots tetrade" data-language-autonym="Norsk nynorsk" data-language-local-name="Norwegian Nynorsk" class="interlanguage-link-target"><span>Norsk nynorsk</span></a></li><li class="interlanguage-link interwiki-or mw-list-item"><a href="https://or.wikipedia.org/wiki/%E0%AC%9F%E0%AD%87%E0%AC%9F%E0%AD%8D%E0%AC%B0%E0%AC%BE%E0%AC%B2%E0%AD%8B%E0%AC%9C%E0%AC%BF_%E0%AC%85%E0%AC%AB_%E0%AC%AB%E0%AD%8D%E0%AD%9F%E0%AC%BE%E0%AC%B2%E0%AC%9F" title="ଟେଟ୍ରାଲୋଜି ଅଫ ଫ୍ୟାଲଟ – Odia" lang="or" hreflang="or" data-title="ଟେଟ୍ରାଲୋଜି ଅଫ ଫ୍ୟାଲଟ" data-language-autonym="ଓଡ଼ିଆ" data-language-local-name="Odia" class="interlanguage-link-target"><span>ଓଡ଼ିଆ</span></a></li><li class="interlanguage-link interwiki-pl mw-list-item"><a href="https://pl.wikipedia.org/wiki/Tetralogia_Fallota" title="Tetralogia Fallota – Polish" lang="pl" hreflang="pl" data-title="Tetralogia Fallota" data-language-autonym="Polski" data-language-local-name="Polish" class="interlanguage-link-target"><span>Polski</span></a></li><li class="interlanguage-link interwiki-pt mw-list-item"><a href="https://pt.wikipedia.org/wiki/Tetralogia_de_Fallot" title="Tetralogia de Fallot – Portuguese" lang="pt" hreflang="pt" data-title="Tetralogia de Fallot" data-language-autonym="Português" data-language-local-name="Portuguese" class="interlanguage-link-target"><span>Português</span></a></li><li class="interlanguage-link interwiki-ro mw-list-item"><a href="https://ro.wikipedia.org/wiki/Tetralogia_Fallot" title="Tetralogia Fallot – Romanian" lang="ro" hreflang="ro" data-title="Tetralogia Fallot" data-language-autonym="Română" data-language-local-name="Romanian" class="interlanguage-link-target"><span>Română</span></a></li><li class="interlanguage-link interwiki-ru mw-list-item"><a href="https://ru.wikipedia.org/wiki/%D0%A2%D0%B5%D1%82%D1%80%D0%B0%D0%B4%D0%B0_%D0%A4%D0%B0%D0%BB%D0%BB%D0%BE" title="Тетрада Фалло – Russian" lang="ru" hreflang="ru" data-title="Тетрада Фалло" data-language-autonym="Русский" data-language-local-name="Russian" class="interlanguage-link-target"><span>Русский</span></a></li><li class="interlanguage-link interwiki-sk mw-list-item"><a href="https://sk.wikipedia.org/wiki/Fallotova_tetral%C3%B3gia" title="Fallotova tetralógia – Slovak" lang="sk" hreflang="sk" data-title="Fallotova tetralógia" data-language-autonym="Slovenčina" data-language-local-name="Slovak" class="interlanguage-link-target"><span>Slovenčina</span></a></li><li class="interlanguage-link interwiki-sr mw-list-item"><a href="https://sr.wikipedia.org/wiki/Tetralogija_Fallot" title="Tetralogija Fallot – Serbian" lang="sr" hreflang="sr" data-title="Tetralogija Fallot" data-language-autonym="Српски / srpski" data-language-local-name="Serbian" class="interlanguage-link-target"><span>Српски / srpski</span></a></li><li class="interlanguage-link interwiki-fi mw-list-item"><a href="https://fi.wikipedia.org/wiki/Fallot%E2%80%99n_tetralogia" title="Fallot’n tetralogia – Finnish" lang="fi" hreflang="fi" data-title="Fallot’n tetralogia" data-language-autonym="Suomi" data-language-local-name="Finnish" class="interlanguage-link-target"><span>Suomi</span></a></li><li class="interlanguage-link interwiki-sv mw-list-item"><a href="https://sv.wikipedia.org/wiki/Fallots_tetrad" title="Fallots tetrad – Swedish" lang="sv" hreflang="sv" data-title="Fallots tetrad" data-language-autonym="Svenska" data-language-local-name="Swedish" class="interlanguage-link-target"><span>Svenska</span></a></li><li class="interlanguage-link interwiki-th mw-list-item"><a href="https://th.wikipedia.org/wiki/%E0%B9%80%E0%B8%95%E0%B8%95%E0%B8%A3%E0%B8%B2%E0%B9%82%E0%B8%A5%E0%B8%88%E0%B8%B5_%E0%B8%AD%E0%B8%AD%E0%B8%9F_%E0%B8%9F%E0%B8%B2%E0%B8%A5%E0%B9%82%E0%B8%A5%E0%B8%95%E0%B9%8C" title="เตตราโลจี ออฟ ฟาลโลต์ – Thai" lang="th" hreflang="th" data-title="เตตราโลจี ออฟ ฟาลโลต์" data-language-autonym="ไทย" data-language-local-name="Thai" class="interlanguage-link-target"><span>ไทย</span></a></li><li class="interlanguage-link interwiki-tr mw-list-item"><a href="https://tr.wikipedia.org/wiki/Fallot_tetralojisi" title="Fallot tetralojisi – Turkish" lang="tr" hreflang="tr" data-title="Fallot tetralojisi" data-language-autonym="Türkçe" data-language-local-name="Turkish" class="interlanguage-link-target"><span>Türkçe</span></a></li><li class="interlanguage-link interwiki-uk mw-list-item"><a href="https://uk.wikipedia.org/wiki/%D0%A2%D0%B5%D1%82%D1%80%D0%B0%D0%B4%D0%B0_%D0%A4%D0%B0%D0%BB%D0%BB%D0%BE" title="Тетрада Фалло – Ukrainian" lang="uk" hreflang="uk" data-title="Тетрада Фалло" data-language-autonym="Українська" data-language-local-name="Ukrainian" class="interlanguage-link-target"><span>Українська</span></a></li><li class="interlanguage-link interwiki-vi mw-list-item"><a href="https://vi.wikipedia.org/wiki/T%E1%BB%A9_ch%E1%BB%A9ng_Fallot" title="Tứ chứng Fallot – Vietnamese" lang="vi" hreflang="vi" data-title="Tứ chứng Fallot" data-language-autonym="Tiếng Việt" data-language-local-name="Vietnamese" class="interlanguage-link-target"><span>Tiếng Việt</span></a></li><li class="interlanguage-link interwiki-zh mw-list-item"><a href="https://zh.wikipedia.org/wiki/%E6%B3%95%E6%B4%9B%E5%9B%9B%E8%81%94%E7%97%87" 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.hatnote{display:none!important}}</style><div role="note" class="hatnote navigation-not-searchable">Not to be confused with <a href="/wiki/Teratology" title="Teratology">Teratology</a>.</div> <div class="shortdescription nomobile noexcerpt noprint searchaux" style="display:none">Medical condition</div><style data-mw-deduplicate="TemplateStyles:r1257001546">.mw-parser-output .infobox-subbox{padding:0;border:none;margin:-3px;width:auto;min-width:100%;font-size:100%;clear:none;float:none;background-color:transparent}.mw-parser-output .infobox-3cols-child{margin:auto}.mw-parser-output .infobox .navbar{font-size:100%}@media screen{html.skin-theme-clientpref-night .mw-parser-output .infobox-full-data:not(.notheme)>div:not(.notheme)[style]{background:#1f1f23!important;color:#f8f9fa}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .infobox-full-data:not(.notheme) div:not(.notheme){background:#1f1f23!important;color:#f8f9fa}}@media(min-width:640px){body.skin--responsive .mw-parser-output .infobox-table{display:table!important}body.skin--responsive .mw-parser-output .infobox-table>caption{display:table-caption!important}body.skin--responsive .mw-parser-output .infobox-table>tbody{display:table-row-group}body.skin--responsive .mw-parser-output .infobox-table tr{display:table-row!important}body.skin--responsive .mw-parser-output .infobox-table th,body.skin--responsive .mw-parser-output .infobox-table td{padding-left:inherit;padding-right:inherit}}</style><table class="infobox ib-medical-condition"><tbody><tr><th colspan="2" class="infobox-above" style="background:#ccc">Tetralogy of Fallot</th></tr><tr><th scope="row" class="infobox-label">Other names</th><td class="infobox-data">Fallot’s syndrome, Fallot’s tetrad, Steno–Fallot tetralogy<sup id="cite_ref-Name2016_1-0" class="reference"><a href="#cite_note-Name2016-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup></td></tr><tr style="background-color: #f8f9fa;"><td colspan="2" class="infobox-full-data"><span typeof="mw:File"><a href="/wiki/File:Tetralogy_of_Fallot.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/e/e6/Tetralogy_of_Fallot.svg/350px-Tetralogy_of_Fallot.svg.png" decoding="async" width="350" height="251" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/e/e6/Tetralogy_of_Fallot.svg/525px-Tetralogy_of_Fallot.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/e/e6/Tetralogy_of_Fallot.svg/700px-Tetralogy_of_Fallot.svg.png 2x" data-file-width="614" data-file-height="441" /></a></span></td></tr><tr><td colspan="2" class="infobox-full-data">Diagram of a healthy heart and one with tetralogy of Fallot</td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_specialty" title="Medical specialty">Specialty</a></th><td class="infobox-data"><a href="/wiki/Cardiac_surgery" title="Cardiac surgery">Cardiac surgery</a>, <a href="/wiki/Pediatrics" title="Pediatrics">pediatrics</a></td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Signs_and_symptoms" title="Signs and symptoms">Symptoms</a></th><td class="infobox-data">Episodes of <a href="/wiki/Cyanosis" title="Cyanosis">bluish color to the skin</a>, difficulty breathing, <a href="/wiki/Heart_murmur" title="Heart murmur">heart murmur</a>, <a href="/wiki/Finger_clubbing" class="mw-redirect" title="Finger clubbing">finger clubbing</a><sup id="cite_ref-NIH2011Sym_2-0" class="reference"><a href="#cite_note-NIH2011Sym-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Complication_(medicine)" title="Complication (medicine)">Complications</a></th><td class="infobox-data"><a href="/wiki/Heart_arrhythmia" class="mw-redirect" title="Heart arrhythmia">Irregular heart rate</a>, <a href="/wiki/Pulmonary_regurgitation" title="Pulmonary regurgitation">pulmonary regurgitation</a><sup id="cite_ref-War2005_3-0" class="reference"><a href="#cite_note-War2005-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label">Usual onset</th><td class="infobox-data">From birth<sup id="cite_ref-NIH2011What_4-0" class="reference"><a href="#cite_note-NIH2011What-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label">Causes</th><td class="infobox-data">Unknown<sup id="cite_ref-Roos-Hesselink_2017_5-0" class="reference"><a href="#cite_note-Roos-Hesselink_2017-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Risk_factor" title="Risk factor">Risk factors</a></th><td class="infobox-data"><a href="/wiki/Alcohol_(drug)" title="Alcohol (drug)">Alcohol</a>, <a href="/wiki/Diabetes" title="Diabetes">diabetes</a>, >40, <a href="/wiki/Rubella" title="Rubella">rubella</a> during pregnancy<sup id="cite_ref-Roos-Hesselink_2017_5-1" class="reference"><a href="#cite_note-Roos-Hesselink_2017-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_diagnosis" title="Medical diagnosis">Diagnostic method</a></th><td class="infobox-data">Based on symptoms, <a href="/wiki/Echocardiogram" class="mw-redirect" title="Echocardiogram">echocardiogram</a><sup id="cite_ref-NIH2011Diag_6-0" class="reference"><a href="#cite_note-NIH2011Diag-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Differential_diagnosis" title="Differential diagnosis">Differential diagnosis</a></th><td class="infobox-data"><a href="/wiki/Transposition_of_the_great_arteries" class="mw-redirect" title="Transposition of the great arteries">Transposition of the great arteries</a>, <a href="/wiki/Eisenmenger_syndrome" title="Eisenmenger syndrome">Eisenmenger syndrome</a>, <a href="/wiki/Ebstein_anomaly" class="mw-redirect" title="Ebstein anomaly">Ebstein anomaly</a><sup id="cite_ref-7" class="reference"><a href="#cite_note-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label">Treatment</th><td class="infobox-data"><a href="/wiki/Open_heart_surgery" class="mw-redirect" title="Open heart surgery">Open heart surgery</a><sup id="cite_ref-NIH2011Tx_8-0" class="reference"><a href="#cite_note-NIH2011Tx-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label">Frequency</th><td class="infobox-data">1 in 2,000 babies<sup id="cite_ref-NIH2011What_4-1" class="reference"><a href="#cite_note-NIH2011What-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></td></tr></tbody></table> <p><b>Tetralogy of Fallot</b> (<b>TOF</b>), formerly known as Steno-Fallot tetralogy,<sup id="cite_ref-Diaz-Frias_2021_9-0" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> is a <a href="/wiki/Congenital_heart_defect" title="Congenital heart defect">congenital heart defect</a> characterized by four specific cardiac defects.<sup id="cite_ref-NIH2011What_4-2" class="reference"><a href="#cite_note-NIH2011What-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Classically, the four defects are:<sup id="cite_ref-NIH2011What_4-3" class="reference"><a href="#cite_note-NIH2011What-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p> <ul><li><a href="/wiki/Pulmonary_stenosis" class="mw-redirect" title="Pulmonary stenosis">pulmonary stenosis</a>, which is narrowing of the exit from the right ventricle;</li> <li>a <a href="/wiki/Ventricular_septal_defect" title="Ventricular septal defect">ventricular septal defect</a>, which is a hole allowing blood to flow between the two <a href="/wiki/Ventricle_(heart)" title="Ventricle (heart)">ventricles</a>;</li> <li><a href="/wiki/Right_ventricular_hypertrophy" title="Right ventricular hypertrophy">right ventricular hypertrophy</a>, which is thickening of the right ventricular muscle; and</li> <li>an <a href="/wiki/Overriding_aorta" title="Overriding aorta">overriding aorta</a>, which is where the <a href="/wiki/Aorta" title="Aorta">aorta</a> expands to allow blood from both ventricles to enter.</li></ul> <p>At birth, children may be asymptomatic or present with many severe symptoms.<sup id="cite_ref-Curr2016_10-0" class="reference"><a href="#cite_note-Curr2016-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> Later in infancy, there are typically episodes of bluish colour to the skin due to a lack of sufficient oxygenation, known as <a href="/wiki/Cyanosis" title="Cyanosis">cyanosis</a>.<sup id="cite_ref-NIH2011Sym_2-1" class="reference"><a href="#cite_note-NIH2011Sym-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> When affected babies cry or have a <a href="/wiki/Bowel_movement" class="mw-redirect" title="Bowel movement">bowel movement</a>, they may undergo a "tet spell" where they turn cyanotic, have difficulty breathing, become limp, and occasionally <a href="/wiki/Unconsciousness" title="Unconsciousness">lose consciousness</a>.<sup id="cite_ref-NIH2011Sym_2-2" class="reference"><a href="#cite_note-NIH2011Sym-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Other symptoms may include a <a href="/wiki/Heart_murmur" title="Heart murmur">heart murmur</a>, <a href="/wiki/Finger_clubbing" class="mw-redirect" title="Finger clubbing">finger clubbing</a>, and easy tiring upon <a href="/wiki/Breastfeeding" title="Breastfeeding">breastfeeding</a>.<sup id="cite_ref-NIH2011Sym_2-3" class="reference"><a href="#cite_note-NIH2011Sym-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p><p>The cause of tetralogy of Fallot is typically not known.<sup id="cite_ref-Roos-Hesselink_2017_5-2" class="reference"><a href="#cite_note-Roos-Hesselink_2017-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> Risk factors include a mother who uses <a href="/wiki/Alcohol_(drug)" title="Alcohol (drug)">alcohol</a>, has <a href="/wiki/Diabetes" title="Diabetes">diabetes</a>, is over the age of 40, or gets <a href="/wiki/Rubella" title="Rubella">rubella</a> during <a href="/wiki/Pregnancy" title="Pregnancy">pregnancy</a>.<sup id="cite_ref-Roos-Hesselink_2017_5-3" class="reference"><a href="#cite_note-Roos-Hesselink_2017-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 62">: 62 </span></sup> It may also be associated with <a href="/wiki/Down_syndrome" title="Down syndrome">Down syndrome</a> and other chromosomal defects that cause congenital heart defects.<sup id="cite_ref-NIH2011Ca_11-0" class="reference"><a href="#cite_note-NIH2011Ca-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> </p><p>TOF is typically treated by <a href="/wiki/Open_heart_surgery" class="mw-redirect" title="Open heart surgery">open heart surgery</a> in the first year of life.<sup id="cite_ref-NIH2011Tx_8-1" class="reference"><a href="#cite_note-NIH2011Tx-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> The timing of surgery depends on the baby's symptoms and size.<sup id="cite_ref-NIH2011Tx_8-2" class="reference"><a href="#cite_note-NIH2011Tx-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> The procedure involves increasing the size of the <a href="/wiki/Pulmonary_valve" title="Pulmonary valve">pulmonary valve</a> and <a href="/wiki/Pulmonary_arteries" class="mw-redirect" title="Pulmonary arteries">pulmonary arteries</a> and repairing the ventricular septal defect.<sup id="cite_ref-NIH2011Tx_8-3" class="reference"><a href="#cite_note-NIH2011Tx-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> In babies who are too small, a temporary surgery may be done with plans for a second surgery when the baby is bigger.<sup id="cite_ref-NIH2011Tx_8-4" class="reference"><a href="#cite_note-NIH2011Tx-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> With proper care, most people who are affected live to be adults.<sup id="cite_ref-NIH2011What_4-4" class="reference"><a href="#cite_note-NIH2011What-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Long-term problems may include an <a href="/wiki/Heart_arrhythmia" class="mw-redirect" title="Heart arrhythmia">irregular heart rate</a> and <a href="/wiki/Pulmonary_regurgitation" title="Pulmonary regurgitation">pulmonary regurgitation</a>.<sup id="cite_ref-War2005_3-1" class="reference"><a href="#cite_note-War2005-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p><p>The prevalence of TOF is estimated to be anywhere from 0.02 to 0.04%.<sup id="cite_ref-NIH2011What_4-5" class="reference"><a href="#cite_note-NIH2011What-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Though males and females were initially thought to be affected equally, more recent studies have found males to be affected more than females.<sup id="cite_ref-NIH2011What_4-6" class="reference"><a href="#cite_note-NIH2011What-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-0" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> It is the most common complex congenital heart defect, accounting for about 10 percent of cases.<sup id="cite_ref-13" class="reference"><a href="#cite_note-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-14" class="reference"><a href="#cite_note-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> It was initially described in 1671 by <a href="/wiki/Nicolas_Steno" title="Nicolas Steno">Niels Steensen</a>.<sup id="cite_ref-Name2016_1-1" class="reference"><a href="#cite_note-Name2016-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-VanP2009_15-0" class="reference"><a href="#cite_note-VanP2009-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> A further description was published in 1888 by the French physician <a href="/wiki/Etienne_Fallot" class="mw-redirect" title="Etienne Fallot">Étienne-Louis Arthur Fallot</a>, after whom it is named.<sup id="cite_ref-Name2016_1-2" class="reference"><a href="#cite_note-Name2016-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Fallot1888_16-0" class="reference"><a href="#cite_note-Fallot1888-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> The first total surgical repair was carried out in 1954.<sup id="cite_ref-War2005_3-2" class="reference"><a href="#cite_note-War2005-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p> <style data-mw-deduplicate="TemplateStyles:r886046785">.mw-parser-output .toclimit-2 .toclevel-1 ul,.mw-parser-output .toclimit-3 .toclevel-2 ul,.mw-parser-output .toclimit-4 .toclevel-3 ul,.mw-parser-output .toclimit-5 .toclevel-4 ul,.mw-parser-output .toclimit-6 .toclevel-5 ul,.mw-parser-output .toclimit-7 .toclevel-6 ul{display:none}</style><div class="toclimit-3"><meta property="mw:PageProp/toc" /></div> <div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=1" title="Edit section: Signs and symptoms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:CongenitalHeartCase-133.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/f/f1/CongenitalHeartCase-133.jpg/290px-CongenitalHeartCase-133.jpg" decoding="async" width="290" height="207" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/f/f1/CongenitalHeartCase-133.jpg/435px-CongenitalHeartCase-133.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/f/f1/CongenitalHeartCase-133.jpg/580px-CongenitalHeartCase-133.jpg 2x" data-file-width="970" data-file-height="692" /></a><figcaption><a href="/wiki/Digital_clubbing" class="mw-redirect" title="Digital clubbing">Digital clubbing</a> with <a href="/wiki/Cyanosis" title="Cyanosis">cyanotic</a> nail beds in an adult with tetralogy of Fallot</figcaption></figure> <p>Tetralogy of Fallot results in low <a href="/wiki/Oxygenation_(medical)" class="mw-redirect" title="Oxygenation (medical)">oxygenation</a> of blood. This is due to a mixing of oxygenated and deoxygenated blood in the left ventricle via the <a href="/wiki/Ventricular_septal_defect" title="Ventricular septal defect">ventricular septal defect</a> (VSD) and preferential flow of the mixed blood from both ventricles through the aorta because of the obstruction to flow through the pulmonary valve. The latter is known as a <a href="/wiki/Right-to-left_shunt" title="Right-to-left shunt">right-to-left shunt</a>.<sup id="cite_ref-Abdulla_2011_17-0" class="reference"><a href="#cite_note-Abdulla_2011-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </p><p>Infants with TOF – a cyanotic heart disease – have low blood oxygen saturation.<sup id="cite_ref-Abdulla_2011_17-1" class="reference"><a href="#cite_note-Abdulla_2011-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> Blood oxygenation varies greatly from one patient to another depending on the severity of the anatomic defects.<sup id="cite_ref-Curr2016_10-1" class="reference"><a href="#cite_note-Curr2016-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> Typical ranges vary from 60% to around 90%.<sup id="cite_ref-Abdulla_2011_17-2" class="reference"><a href="#cite_note-Abdulla_2011-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> Depending on the degree of obstruction, symptoms vary from no cyanosis or mild cyanosis to profound cyanosis at birth.<sup id="cite_ref-Curr2016_10-2" class="reference"><a href="#cite_note-Curr2016-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> If the baby is not cyanotic, then it is sometimes referred to as a "pink tet".<sup id="cite_ref-urlTetralogy_of_Fallot:_Overview_-_eMedicine_18-0" class="reference"><a href="#cite_note-urlTetralogy_of_Fallot:_Overview_-_eMedicine-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> Other symptoms include a <a href="/wiki/Heart_murmur" title="Heart murmur">heart murmur</a> which may range from almost imperceptible to very loud, difficulty in feeding, failure to gain weight, retarded growth and physical development, labored breathing (dyspnea) on exertion, clubbing of the fingers and toes, and <a href="/wiki/Polycythemia" title="Polycythemia">polycythemia</a>.<sup id="cite_ref-NIH2011Sym_2-4" class="reference"><a href="#cite_note-NIH2011Sym-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> The baby may turn blue with breastfeeding or crying.<sup id="cite_ref-NIH2011Sym_2-5" class="reference"><a href="#cite_note-NIH2011Sym-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p><p>Those born with tetralogy of Fallot are more likely to experience psychiatric disorders such as <a href="/wiki/Attention_deficit_hyperactivity_disorder" title="Attention deficit hyperactivity disorder">attention deficit hyperactivity disorder</a> (ADHD) in later life, potentially due to underlying genetic changes that predispose to both conditions.<sup id="cite_ref-19" class="reference"><a href="#cite_note-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Tet_spells">Tet spells</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=2" title="Edit section: Tet spells"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Infants and children with unrepaired tetralogy of Fallot may develop "tet spells".<sup id="cite_ref-Abdulla_2011_17-3" class="reference"><a href="#cite_note-Abdulla_2011-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> These are acute hypoxia spells, characterized by shortness of breath, cyanosis, agitation, and loss of consciousness.<sup id="cite_ref-Munoz_2010_20-0" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 200">: 200 </span></sup> This may be initiated by any event – such as anxiety, pain, dehydration, or fever<sup id="cite_ref-Munoz_2010_20-1" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup> – leading to decreased oxygen saturation or that causes decreased systemic vascular resistance, which in turn leads to increased shunting through the ventricular septal defect.<sup id="cite_ref-Abdulla_2011_17-4" class="reference"><a href="#cite_note-Abdulla_2011-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> Typically, these spells decrease in frequency after the first four years of life.<sup id="cite_ref-Diaz-Frias_2021_9-1" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> </p><p>Clinically, tet spells are characterized by a sudden, marked increase in cyanosis followed by <a href="/wiki/Syncope_(medicine)" title="Syncope (medicine)">syncope</a>.<sup id="cite_ref-Munoz_2010_20-2" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 200">: 200 </span></sup> </p><p>Older children will often <a href="/wiki/Squatting_position" title="Squatting position">squat</a> instinctively during a tet spell.<sup id="cite_ref-Abdulla_2011_17-5" class="reference"><a href="#cite_note-Abdulla_2011-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> This increases <a href="/wiki/Systemic_vascular_resistance" class="mw-redirect" title="Systemic vascular resistance">systemic vascular resistance</a> and allows for a temporary reversal of the <a href="/wiki/Cardiac_shunt" title="Cardiac shunt">shunt</a>. It increases pressure on the left side of the heart, decreasing the right to left shunt, thus decreasing the amount of deoxygenated blood entering the systemic circulation.<sup id="cite_ref-Squatting:_the_hemodynamic_change_i_21-0" class="reference"><a href="#cite_note-Squatting:_the_hemodynamic_change_i-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-pmid5638470_22-0" class="reference"><a href="#cite_note-pmid5638470-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Cause">Cause</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=3" title="Edit section: Cause"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>While the specific causes of TOF have not been fully identified, there are various environmental or genetic factors that have been associated with TOF. So far, around 20% of overall congenital heart defect cases have been due to known causes such as genetic defects and <a href="/wiki/Teratogens" class="mw-redirect" title="Teratogens">teratogens</a> which are various factors causing embryo development abnormalities or birth defects.<sup id="cite_ref-Wang_2014_23-0" class="reference"><a href="#cite_note-Wang_2014-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup> However, the other 80% of cases have little known about their cause.<sup id="cite_ref-Wang_2014_23-1" class="reference"><a href="#cite_note-Wang_2014-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup> </p><p>Genetic factors linked to TOF include various gene mutations or deletions. Gene deletions associated with TOF include chromosome 22 deletion as well as <a href="/wiki/DiGeorge_syndrome" title="DiGeorge syndrome">DiGeorge syndrome</a>.<sup id="cite_ref-Francois_2016_24-0" class="reference"><a href="#cite_note-Francois_2016-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup> </p><p>Specific genes associations with TOF include: </p> <ul><li><a href="/wiki/JAG1" title="JAG1">JAG1</a> codes for ligands within the Notch family of proteins and is highly expressed in the developing heart.<sup id="cite_ref-pmid11152664_25-0" class="reference"><a href="#cite_note-pmid11152664-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup> Mutations of the JAG1 gene can lead to abnormal heart development associated with TOF.<sup id="cite_ref-pmid11152664_25-1" class="reference"><a href="#cite_note-pmid11152664-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Nkx2-5" class="mw-redirect" title="Nkx2-5">NKX2-5</a> codes for cardiac morphogenesis regulators to allow for proper heart development.<sup id="cite_ref-26" class="reference"><a href="#cite_note-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup> Defects in this gene typically causes septal defects and has been associated with around 4% of all TOF cases.<sup id="cite_ref-pmid11714651_27-0" class="reference"><a href="#cite_note-pmid11714651-27"><span class="cite-bracket">[</span>27<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/ZFPM2" title="ZFPM2">ZFPM2</a> is another cardiac regulator involved in regulation of GATA4.<sup id="cite_ref-pmid14517948_28-0" class="reference"><a href="#cite_note-pmid14517948-28"><span class="cite-bracket">[</span>28<span class="cite-bracket">]</span></a></sup> Mutations of the ZFPM2 gene lead to reduced GATA production and have been seen in some TOF cases.<sup id="cite_ref-pmid14517948_28-1" class="reference"><a href="#cite_note-pmid14517948-28"><span class="cite-bracket">[</span>28<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/VEGF" class="mw-redirect" title="VEGF">VEGF</a> a well-known endothelial growth factor involved in the vascularization of the heart.<sup id="cite_ref-pmid15937089_29-0" class="reference"><a href="#cite_note-pmid15937089-29"><span class="cite-bracket">[</span>29<span class="cite-bracket">]</span></a></sup> Decreased VEGF expression has been shown to be a modifier of TOF.<sup id="cite_ref-pmid15937089_29-1" class="reference"><a href="#cite_note-pmid15937089-29"><span class="cite-bracket">[</span>29<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Notch_1" title="Notch 1">NOTCH1</a> is involved in the vascularization of tissues and is the most common site of genetic variations involved with TOF, accounting for 7% of all TOF cases.<sup id="cite_ref-Page_2019_30-0" class="reference"><a href="#cite_note-Page_2019-30"><span class="cite-bracket">[</span>30<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/TBX1" title="TBX1">TBX1</a> expresses progenitors involved with the development of the right ventricle.<sup id="cite_ref-Griffin_2010_31-0" class="reference"><a href="#cite_note-Griffin_2010-31"><span class="cite-bracket">[</span>31<span class="cite-bracket">]</span></a></sup> Chromosome 22q11 deletions also deleting TBX1 gene have been seen in 17% TOF cases.<sup id="cite_ref-Griffin_2010_31-1" class="reference"><a href="#cite_note-Griffin_2010-31"><span class="cite-bracket">[</span>31<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/FLT4" title="FLT4">FLT4</a> gene expression leads to Vascular endothelial growth factor receptor 3 (<a href="/wiki/VEGFR-3" class="mw-redirect" title="VEGFR-3">VEGFR-3</a>) which helps vascularization.<sup id="cite_ref-Page_2019_30-1" class="reference"><a href="#cite_note-Page_2019-30"><span class="cite-bracket">[</span>30<span class="cite-bracket">]</span></a></sup> Mutations of this gene have been associated with TOF, accounting for 2.4% of all cases.<sup id="cite_ref-Page_2019_30-2" class="reference"><a href="#cite_note-Page_2019-30"><span class="cite-bracket">[</span>30<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/FOXC2" title="FOXC2">FOXC2</a> is another gene involved in embryonic development of the cardiac system.<sup id="cite_ref-Morgenthau_2018_32-0" class="reference"><a href="#cite_note-Morgenthau_2018-32"><span class="cite-bracket">[</span>32<span class="cite-bracket">]</span></a></sup> Mutations of this gene have been shown to result in dysfunctional lymphatic syndrome and TOF.<sup id="cite_ref-Morgenthau_2018_32-1" class="reference"><a href="#cite_note-Morgenthau_2018-32"><span class="cite-bracket">[</span>32<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/GATA4" title="GATA4">GATA4</a> aids in cardiac development by helping increase the production of cardiomyocytes.<sup id="cite_ref-33" class="reference"><a href="#cite_note-33"><span class="cite-bracket">[</span>33<span class="cite-bracket">]</span></a></sup> Mutations of this gene have been seen in various familial TOF cases often lasting 2-3 generations.<sup id="cite_ref-Morgenthau_2018_32-2" class="reference"><a href="#cite_note-Morgenthau_2018-32"><span class="cite-bracket">[</span>32<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/FLNA" title="FLNA">FLNA</a> is a protein coded by the gene of the same name that crosslinks <a href="/wiki/Actin_filaments" class="mw-redirect" title="Actin filaments">actin filaments</a> into networks in <a href="/wiki/Cytoplasm" title="Cytoplasm">cytoplasm</a> and helps anchor membrane proteins for the actin <a href="/wiki/Cytoskeleton" title="Cytoskeleton">cytoskeleton</a>. Mutations of this gene were seen to cause TOF in some patients.<sup id="cite_ref-34" class="reference"><a href="#cite_note-34"><span class="cite-bracket">[</span>34<span class="cite-bracket">]</span></a></sup></li></ul> <p>The Environmental Factors that have been studied to potentially be associated with TOF include: </p> <ul><li>Maternal Alcohol consumption: During <a href="/wiki/Embryonic_development" class="mw-redirect" title="Embryonic development">embryonic development</a>, many of the body’s processing and filtration systems are not fully developed.<sup id="cite_ref-Zhu_2015_35-0" class="reference"><a href="#cite_note-Zhu_2015-35"><span class="cite-bracket">[</span>35<span class="cite-bracket">]</span></a></sup> Fetal body is unable to process alcohol as well as adults which can lead to improper development, including <a href="/wiki/Cardiogenesis" class="mw-redirect" title="Cardiogenesis">cardiogenesis</a>.<sup id="cite_ref-Zhu_2015_35-1" class="reference"><a href="#cite_note-Zhu_2015-35"><span class="cite-bracket">[</span>35<span class="cite-bracket">]</span></a></sup> While no conclusive evidence has been found between effects of alcohol consumption and TOF, maternal alcohol consumption has been seen in various patients with TOF.<sup id="cite_ref-Zhu_2015_35-2" class="reference"><a href="#cite_note-Zhu_2015-35"><span class="cite-bracket">[</span>35<span class="cite-bracket">]</span></a></sup></li> <li>Maternal smoking: Maternal smoking has been associated with various fetal complications such as premature delivery and low birth weight which can lead to TOF.<sup id="cite_ref-Wang_2014_23-2" class="reference"><a href="#cite_note-Wang_2014-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup> In the famous Baltimore-Washington Study, it was reported that smoking more than one pack per day while pregnant was associated with two specific cardiac deflects, both part of TOF: pulmonary stenosis and transposition with VSD.<sup id="cite_ref-Wang_2014_23-3" class="reference"><a href="#cite_note-Wang_2014-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup></li> <li>Maternal diabetes: Maternal diabetes, <a href="/wiki/Diabetes_mellitus" class="mw-redirect" title="Diabetes mellitus">diabetes Mellitus</a>, and <a href="/wiki/Gestational_diabetes" title="Gestational diabetes">gestational diabetes</a> are well-known risk factors of fetal CHD, including TOF.<sup id="cite_ref-Priest_2015_36-0" class="reference"><a href="#cite_note-Priest_2015-36"><span class="cite-bracket">[</span>36<span class="cite-bracket">]</span></a></sup> Maternal diabetes has been shown to increase the risk of cardiovascular deformations, namely the transposition of great arteries, one of the four deformations in TOF.<sup id="cite_ref-37" class="reference"><a href="#cite_note-37"><span class="cite-bracket">[</span>37<span class="cite-bracket">]</span></a></sup> Studies have also looked at whether diabetes increases the risk of malformation or poor sugar regulation and have found that sugar regulation does not significantly affect cardiac malformations.<sup id="cite_ref-Priest_2015_36-1" class="reference"><a href="#cite_note-Priest_2015-36"><span class="cite-bracket">[</span>36<span class="cite-bracket">]</span></a></sup> Retrospective studies have shown that diabetic mothers with good glucose control still retained the elevated CHD risk.<sup id="cite_ref-Priest_2015_36-2" class="reference"><a href="#cite_note-Priest_2015-36"><span class="cite-bracket">[</span>36<span class="cite-bracket">]</span></a></sup></li> <li>Rubella: Rubella is characterized as mild, contagious viral disease with often unnoticed consequences.<sup id="cite_ref-Yazigi_2017_38-0" class="reference"><a href="#cite_note-Yazigi_2017-38"><span class="cite-bracket">[</span>38<span class="cite-bracket">]</span></a></sup> Infection with rubella during the first trimester has been seen to cause various fetal malformations, including TOF.<sup id="cite_ref-Yazigi_2017_38-1" class="reference"><a href="#cite_note-Yazigi_2017-38"><span class="cite-bracket">[</span>38<span class="cite-bracket">]</span></a></sup></li> <li>Maternal Age: Older maternal age, especially after 35 can have various pregnancy risks due to existing co-morbidities such as hypertension, diabetes, hypothyroidism, and consanguinity.<sup id="cite_ref-Hashim_2020_39-0" class="reference"><a href="#cite_note-Hashim_2020-39"><span class="cite-bracket">[</span>39<span class="cite-bracket">]</span></a></sup> These risk factors can effect fetal development and lead to various fetal conditions such as CHD (including TOF), Down Syndrome and Autism.<sup id="cite_ref-Hashim_2020_39-1" class="reference"><a href="#cite_note-Hashim_2020-39"><span class="cite-bracket">[</span>39<span class="cite-bracket">]</span></a></sup></li></ul> <p><a href="/wiki/Embryology" title="Embryology">Embryology</a> studies show that <a href="/wiki/Anterior" class="mw-redirect" title="Anterior">anterior</a> malalignment of the <a href="/wiki/Aorticopulmonary_septum" title="Aorticopulmonary septum">aorticopulmonary septum</a> results in the clinical combination of a <a href="/wiki/Ventricular_septal_defect" title="Ventricular septal defect">ventricular septal defect</a> (VSD), <a href="/wiki/Pulmonary_Stenosis" class="mw-redirect" title="Pulmonary Stenosis">pulmonary stenosis</a>, and an <a href="/wiki/Overriding_aorta" title="Overriding aorta">overriding aorta</a>.<sup id="cite_ref-Munoz_2010_20-3" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 200">: 200 </span></sup> <a href="/wiki/Right_ventricular_hypertrophy" title="Right ventricular hypertrophy">Right ventricular hypertrophy</a> develops progressively from resistance to blood flow through the <a href="/wiki/Ventricle_(heart)" title="Ventricle (heart)">right ventricular</a> outflow tract.<sup id="cite_ref-Curr2016_10-3" class="reference"><a href="#cite_note-Curr2016-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Pathophysiology">Pathophysiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=4" title="Edit section: Pathophysiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <figure class="mw-default-size" typeof="mw:File/Thumb"><span><video id="mwe_player_0" poster="//upload.wikimedia.org/wikipedia/commons/thumb/d/d3/Tetralogy_of_fallot_video.webm/220px--Tetralogy_of_fallot_video.webm.jpg" controls="" preload="none" data-mw-tmh="" class="mw-file-element" width="220" height="124" data-durationhint="364" data-mwtitle="Tetralogy_of_fallot_video.webm" data-mwprovider="wikimediacommons" resource="/wiki/File:Tetralogy_of_fallot_video.webm"><source src="//upload.wikimedia.org/wikipedia/commons/transcoded/d/d3/Tetralogy_of_fallot_video.webm/Tetralogy_of_fallot_video.webm.480p.vp9.webm" type="video/webm; codecs="vp9, opus"" data-transcodekey="480p.vp9.webm" data-width="854" data-height="480" /><source src="//upload.wikimedia.org/wikipedia/commons/transcoded/d/d3/Tetralogy_of_fallot_video.webm/Tetralogy_of_fallot_video.webm.720p.vp9.webm" type="video/webm; codecs="vp9, opus"" data-transcodekey="720p.vp9.webm" data-width="1280" data-height="720" /><source src="//upload.wikimedia.org/wikipedia/commons/d/d3/Tetralogy_of_fallot_video.webm" type="video/webm; codecs="vp9, opus"" data-width="1812" data-height="1018" /><source src="//upload.wikimedia.org/wikipedia/commons/transcoded/d/d3/Tetralogy_of_fallot_video.webm/Tetralogy_of_fallot_video.webm.240p.vp9.webm" type="video/webm; codecs="vp9, opus"" data-transcodekey="240p.vp9.webm" data-width="426" data-height="240" /><source src="//upload.wikimedia.org/wikipedia/commons/transcoded/d/d3/Tetralogy_of_fallot_video.webm/Tetralogy_of_fallot_video.webm.360p.vp9.webm" type="video/webm; codecs="vp9, opus"" data-transcodekey="360p.vp9.webm" data-width="640" data-height="360" /><source src="//upload.wikimedia.org/wikipedia/commons/transcoded/d/d3/Tetralogy_of_fallot_video.webm/Tetralogy_of_fallot_video.webm.360p.webm" type="video/webm; codecs="vp8, vorbis"" data-transcodekey="360p.webm" data-width="640" data-height="360" /><track src="https://commons.wikimedia.org/w/api.php?action=timedtext&title=File%3ATetralogy_of_fallot_video.webm&lang=ar&trackformat=vtt&origin=%2A" kind="subtitles" type="text/vtt" srclang="ar" label="العربية (ar)" data-dir="rtl" /><track src="https://commons.wikimedia.org/w/api.php?action=timedtext&title=File%3ATetralogy_of_fallot_video.webm&lang=de&trackformat=vtt&origin=%2A" kind="subtitles" type="text/vtt" srclang="de" label="Deutsch (de)" data-dir="ltr" /><track src="https://commons.wikimedia.org/w/api.php?action=timedtext&title=File%3ATetralogy_of_fallot_video.webm&lang=es&trackformat=vtt&origin=%2A" kind="subtitles" type="text/vtt" srclang="es" label="español (es)" data-dir="ltr" /><track src="https://commons.wikimedia.org/w/api.php?action=timedtext&title=File%3ATetralogy_of_fallot_video.webm&lang=fr&trackformat=vtt&origin=%2A" kind="subtitles" type="text/vtt" srclang="fr" label="français (fr)" data-dir="ltr" /><track src="https://commons.wikimedia.org/w/api.php?action=timedtext&title=File%3ATetralogy_of_fallot_video.webm&lang=it&trackformat=vtt&origin=%2A" kind="subtitles" type="text/vtt" srclang="it" label="italiano (it)" data-dir="ltr" /><track src="https://commons.wikimedia.org/w/api.php?action=timedtext&title=File%3ATetralogy_of_fallot_video.webm&lang=pt&trackformat=vtt&origin=%2A" kind="subtitles" type="text/vtt" srclang="pt" label="português (pt)" data-dir="ltr" /><track src="https://commons.wikimedia.org/w/api.php?action=timedtext&title=File%3ATetralogy_of_fallot_video.webm&lang=ru&trackformat=vtt&origin=%2A" kind="subtitles" type="text/vtt" srclang="ru" label="русский (ru)" data-dir="ltr" /></video></span><figcaption>Video explanation</figcaption></figure> <p>In healthy individuals, the human heart develops around the 20th day of gestation, when the outer endocardial tubes merge into a single cardiac tube. Thereafter, the cardiac tube begins to fold, developing into the atrium and ventricle. The right ventricle is dominant prior to birth, receiving 65% of the venous return to the heart, and is the main contributor of blood flow to the lower part of the body, the placenta, and the lungs. Though the exact cause of TOF is unknown, an association that has been observed is an anterior deviation of the infundibular septum that results in a misaligned VSD, with an overriding aorta causing a subsequent right ventricular obstruction.<sup id="cite_ref-Diaz-Frias_2021_9-2" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> </p><p>Different factors such as pulmonary stenosis can also contribute with the right ventricular outflow obstruction. During tet spells, a decrease in systemic vascular resistance or an increase in pulmonary resistance would be physiologically observed. </p><p>The main anatomic defect in TOF is the anterior deviation of the pulmonary outflow septum.<sup id="cite_ref-Curr2016_10-4" class="reference"><a href="#cite_note-Curr2016-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> This defect results in narrowing of the right ventricular outflow tract (RVOT), override of the aorta, and a VSD.<sup id="cite_ref-40" class="reference"><a href="#cite_note-40"><span class="cite-bracket">[</span>40<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Four_malformations">Four malformations</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=5" title="Edit section: Four malformations"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>"<a href="/wiki/Tetralogy" title="Tetralogy">Tetralogy</a>" denotes four parts, here implying the syndrome's four anatomic defects.<sup id="cite_ref-NIH2011Sym_2-6" class="reference"><a href="#cite_note-NIH2011Sym-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> This is not to be confused with the similarly named <a href="/wiki/Teratology" title="Teratology">teratology</a>, a field of medicine concerned with abnormal development and congenital malformations (including tetralogy of Fallot). Below are the four <a href="/wiki/Heart" title="Heart">heart</a> malformations that present together in tetralogy of Fallot: </p> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:Heart_tetralogy_fallot.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/1/12/Heart_tetralogy_fallot.svg/220px-Heart_tetralogy_fallot.svg.png" decoding="async" width="220" height="236" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/1/12/Heart_tetralogy_fallot.svg/330px-Heart_tetralogy_fallot.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/1/12/Heart_tetralogy_fallot.svg/440px-Heart_tetralogy_fallot.svg.png 2x" data-file-width="496" data-file-height="531" /></a><figcaption>Heart with tetralogy of fallot A: Pulmonary stenosis; B: Overriding aorta; C: Ventricular septal defect (VSD); D: Right ventricular hypertrophy</figcaption></figure> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:Heart_normal.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/8/86/Heart_normal.svg/220px-Heart_normal.svg.png" decoding="async" width="220" height="274" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/8/86/Heart_normal.svg/330px-Heart_normal.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/8/86/Heart_normal.svg/440px-Heart_normal.svg.png 2x" data-file-width="372" data-file-height="463" /></a><figcaption>Normal heart</figcaption></figure> <table class="wikitable"> <tbody><tr> <th>Condition </th> <th>Description </th></tr> <tr> <td><a href="/wiki/Pulmonary_stenosis" class="mw-redirect" title="Pulmonary stenosis">Pulmonary Infundibular Stenosis</a> </td> <td>A narrowing of the right ventricular outflow tract. It can occur at the <a href="/wiki/Pulmonary_valve" title="Pulmonary valve">pulmonary valve</a> (valvular stenosis) or just below the <a href="/wiki/Heart" title="Heart">pulmonary valve</a> (infundibular stenosis).<sup id="cite_ref-NIH2011What_4-7" class="reference"><a href="#cite_note-NIH2011What-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Infundibular pulmonic stenosis is mostly caused by the overgrowth of the heart muscle wall (hypertrophy of the septoparietal trabeculae),<sup id="cite_ref-gatzoulis_41-0" class="reference"><a href="#cite_note-gatzoulis-41"><span class="cite-bracket">[</span>41<span class="cite-bracket">]</span></a></sup> however, the events leading to the formation of the overriding aorta are also believed to be a cause. The pulmonic stenosis is the major cause of the malformations, with the other associated malformations acting as compensatory mechanisms to the pulmonic stenosis.<sup id="cite_ref-42" class="reference"><a href="#cite_note-42"><span class="cite-bracket">[</span>42<span class="cite-bracket">]</span></a></sup> The degree of stenosis varies between individuals with TOF and is the primary determinant of symptoms and severity. This malformation is infrequently described as <i>sub-pulmonary stenosis</i> or <i>subpulmonary obstruction</i>.<sup id="cite_ref-43" class="reference"><a href="#cite_note-43"><span class="cite-bracket">[</span>43<span class="cite-bracket">]</span></a></sup> </td></tr> <tr> <td><a href="/wiki/Overriding_aorta" title="Overriding aorta">Overriding aorta</a> </td> <td>An <a href="/wiki/Aortic_valve" title="Aortic valve">aortic valve</a> with biventricular connection, that is, it is situated above the ventricular septal defect and connected to both the right and the left ventricle. The degree to which the aorta is attached to the right ventricle is referred to as its degree of "override." The aortic root can be displaced toward the front (anteriorly) or directly above the septal defect, but it is always abnormally located to the right of the root of the pulmonary artery. The degree of override is extremely variable, with 5–95% of the valve being connected to the right ventricle.<sup id="cite_ref-gatzoulis_41-1" class="reference"><a href="#cite_note-gatzoulis-41"><span class="cite-bracket">[</span>41<span class="cite-bracket">]</span></a></sup> </td></tr> <tr> <td><a href="/wiki/Ventricular_septal_defect" title="Ventricular septal defect">Ventricular septal defect</a> (VSD) </td> <td>A hole between the two bottom chambers (ventricles) of the heart. The defect is centered around the most superior aspect of the ventricular septum (the outlet septum), and in the majority of cases is single and large. In some cases, thickening of the septum (septal hypertrophy) can narrow the margins of the defect.<sup id="cite_ref-gatzoulis_41-2" class="reference"><a href="#cite_note-gatzoulis-41"><span class="cite-bracket">[</span>41<span class="cite-bracket">]</span></a></sup> </td></tr> <tr> <td><a href="/wiki/Right_ventricular_hypertrophy" title="Right ventricular hypertrophy">Right ventricular hypertrophy</a> </td> <td>The <a href="/wiki/Right_ventricle" class="mw-redirect" title="Right ventricle">right ventricle</a> is more muscular than normal, causing a characteristic boot-shaped (<a href="/wiki/Coeur-en-sabot" class="mw-redirect" title="Coeur-en-sabot">coeur-en-sabot</a>) appearance as seen by chest X-ray. Due to the misarrangement of the external ventricular septum, the right ventricular wall increases in size to deal with the increased obstruction to the right outflow tract. This feature is now generally agreed to be a secondary anomaly, as the level of hypertrophy tends to increase with age.<sup id="cite_ref-44" class="reference"><a href="#cite_note-44"><span class="cite-bracket">[</span>44<span class="cite-bracket">]</span></a></sup> </td></tr></tbody></table> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:PLC_Fallots_tetralogy.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/e/eb/PLC_Fallots_tetralogy.jpg/220px-PLC_Fallots_tetralogy.jpg" decoding="async" width="220" height="232" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/e/eb/PLC_Fallots_tetralogy.jpg/330px-PLC_Fallots_tetralogy.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/e/eb/PLC_Fallots_tetralogy.jpg/440px-PLC_Fallots_tetralogy.jpg 2x" data-file-width="665" data-file-height="700" /></a><figcaption>Fallot's tetralogy specimen, from the UCT Pathology Learning Centre</figcaption></figure> <p>There is anatomic variation between the hearts of individuals with tetralogy of Fallot.<sup id="cite_ref-Curr2016_10-5" class="reference"><a href="#cite_note-Curr2016-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> Primarily, the degree of right ventricular outflow tract obstruction varies between patients and generally determines clinical symptoms and disease progression.<sup id="cite_ref-Curr2016_10-6" class="reference"><a href="#cite_note-Curr2016-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> </p><p>Presumably, this arises from an unequal growth of the <a href="/wiki/Aorticopulmonary_septum" title="Aorticopulmonary septum">aorticopulmonary septum</a> (aka pulmonary outflow septum).<sup id="cite_ref-Munoz_2010_20-4" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 199">: 199 </span></sup> The aorta is too large, thus "overriding," and this "steals" from the pulmonary artery, which is therefore stenosed. This then prevents ventricular wall closure, therefore VSD, and this increases the pressures on the right side, and so the R ventricle becomes bigger to handle the work.<sup id="cite_ref-Munoz_2010_20-5" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 199">: 199 </span></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Additional_anomalies">Additional anomalies</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=6" title="Edit section: Additional anomalies"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>In addition, tetralogy of Fallot may present with other anatomical anomalies, including:<sup id="cite_ref-Francois_2016_24-1" class="reference"><a href="#cite_note-Francois_2016-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 66–68">: 66–68 </span></sup><sup id="cite_ref-45" class="reference"><a href="#cite_note-45"><span class="cite-bracket">[</span>45<span class="cite-bracket">]</span></a></sup> </p> <ul><li>stenosis of the <a href="/wiki/Left_pulmonary_artery" class="mw-redirect" title="Left pulmonary artery">left pulmonary artery</a>, in 40%</li> <li>a <a href="/w/index.php?title=Bicuspid_pulmonary_valve&action=edit&redlink=1" class="new" title="Bicuspid pulmonary valve (page does not exist)">bicuspid pulmonary valve</a>, in 60%</li> <li><a href="/wiki/Right-sided_aortic_arch" title="Right-sided aortic arch">right-sided aortic arch</a>, in 25%</li> <li><a href="/wiki/Coronary_artery" class="mw-redirect" title="Coronary artery">coronary artery</a> anomalies, in 10%</li> <li>a patent <a href="/wiki/Foramen_ovale_(heart)" title="Foramen ovale (heart)">foramen ovale</a> or <a href="/wiki/Atrial_septal_defect" title="Atrial septal defect">atrial septal defect</a>, in which case the syndrome is sometimes called a pentalogy of Fallot<sup id="cite_ref-pmid7787464_46-0" class="reference"><a href="#cite_note-pmid7787464-46"><span class="cite-bracket">[</span>46<span class="cite-bracket">]</span></a></sup></li> <li>an <a href="/wiki/Atrioventricular_septal_defect" title="Atrioventricular septal defect">atrioventricular septal defect</a></li> <li>partially or totally <a href="/wiki/Anomalous_pulmonary_venous_return" class="mw-redirect" title="Anomalous pulmonary venous return">anomalous pulmonary venous return</a></li></ul> <p>Tetralogy of Fallot with <a href="/wiki/Pulmonary_atresia" title="Pulmonary atresia">pulmonary atresia</a> (<i>pseudotruncus arteriosus</i>) is a severe variant<sup id="cite_ref-pmid19079949_47-0" class="reference"><a href="#cite_note-pmid19079949-47"><span class="cite-bracket">[</span>47<span class="cite-bracket">]</span></a></sup> in which there is complete obstruction (atresia) of the right ventricular outflow tract, causing an absence of the pulmonary trunk during embryonic development.<sup id="cite_ref-Francois_2016_24-2" class="reference"><a href="#cite_note-Francois_2016-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 67–68">: 67–68 </span></sup> In these individuals, blood shunts completely from the right ventricle to the left where it is pumped only through the aorta. The lungs are perfused via extensive collaterals from the systemic arteries, and sometimes also via the ductus arteriosus.<sup id="cite_ref-Francois_2016_24-3" class="reference"><a href="#cite_note-Francois_2016-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 67–68">: 67–68 </span></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=7" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:HeartTOP.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/6/66/HeartTOP.jpg/220px-HeartTOP.jpg" decoding="async" width="220" height="243" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/6/66/HeartTOP.jpg/330px-HeartTOP.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/6/66/HeartTOP.jpg/440px-HeartTOP.jpg 2x" data-file-width="748" data-file-height="826" /></a><figcaption>A chest X-ray of a child with tetralogy of Fallot</figcaption></figure> <p>There are three different useful diagnostic tests used for the diagnosis of tetralogy of Fallot.<sup id="cite_ref-Bailliard_2009_48-0" class="reference"><a href="#cite_note-Bailliard_2009-48"><span class="cite-bracket">[</span>48<span class="cite-bracket">]</span></a></sup> These include a chest radiograph, electrocardiogram, and echocardiogram.<sup id="cite_ref-Bailliard_2009_48-1" class="reference"><a href="#cite_note-Bailliard_2009-48"><span class="cite-bracket">[</span>48<span class="cite-bracket">]</span></a></sup> The echocardiography determines the final diagnosis and typically offers enough information for surgical treatment planning.<sup id="cite_ref-Bailliard_2009_48-2" class="reference"><a href="#cite_note-Bailliard_2009-48"><span class="cite-bracket">[</span>48<span class="cite-bracket">]</span></a></sup> About half of all patients are now diagnosed before they are born.<sup id="cite_ref-Bailliard_2009_48-3" class="reference"><a href="#cite_note-Bailliard_2009-48"><span class="cite-bracket">[</span>48<span class="cite-bracket">]</span></a></sup> Differential diagnosis is when physicians diagnose between two or more conditions for a person's symptoms and this can include primary pulmonary causes of cyanosis, cyanotic heart lesions, pulmonary stenosis and transposed arterial trunks.<sup id="cite_ref-Bailliard_2009_48-4" class="reference"><a href="#cite_note-Bailliard_2009-48"><span class="cite-bracket">[</span>48<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Chest_radiograph">Chest radiograph</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=8" title="Edit section: Chest radiograph"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Before more sophisticated techniques became available, chest X-ray was the definitive method of diagnosis. The abnormal "<a href="/wiki/Coeur-en-sabot" class="mw-redirect" title="Coeur-en-sabot">coeur-en-sabot</a>" (boot-like) appearance of a heart with tetralogy of Fallot is classically visible via chest X-ray, although most infants with tetralogy may not show this finding.<sup id="cite_ref-49" class="reference"><a href="#cite_note-49"><span class="cite-bracket">[</span>49<span class="cite-bracket">]</span></a></sup> The boot like shape is due to the right ventricular hypertrophy present in TOF. Lung fields are often dark (absence of interstitial lung markings) due to decreased pulmonary blood flow.<sup id="cite_ref-Abdulla_20112_50-0" class="reference"><a href="#cite_note-Abdulla_20112-50"><span class="cite-bracket">[</span>50<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 171–172">: 171–172 </span></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Electrocardiogram">Electrocardiogram</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=9" title="Edit section: Electrocardiogram"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>An electrocardiogram (ECG) is one of the most basic procedures for assessing the heart.<sup id="cite_ref-Woods_1952_51-0" class="reference"><a href="#cite_note-Woods_1952-51"><span class="cite-bracket">[</span>51<span class="cite-bracket">]</span></a></sup> Tiny electrodes are applied to specific areas on the body, near the chest, arm, and neck. Lead cables connect the electrodes to an ECG machine. The heart's electrical activity is then measured.<sup id="cite_ref-Woods_1952_51-1" class="reference"><a href="#cite_note-Woods_1952-51"><span class="cite-bracket">[</span>51<span class="cite-bracket">]</span></a></sup> Natural electrical impulses help maintain blood flowing properly by coordinating contractions in different areas of the heart.<sup id="cite_ref-Woods_1952_51-2" class="reference"><a href="#cite_note-Woods_1952-51"><span class="cite-bracket">[</span>51<span class="cite-bracket">]</span></a></sup> These impulses are recorded by an ECG, which shows how fast, the rhythm, intensity and timing of the electrical impulses as they travel through the heart.<sup id="cite_ref-Woods_1952_51-3" class="reference"><a href="#cite_note-Woods_1952-51"><span class="cite-bracket">[</span>51<span class="cite-bracket">]</span></a></sup> </p><p>Electrocardiography shows right ventricular hypertrophy (RVH), along with right axis deviation.<sup id="cite_ref-Francois_2016_24-4" class="reference"><a href="#cite_note-Francois_2016-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup> RVH is noted on EKG as tall R-waves in lead V1 and deep S-waves in lead V5–V6.<sup id="cite_ref-52" class="reference"><a href="#cite_note-52"><span class="cite-bracket">[</span>52<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Echocardiogram">Echocardiogram</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=10" title="Edit section: Echocardiogram"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Congenital heart defects are now diagnosed with <a href="/wiki/Echocardiography" title="Echocardiography">echocardiography</a>, which is quick, involves no radiation, is very specific, and can be done prenatally.<sup id="cite_ref-53" class="reference"><a href="#cite_note-53"><span class="cite-bracket">[</span>53<span class="cite-bracket">]</span></a></sup> </p><p><a href="/wiki/Echocardiography" title="Echocardiography">Echocardiography</a> establishes the presence of TOF by demonstrating a VSD, RVH, and aortic override. Many patients are diagnosed prenatally. Color Doppler (type of echocardiography) measures the degree of pulmonary stenosis. Additionally, close monitoring of the ductus arteriosus is done in the neonatal period to ensure that there is adequate blood flow through the pulmonary valve.<sup id="cite_ref-Francois_2016_24-5" class="reference"><a href="#cite_note-Francois_2016-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Abdulla_20112_50-1" class="reference"><a href="#cite_note-Abdulla_20112-50"><span class="cite-bracket">[</span>50<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 171–172">: 171–172 </span></sup> </p><p>In certain cases, coronary artery anatomy cannot be clearly viewed using echocardiogram. In this case, cardiac catheterization can be done.<sup id="cite_ref-Munoz_2010_20-6" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 37, 201">: 37, 201 </span></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Genetics">Genetics</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=11" title="Edit section: Genetics"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>From a genetics perspective, it is important to screen for DiGeorge in all babies with TOF.<sup id="cite_ref-Munoz_2010_20-7" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 37, 201">: 37, 201 </span></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Treatment">Treatment</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=12" title="Edit section: Treatment"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="mw-heading mw-heading3"><h3 id="Tet_spells_2">Tet spells</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=13" title="Edit section: Tet spells"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Tet spells are defined as cyanotic spells occurring due to the obstruction right ventricular outflow.<sup id="cite_ref-54" class="reference"><a href="#cite_note-54"><span class="cite-bracket">[</span>54<span class="cite-bracket">]</span></a></sup> Tet spells can be triggered by various factors such as crying, <a href="/wiki/Tachypnea" title="Tachypnea">progressive tachypnea</a>, and deep breathing, with symptoms including but not limited to blue skin, nails and lips, profound crying and difficulty breathing.<sup id="cite_ref-55" class="reference"><a href="#cite_note-55"><span class="cite-bracket">[</span>55<span class="cite-bracket">]</span></a></sup> </p><p>Tet spells may be treated with <a href="/wiki/Beta-blockers" class="mw-redirect" title="Beta-blockers">beta-blockers</a> such as <a href="/wiki/Propranolol" title="Propranolol">propranolol</a>, but acute episodes require rapid intervention with <a href="/wiki/Morphine" title="Morphine">morphine</a> or intranasal <a href="/wiki/Fentanyl" title="Fentanyl">fentanyl</a><sup id="cite_ref-56" class="reference"><a href="#cite_note-56"><span class="cite-bracket">[</span>56<span class="cite-bracket">]</span></a></sup> to reduce ventilatory drive, a vasopressor such as <a href="/wiki/Phenylephrine" title="Phenylephrine">phenylephrine</a>, or <a href="/wiki/Norepinephrine" title="Norepinephrine">norepinephrine</a> to increase systemic vascular resistance, and IV fluids for volume expansion.<sup id="cite_ref-Munoz_2010_20-8" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 18, 201">: 18, 201 </span></sup> </p><p>Oxygen (100%) may be effective in treating spells because it is a potent pulmonary vasodilator and systemic vasoconstrictor. This allows more blood flow to the lungs by decreasing shunting of deoxygenated blood from the right to left ventricle through the VSD. There are also simple procedures such as <a href="/wiki/Squatting_position" title="Squatting position">squatting</a> and the knee chest position which increase systemic vascular resistance and decrease right-to-left shunting of deoxygenated blood into the systemic circulation.<sup id="cite_ref-Munoz_2010_20-9" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 18, 201">: 18, 201 </span></sup><sup id="cite_ref-Squatting:_the_hemodynamic_change_i_21-1" class="reference"><a href="#cite_note-Squatting:_the_hemodynamic_change_i-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup> </p><p>If the spells are refractory to the above treatments, people are usually intubated and sedated. The treatment of last resort for tet spells is <a href="/wiki/Extracorporeal_membrane_oxygenation" title="Extracorporeal membrane oxygenation">extracorporeal membrane oxygenation</a> (ECMO) along with consideration of Blalock-Thomas-Taussig shunt (BTT shunt).<sup id="cite_ref-Munoz_2010_20-10" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 18, 201">: 18, 201 </span></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Total_surgical_repair">Total surgical repair</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=14" title="Edit section: Total surgical repair"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Total surgical repair of TOF is a curative surgery. Different techniques can be used in performing TOF repair. One method to permit pulmonary blood flow post-birth is the stenting of the ductus arterious (DA) through the inducement of a systemic-to-pulmonary shunt. This surgical approach has an 83% success rate. <sup id="cite_ref-57" class="reference"><a href="#cite_note-57"><span class="cite-bracket">[</span>57<span class="cite-bracket">]</span></a></sup>However, a transatrial, transpulmonary artery approach is used for most cases.<sup id="cite_ref-Mavroudis_2015_58-0" class="reference"><a href="#cite_note-Mavroudis_2015-58"><span class="cite-bracket">[</span>58<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 153">: 153 </span></sup> The repair consists of two main steps: closure of the VSD with a patch and reconstruction of the right ventricular outflow tract.<sup id="cite_ref-Corno_2009_59-0" class="reference"><a href="#cite_note-Corno_2009-59"><span class="cite-bracket">[</span>59<span class="cite-bracket">]</span></a></sup> </p><p>This open-heart surgery is designed to relieve the right ventricular outflow tract stenosis by careful <a href="/wiki/Segmental_resection" title="Segmental resection">resection</a> of muscle and to repair the VSD.<sup id="cite_ref-Mavroudis_2015_58-1" class="reference"><a href="#cite_note-Mavroudis_2015-58"><span class="cite-bracket">[</span>58<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 154">: 154 </span></sup>The right ventricle outflow tract can be reconstructed using mainly 2 procedures: a transannular patch (TAP) or a pulmonary valve-sparing procedure (PVS). The decision on the type of the procedure depends on individual anatomy (especially the size of the pulmonary valve). PVS showed better overall survival, event-free survival and less pulmonary regurgitation at 10, 20 and 30 years after the operation.  PVS  can be performed with or without <a href="/wiki/Ventriculotomy_(cardiac)" title="Ventriculotomy (cardiac)">ventriculotomy</a>. A study found similar overall and event-free survival and pulmonary regurgitation rate between patients who underwent PVS with ventriculotomy and the ones who did not.<sup id="cite_ref-60" class="reference"><a href="#cite_note-60"><span class="cite-bracket">[</span>60<span class="cite-bracket">]</span></a></sup> </p><p>Additional reparative or reconstructive surgery may be done on patients as required by their particular cardiac anatomy.<sup id="cite_ref-Mavroudis_2015_58-2" class="reference"><a href="#cite_note-Mavroudis_2015-58"><span class="cite-bracket">[</span>58<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 153">: 153 </span></sup> </p><p>Timing of surgery in asymptomatic patients is usually between the ages of two months to one year.<sup id="cite_ref-Munoz_2010_20-11" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 201–202">: 201–202 </span></sup> However, in symptomatic patients showing worsening blood oxygen levels, severe tet-spells (cyanotic spells), or dependence on prostaglandins from early neonatal period (to keep the ductus arteriosus open) need to be planned fairly urgently<sup id="cite_ref-Munoz_2010_20-12" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 201–202">: 201–202 </span></sup> </p><p>Potential surgical repair complications include residual ventricular septal defect, residual outflow tract obstruction, complete atrioventricular block, arrhythmias, aneurysm of right ventricular outflow patch, and pulmonary valve insufficiency.<sup id="cite_ref-Corno_2009_59-1" class="reference"><a href="#cite_note-Corno_2009-59"><span class="cite-bracket">[</span>59<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 59">: 59 </span></sup> Long-term complications most commonly include pulmonary valve regurgitation, and arrhythmias.<sup id="cite_ref-Chessa_2012_61-0" class="reference"><a href="#cite_note-Chessa_2012-61"><span class="cite-bracket">[</span>61<span class="cite-bracket">]</span></a></sup> Adults with repaired TOF require lifelong surveillance to monitor for such complications, with frequency of monitoring varying according to age, diagnostic methods, and the severity of the condition. <sup id="cite_ref-62" class="reference"><a href="#cite_note-62"><span class="cite-bracket">[</span>62<span class="cite-bracket">]</span></a></sup> </p><p>Total repair of tetralogy of Fallot initially carried a high mortality risk, but this risk has gone down steadily over the years. Surgery is now often carried out in infants one year of age or younger with less than 5% perioperative mortality.<sup id="cite_ref-Munoz_2010_20-13" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 205">: 205 </span></sup> Post surgery, most patients enjoy an active life free of symptoms.<sup id="cite_ref-Munoz_2010_20-14" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 205">: 205 </span></sup> Currently, long-term survival is close to 90%.<sup id="cite_ref-Munoz_2010_20-15" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 167">: 167 </span></sup> Today the adult TOF population continues to grow and is one of the most common congenital heart defects seen in adult outpatient clinics.<sup id="cite_ref-Roos-Hesselink_2017_5-4" class="reference"><a href="#cite_note-Roos-Hesselink_2017-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 100–101">: 100–101 </span></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Palliative_surgery">Palliative surgery</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=15" title="Edit section: Palliative surgery"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Initially surgery involved forming a side to end <a href="/wiki/Surgical_anastomosis" title="Surgical anastomosis">anastomosis</a> between the <a href="/wiki/Subclavian_artery" title="Subclavian artery">subclavian artery</a> and the <a href="/wiki/Pulmonary_artery" title="Pulmonary artery">pulmonary artery</a> -i.e. a systemic to pulmonary arterial shunt.<sup id="cite_ref-Corno_2009_59-2" class="reference"><a href="#cite_note-Corno_2009-59"><span class="cite-bracket">[</span>59<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 57">: 57 </span></sup> This redirected a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and relieving symptoms. The first <a href="/wiki/Blalock%E2%80%93Thomas%E2%80%93Taussig_shunt" title="Blalock–Thomas–Taussig shunt">Blalock–Thomas–Taussig shunt</a> surgery was performed on 15-month-old <a href="/wiki/Eileen_Saxon" title="Eileen Saxon">Eileen Saxon</a> on November 29, 1944 with the surgery ending in momentary success. Months later Saxon experienced more symptoms, and was operated on again, shortly before her 2nd birthday. She soon after died.<sup id="cite_ref-JHMI_63-0" class="reference"><a href="#cite_note-JHMI-63"><span class="cite-bracket">[</span>63<span class="cite-bracket">]</span></a></sup> </p><p>The Potts shunt<sup id="cite_ref-pmid15619282_64-0" class="reference"><a href="#cite_note-pmid15619282-64"><span class="cite-bracket">[</span>64<span class="cite-bracket">]</span></a></sup> and the Waterston–Cooley shunt<sup id="cite_ref-pmid15854971_65-0" class="reference"><a href="#cite_note-pmid15854971-65"><span class="cite-bracket">[</span>65<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-urlSystemic_to_Pulmonary_Artery_Shunting_for_Palliation:_-_eMedicine_66-0" class="reference"><a href="#cite_note-urlSystemic_to_Pulmonary_Artery_Shunting_for_Palliation:_-_eMedicine-66"><span class="cite-bracket">[</span>66<span class="cite-bracket">]</span></a></sup> are other shunt procedures which were developed for the same purpose. These are no longer used. </p><p>Currently, palliative surgery is not normally performed on infants with TOF except for extreme cases.<sup id="cite_ref-Abdulla_2011_17-6" class="reference"><a href="#cite_note-Abdulla_2011-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 173">: 173 </span></sup> For example, in symptomatic infants, a two-stage repair (initial systemic to arterial shunt placement followed by total surgical repair) may be done.<sup id="cite_ref-67" class="reference"><a href="#cite_note-67"><span class="cite-bracket">[</span>67<span class="cite-bracket">]</span></a></sup> Potential complications include inadequate pulmonary blood flow, pulmonary artery distortion, inadequate growth of the pulmonary arteries, and acquired pulmonary atresia.<sup id="cite_ref-Corno_2009_59-3" class="reference"><a href="#cite_note-Corno_2009-59"><span class="cite-bracket">[</span>59<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 59">: 59 </span></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Approaches_to_surgical_repair">Approaches to surgical repair</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=16" title="Edit section: Approaches to surgical repair"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>After years of tetralogy of Fallot surgical repair expertise, the attention shifted to the emerging evidence that long-term pulmonary insufficiency is detrimental to right ventricular function and clinical prognosis.<sup id="cite_ref-Bové_2017_68-0" class="reference"><a href="#cite_note-Bové_2017-68"><span class="cite-bracket">[</span>68<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-van_der_Ven_2019_69-0" class="reference"><a href="#cite_note-van_der_Ven_2019-69"><span class="cite-bracket">[</span>69<span class="cite-bracket">]</span></a></sup> As a result, the hunt for surgical procedures to relieve right ventricular outflow tract obstruction while minimizing pulmonary regurgitation has intensified.<sup id="cite_ref-Bové_2017_68-1" class="reference"><a href="#cite_note-Bové_2017-68"><span class="cite-bracket">[</span>68<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-van_der_Ven_2019_69-1" class="reference"><a href="#cite_note-van_der_Ven_2019-69"><span class="cite-bracket">[</span>69<span class="cite-bracket">]</span></a></sup> </p><p>A constrained right ventricular outflow tract reconstruction with a Dacron patch matched to a nominal pulmonary annulus expansion or an annulus-sparing approach yielded primary complete repair outcomes in 94 TOF infants.<sup id="cite_ref-Bové_2017_68-2" class="reference"><a href="#cite_note-Bové_2017-68"><span class="cite-bracket">[</span>68<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-van_der_Ven_2019_69-2" class="reference"><a href="#cite_note-van_der_Ven_2019-69"><span class="cite-bracket">[</span>69<span class="cite-bracket">]</span></a></sup> The pulmonary annulus size was larger in babies treated with the latter technique, as predicted.<sup id="cite_ref-Bové_2017_68-3" class="reference"><a href="#cite_note-Bové_2017-68"><span class="cite-bracket">[</span>68<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-van_der_Ven_2019_69-3" class="reference"><a href="#cite_note-van_der_Ven_2019-69"><span class="cite-bracket">[</span>69<span class="cite-bracket">]</span></a></sup> After an average follow-up of around eight years, the first group had a higher than moderate PR, yet there was no significant difference in independence from severe PR after ten years.<sup id="cite_ref-Bové_2017_68-4" class="reference"><a href="#cite_note-Bové_2017-68"><span class="cite-bracket">[</span>68<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-van_der_Ven_2019_69-4" class="reference"><a href="#cite_note-van_der_Ven_2019-69"><span class="cite-bracket">[</span>69<span class="cite-bracket">]</span></a></sup> </p><p>Furthermore, there was no significant difference in right ventricular dilation between the two techniques.<sup id="cite_ref-Bové_2017_68-5" class="reference"><a href="#cite_note-Bové_2017-68"><span class="cite-bracket">[</span>68<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-van_der_Ven_2019_69-5" class="reference"><a href="#cite_note-van_der_Ven_2019-69"><span class="cite-bracket">[</span>69<span class="cite-bracket">]</span></a></sup> Finally, they found that reconstructing the pulmonary annulus in TOF with only a tiny transannular incision and a stiff Dacron patch to inhibit pulmonary annulus extension throughout the normal growing phase produces the same long-term benefits as preserving the full pulmonary annulus integrity.<sup id="cite_ref-Bové_2017_68-6" class="reference"><a href="#cite_note-Bové_2017-68"><span class="cite-bracket">[</span>68<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-van_der_Ven_2019_69-6" class="reference"><a href="#cite_note-van_der_Ven_2019-69"><span class="cite-bracket">[</span>69<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Complications">Complications</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=17" title="Edit section: Complications"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="mw-heading mw-heading3"><h3 id="Short-term">Short-term</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=18" title="Edit section: Short-term"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Residual ventricular septal defects and persistent right ventricular outflow blockage are common problems in the immediate postoperative period. Arrhythmias such as ventricular tachycardia, atrial fibrillation/flutter, and intra-atrial re-entrant tachycardia can occur after tetralogy repair.<sup id="cite_ref-Diaz-Frias_2021_9-3" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> With broad complex tachycardia, the ECG will likely show a right bundle branch block or left bundle branch block patterns. Patients who have had their hearts repaired may experience sudden cardiac death. Risk factors for abnormal heart rhythms include: </p> <ul><li>Age (at repair)<sup id="cite_ref-Diaz-Frias_2021_9-4" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup></li> <li>Male gender<sup id="cite_ref-Diaz-Frias_2021_9-5" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup></li> <li>Transient complete heart block beyond post operative day three<sup id="cite_ref-Diaz-Frias_2021_9-6" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup></li> <li>QRS duration greater than 180 milliseconds<sup id="cite_ref-Diaz-Frias_2021_9-7" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup></li></ul> <div class="mw-heading mw-heading3"><h3 id="Long-term">Long-term</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=19" title="Edit section: Long-term"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Adult patients with congenital cardiac disease are on the rise at a rate of about 5% per year, outpacing the pediatric population.<sup id="cite_ref-Diaz-Frias_2021_9-8" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> Right ventricular volume overload form pulmonary insufficiency, right ventricular aneurysm from outflow patch or ventriculotomy, distal pulmonary artery obstruction, ventricular hypertrophy, chamber enlargement, biventricular dysfunction, and aortic root dilation and insufficient are all long-term complications seen in these patients.<sup id="cite_ref-Diaz-Frias_2021_9-9" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> Arrhythmia, heart failure, and complications from reoperations are the three primary causes of death in individuals with corrected tetralogy of Fallot. QRS duration greater than 180 milliseconds, older age at repair (greater than three years), significant pulmonary valve or tricuspid valve regurgitation, history of syncope, multifocal premature ventricular contractions, and ventricular tachycardia are some of the factors associated with sudden death after 30 years of procedure.<sup id="cite_ref-Singab_2021_70-0" class="reference"><a href="#cite_note-Singab_2021-70"><span class="cite-bracket">[</span>70<span class="cite-bracket">]</span></a></sup> Pulmonary insufficiency is the most common reason for reoperation, and pulmonary valve replacement criteria have traditionally been based on the severity of the regurgitant fraction on a magnetic resonance or CT scan. Right and left ventricular end-systolic and end-diastolic volume indices, ejection fractions, and the existence of aneurysm generating obstructive outflow are all parameters seen in this research. Exercise intolerance, heart failure signs and symptoms, syncope, and prolonged ventricular tachycardia are all possible symptoms. A transcatheter pulmonary valve method can also be used to replace a pulmonary valve.<sup id="cite_ref-Singab_2021_70-1" class="reference"><a href="#cite_note-Singab_2021-70"><span class="cite-bracket">[</span>70<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Pregnancy">Pregnancy</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=20" title="Edit section: Pregnancy"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>In comparison to the general obstetric population, women who had their tetralogy of Fallot repaired completely have similar outcomes.<sup id="cite_ref-Singh_2021_71-0" class="reference"><a href="#cite_note-Singh_2021-71"><span class="cite-bracket">[</span>71<span class="cite-bracket">]</span></a></sup> The degree of pulmonary regurgitation with right or left ventricular dysfunction, as well as the level of pulmonary hypertension, are linked to an increased risk of pregnancy complications.<sup id="cite_ref-Singh_2021_71-1" class="reference"><a href="#cite_note-Singh_2021-71"><span class="cite-bracket">[</span>71<span class="cite-bracket">]</span></a></sup> Fetal death is more likely in women who have moderate right ventricular hypertension or who have undergone a palliative shunt. In comparison to 0.8% of the general population, offspring of women with tetralogy have a 3–5% chance of developing congenital cardiac disease. If the 22q11 deletion is present, there is a 50% chance of transferring the damaged chromosome, with a high risk of a congenital cardiac abnormality.<sup id="cite_ref-Singh_2021_71-2" class="reference"><a href="#cite_note-Singh_2021-71"><span class="cite-bracket">[</span>71<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Prognosis">Prognosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=21" title="Edit section: Prognosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Untreated, tetralogy of Fallot rapidly results in progressive <a href="/wiki/Right_ventricular_hypertrophy" title="Right ventricular hypertrophy">right ventricular hypertrophy</a> due to the increased resistance caused by narrowing of the pulmonary trunk.<sup id="cite_ref-Munoz_2010_20-16" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 199">: 199 </span></sup> This progresses to <a href="/wiki/Heart_failure" title="Heart failure">heart failure</a> which begins in the right ventricle and often leads to left heart failure and <a href="/wiki/Dilated_cardiomyopathy" title="Dilated cardiomyopathy">dilated cardiomyopathy</a>. Mortality rate depends on the severity of the tetralogy of Fallot. If left untreated, TOF carries a 35% mortality rate in the first year of life, and a 50% mortality rate in the first three years of life.<sup id="cite_ref-Chessa_2012_61-1" class="reference"><a href="#cite_note-Chessa_2012-61"><span class="cite-bracket">[</span>61<span class="cite-bracket">]</span></a></sup> Patients with untreated TOF rarely progress to adulthood.<sup id="cite_ref-Chessa_2012_61-2" class="reference"><a href="#cite_note-Chessa_2012-61"><span class="cite-bracket">[</span>61<span class="cite-bracket">]</span></a></sup> </p><p>Patients who have undergone total surgical repair of tetralogy of Fallot have improved hemodynamics and often have good to excellent cardiac function after the operation with some to no exercise intolerance (New York Heart Association Class I-II).<sup id="cite_ref-Cobanoglu_2002_72-0" class="reference"><a href="#cite_note-Cobanoglu_2002-72"><span class="cite-bracket">[</span>72<span class="cite-bracket">]</span></a></sup> Long-term outcome is usually excellent for most patients, however residual post-surgical defects such as pulmonary regurgitation, pulmonary artery stenosis, residual VSD, right ventricular dysfunction, right ventricular outflow tract obstruction may affect life expectancy and increase the need for reoperation.<sup id="cite_ref-Munoz_2010_20-17" class="reference"><a href="#cite_note-Munoz_2010-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 205">: 205 </span></sup> </p><p>Cardiovascular and cerebrovascular complications in patients with repaired CHD such as TOF occur earlier in life compared to healthy subjects.<sup id="cite_ref-73" class="reference"><a href="#cite_note-73"><span class="cite-bracket">[</span>73<span class="cite-bracket">]</span></a></sup> Chronic pulmonary regurgitation and right ventricular dilation and dysfunction is also common.<sup id="cite_ref-74" class="reference"><a href="#cite_note-74"><span class="cite-bracket">[</span>74<span class="cite-bracket">]</span></a></sup> </p><p>Within 30 years after correction, 50% of patients will require reoperation.<sup id="cite_ref-Chessa_2012_61-3" class="reference"><a href="#cite_note-Chessa_2012-61"><span class="cite-bracket">[</span>61<span class="cite-bracket">]</span></a></sup> The most common cause of reoperation is a leaky pulmonary valve (<a href="/wiki/Pulmonary_insufficiency" class="mw-redirect" title="Pulmonary insufficiency">pulmonary valve insufficiency</a>).<sup id="cite_ref-Chessa_2012_61-4" class="reference"><a href="#cite_note-Chessa_2012-61"><span class="cite-bracket">[</span>61<span class="cite-bracket">]</span></a></sup> This is usually corrected with a procedure called <a href="/wiki/Valve_replacement" title="Valve replacement">pulmonary valve replacement</a>.<sup id="cite_ref-Francois_2016_24-6" class="reference"><a href="#cite_note-Francois_2016-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 136">: 136 </span></sup> </p><p>One common prognostic factor with TOF is the development of <a href="/wiki/Ischemia-reperfusion_injury" class="mw-redirect" title="Ischemia-reperfusion injury">ischemia reperfusion injury</a>. Insufficient myocardial protection is considered one of the main causes of death in the correction of TOF.<sup id="cite_ref-75" class="reference"><a href="#cite_note-75"><span class="cite-bracket">[</span>75<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-76" class="reference"><a href="#cite_note-76"><span class="cite-bracket">[</span>76<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Comorbidities">Comorbidities</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=22" title="Edit section: Comorbidities"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>There are many <a href="/wiki/Comorbidity" title="Comorbidity">comorbid</a> conditions that can occur with TOF that may exacerbate the condition. Often, TOF can present with low birth weight and prematurity. In both of these cases, mortality and morbidity were both seen to increase.<sup id="cite_ref-77" class="reference"><a href="#cite_note-77"><span class="cite-bracket">[</span>77<span class="cite-bracket">]</span></a></sup> Differences in right atrial and ventricular mechanics and liver stiffness was also observed in adults with repaired TOF, as well as pulmonary atresia and persistent pulmonary stenosis.<sup id="cite_ref-78" class="reference"><a href="#cite_note-78"><span class="cite-bracket">[</span>78<span class="cite-bracket">]</span></a></sup> In patients with pulmonary atresia, there is complete failure of forward flow from the right ventricle to the pulmonary arterial vasculature. As such, pulmonary blood flow is entirely dependent on shunting from the systemic circulation, typically through a patent ductus arteriosus. The pathophysiology of TOF together with pulmonary arteriosus is uniquely attributable to defects of the pulmonary arteries. Even after operative care, these patients remain at higher risk for pulmonary arterial stenoses and <a href="/wiki/Pulmonary_hypertension" title="Pulmonary hypertension">pulmonary hypertension</a>.<sup id="cite_ref-79" class="reference"><a href="#cite_note-79"><span class="cite-bracket">[</span>79<span class="cite-bracket">]</span></a></sup> </p><p><a href="/wiki/Danon_disease" title="Danon disease">Danon disease</a>, which is a rare genetic disorder, was also observed to complicate TOF. In particular, elongation of the <a href="/wiki/QRS_complex" title="QRS complex">QRS complex</a> and a shortened <a href="/wiki/PR_interval" title="PR interval">PR interval</a>. Genetic abnormalities found in TOF may lead to the earlier diagnosis of Danon disease, helping to improve prognostic outcomes.<sup id="cite_ref-80" class="reference"><a href="#cite_note-80"><span class="cite-bracket">[</span>80<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Epidemiology">Epidemiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=23" title="Edit section: Epidemiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The prevalence of tetralogy of Fallot is estimated to be 0.02–0.04%, which corresponds to approximately 200 to 400 cases per million live births.<sup id="cite_ref-pmid152344302_81-0" class="reference"><a href="#cite_note-pmid152344302-81"><span class="cite-bracket">[</span>81<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-1" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> It accounts for 7–10% of all congenital heart abnormalities, making it the most common cyanotic heart defect.<sup id="cite_ref-Roos-Hesselink_2017_5-5" class="reference"><a href="#cite_note-Roos-Hesselink_2017-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 100–101">: 100–101 </span></sup> Although males and females were initially believed to be affected equally, more recent studies have shown TOF affects males more than females.<sup id="cite_ref-NIH2011What2_82-0" class="reference"><a href="#cite_note-NIH2011What2-82"><span class="cite-bracket">[</span>82<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-2" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> About 1 in 100 newborns is diagnosed with a congential heart defect, of which 10% are diagnosed with TOF.<sup id="cite_ref-NORD_12-3" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Genetically, it is most commonly associated with Down syndrome and DiGeorge syndrome.<sup id="cite_ref-Roos-Hesselink_2017_5-6" class="reference"><a href="#cite_note-Roos-Hesselink_2017-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Francois_2016_24-7" class="reference"><a href="#cite_note-Francois_2016-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup> Down syndrome and other chromosomal disorders are known to occur alongside congential heart defects such as TOF.<sup id="cite_ref-NORD_12-4" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="History">History</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=24" title="Edit section: History"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Tetralogy of Fallot was initially described in 1671 by the Danish researcher <a href="/wiki/Nicolas_Steno" title="Nicolas Steno">Niels Steensen</a>.<sup id="cite_ref-Name2016_1-3" class="reference"><a href="#cite_note-Name2016-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-VanP2009_15-1" class="reference"><a href="#cite_note-VanP2009-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> Also referenced as Nicolaus Steno in Latin, Stensen was a pioneer in anatomy and geology, his work making significant specific contribution to the fields of cardiac anatomy and pathology.<sup id="cite_ref-Diaz-Frias_2021_9-10" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> A further description was published in 1888 by the French physician <a href="/wiki/Etienne_Fallot" class="mw-redirect" title="Etienne Fallot">Étienne-Louis Arthur Fallot</a>, after whom it was ultimately named.<sup id="cite_ref-Name2016_1-4" class="reference"><a href="#cite_note-Name2016-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Fallot1888_16-1" class="reference"><a href="#cite_note-Fallot1888-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> In 1924, Maude Elizabeth Seymour Abbott, a pediatric cardiologist from Montreal, Canada, named it tetralogy of Fallot.<sup id="cite_ref-83" class="reference"><a href="#cite_note-83"><span class="cite-bracket">[</span>83<span class="cite-bracket">]</span></a></sup> </p><p>The short paper "Dissection of a Monstrous Foetus in Paris" in 1671 first described the conditions that would later together be known as TOF. In particular, it highlighted the unusual formation of arteries, the stenosing of the pulmonary artery, the absence of the ductus arteriosus, an overriding aorta, and fetal cardiac circulation where blood was redirected to the aorta from the pulmonary artery.<sup id="cite_ref-Diaz-Frias_2021_9-11" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> Over a hundred years later in 1777, Dutch physician <a href="/wiki/Eduard_Sandifort" title="Eduard Sandifort">Eduard Sandifort</a> reported what he referred to as "the blue boy" patient. This patient, who was 16 months old, was initially thought to have <a href="/wiki/Asthma" title="Asthma">asthma</a>, though an autopsy postmortem revealed a cardiac malformation with no ductus arteriosus or ligamentum arteriosum, indicating that the child may have died from TOF.<sup id="cite_ref-Diaz-Frias_2021_9-12" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> Another 13-year-old patient was reported by Scottish physician <a href="/wiki/William_Hunter_(anatomist)" title="William Hunter (anatomist)">William Hunter</a> in 1782. Hunter described the patient, along with three others, as suffering from cyanosis after a posthumous examination in 1774.<sup id="cite_ref-Diaz-Frias_2021_9-13" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> </p><p>Other cases, such as those presented by Pulteney (1785), Abernethy (1793), Bell (1797), Dorsey (1812), and Farre (1814) also contributed to modern understandings of TOF. The first reported case of TOF was in America at the <a href="/wiki/University_of_Pennsylvania" title="University of Pennsylvania">University of Pennsylvania</a> in 1816, with more cases being reported by Peacock (1858 and 1869), Widman (1881), and finally Fallot (1888), after whom the condition is named.<sup id="cite_ref-Diaz-Frias_2021_9-14" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> Fallot was the first to elegantly describe the four key features that differentiate it from other cyanotic cardiac conditions, and was prominent in the disqualification of a <a href="/wiki/Patent_foramen_ovale" class="mw-redirect" title="Patent foramen ovale">patent foramen ovale</a> as a fifth feature. Fallot initially referred to it as "La maladie bleue", which is French for "the blue disease" or "cyanose cardiaque", translating to "cardiac cyanosis".<sup id="cite_ref-Diaz-Frias_2021_9-15" class="reference"><a href="#cite_note-Diaz-Frias_2021-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> </p><p>The first surgical repair was carried out in 1944 at Johns Hopkins.<sup id="cite_ref-84" class="reference"><a href="#cite_note-84"><span class="cite-bracket">[</span>84<span class="cite-bracket">]</span></a></sup> The procedure was conducted by surgeon <a href="/wiki/Alfred_Blalock" title="Alfred Blalock">Alfred Blalock</a> and cardiologist <a href="/wiki/Helen_B._Taussig" title="Helen B. Taussig">Helen B. Taussig</a>, with <a href="/wiki/Vivien_Thomas" title="Vivien Thomas">Vivien Thomas</a> also providing substantial contributions and listed as an assistant.<sup id="cite_ref-War2005_3-3" class="reference"><a href="#cite_note-War2005-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> This first surgery was depicted in the film <i><a href="/wiki/Something_the_Lord_Made" title="Something the Lord Made">Something the Lord Made</a></i>.<sup id="cite_ref-JHMI_63-1" class="reference"><a href="#cite_note-JHMI-63"><span class="cite-bracket">[</span>63<span class="cite-bracket">]</span></a></sup> It was actually Helen Taussig who convinced Alfred Blalock that the shunt was going to work. 15-month-old <a href="/wiki/Eileen_Saxon" title="Eileen Saxon">Eileen Saxon</a> was the first person to receive a <a href="/wiki/Blalock%E2%80%93Taussig_shunt" class="mw-redirect" title="Blalock–Taussig shunt">Blalock–Thomas–Taussig shunt</a>.<sup id="cite_ref-JHMI_63-2" class="reference"><a href="#cite_note-JHMI-63"><span class="cite-bracket">[</span>63<span class="cite-bracket">]</span></a></sup> Furthermore, the Blalock-Thomas-Taussig procedure, initially the only surgical treatment available for tetralogy of Fallot, was palliative but not curative. The first total repair of tetralogy of Fallot was done by a team led by <a href="/wiki/C._Walton_Lillehei" title="C. Walton Lillehei">C. Walton Lillehei</a> at the <a href="/wiki/University_of_Minnesota" title="University of Minnesota">University of Minnesota</a> in 1954 on an 11-year-old boy.<sup id="cite_ref-PMC1465089_85-0" class="reference"><a href="#cite_note-PMC1465089-85"><span class="cite-bracket">[</span>85<span class="cite-bracket">]</span></a></sup> Total repair on infants has had success from 1981, with research indicating that it has a comparatively low mortality rate.<sup id="cite_ref-Cobanoglu_2002_72-1" class="reference"><a href="#cite_note-Cobanoglu_2002-72"><span class="cite-bracket">[</span>72<span class="cite-bracket">]</span></a></sup> Today the adult TOF population continues to grow and is one of the most common congenital heart defect seen in adult outpatient clinics.<sup id="cite_ref-Roos-Hesselink_2017_5-7" class="reference"><a href="#cite_note-Roos-Hesselink_2017-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup class="reference nowrap"><span title="Page / location: 100–101">: 100–101 </span></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Related_disorders">Related disorders</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=25" title="Edit section: Related disorders"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The following illnesses have symptoms that are comparable to tetralogy of Fallot. For a differential diagnosis, comparisons between these disorders provides valuable knowledge. </p> <div class="mw-heading mw-heading3"><h3 id="Atrial_septal_defects">Atrial septal defects</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=26" title="Edit section: Atrial septal defects"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Atrial septal defects (ASDs) are a kind of congenital heart abnormality in which a tiny opening exists between the two atria of the heart.<sup id="cite_ref-Thorne_2011_86-0" class="reference"><a href="#cite_note-Thorne_2011-86"><span class="cite-bracket">[</span>86<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-5" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> The burden on the right side of the heart is increased as a result of these abnormalities, as is the blood flow to the lungs.<sup id="cite_ref-Thorne_2011_86-1" class="reference"><a href="#cite_note-Thorne_2011-86"><span class="cite-bracket">[</span>86<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-6" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> This leads to excessive blood flow to the lungs and an increased workload on the right side of the heart.<sup id="cite_ref-Thorne_2011_86-2" class="reference"><a href="#cite_note-Thorne_2011-86"><span class="cite-bracket">[</span>86<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-7" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Another common finding associated with ASDs is right ventricular hypertrophy, also known as enlargement of the right ventricle.<sup id="cite_ref-NORD_12-8" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Ventricular_septal_defects">Ventricular septal defects</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=27" title="Edit section: Ventricular septal defects"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Ventricular septal defects (VSDs) are a kind of congenital heart abnormality in which one of the ventricles is missing.<sup id="cite_ref-Dakkak_2021_87-0" class="reference"><a href="#cite_note-Dakkak_2021-87"><span class="cite-bracket">[</span>87<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-9" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Two atria and one big ventricle are common in infants with congenital abnormalities.<sup id="cite_ref-Dakkak_2021_87-1" class="reference"><a href="#cite_note-Dakkak_2021-87"><span class="cite-bracket">[</span>87<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-10" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Symptoms of these diseases include an unusually high rate of breathing (tachypnea), a blue hue to the skin (cyanosis), wheezing, a rapid heartbeat (tachycardia), and/or an abnormally enlarged liver, which are similar to those of other congenital heart problems (hepatomegaly).<sup id="cite_ref-Dakkak_2021_87-2" class="reference"><a href="#cite_note-Dakkak_2021-87"><span class="cite-bracket">[</span>87<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-11" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> VSDs can also lead to a build-up of fluid around the heart, which can lead to congestive heart failure.<sup id="cite_ref-Dakkak_2021_87-3" class="reference"><a href="#cite_note-Dakkak_2021-87"><span class="cite-bracket">[</span>87<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-12" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Atrioventricular_septal_defect">Atrioventricular septal defect</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=28" title="Edit section: Atrioventricular septal defect"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Atrioventricular septal defect (AVSD) is an uncommon congenital heart condition characterized by faulty development of the heart's septa and valves.<sup id="cite_ref-Ahmed_2021_88-0" class="reference"><a href="#cite_note-Ahmed_2021-88"><span class="cite-bracket">[</span>88<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-13" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Congestive heart failure is common in infants with the entire version of the condition.<sup id="cite_ref-Ahmed_2021_88-1" class="reference"><a href="#cite_note-Ahmed_2021-88"><span class="cite-bracket">[</span>88<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-14" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Fluid builds up in other parts of the body, particularly the lungs.<sup id="cite_ref-Ahmed_2021_88-2" class="reference"><a href="#cite_note-Ahmed_2021-88"><span class="cite-bracket">[</span>88<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-15" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Breathing difficulties may result from pulmonary congestion (dyspnea).<sup id="cite_ref-Ahmed_2021_88-3" class="reference"><a href="#cite_note-Ahmed_2021-88"><span class="cite-bracket">[</span>88<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-16" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Mitral_valve_stenosis">Mitral valve stenosis</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=29" title="Edit section: Mitral valve stenosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Mitral valve stenosis is an uncommon cardiac abnormality that can occur at birth (congenital) or develop later in life (acquired).<sup id="cite_ref-Shah_2021_89-0" class="reference"><a href="#cite_note-Shah_2021-89"><span class="cite-bracket">[</span>89<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-17" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> The aberrant narrowing of the mitral valve's opening characterizes this condition.<sup id="cite_ref-Shah_2021_89-1" class="reference"><a href="#cite_note-Shah_2021-89"><span class="cite-bracket">[</span>89<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-NORD_12-18" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> There are two versions of this condition known as congenital and acquired characterized by different symptoms.<sup id="cite_ref-NORD_12-19" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Congenital Mitral valve stenosis symptoms include a wide array such as respiratory infections, breathing difficulties, heart palpitations and coughing.<sup id="cite_ref-NORD_12-20" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Acquired mitral valve stenosis symptoms also include a wide array such as consciousness losses, angina, general weakness and abdominal discomfort.<sup id="cite_ref-NORD_12-21" class="reference"><a href="#cite_note-NORD-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Notable_cases">Notable cases</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=30" title="Edit section: Notable cases"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><a href="/wiki/Shaun_White" title="Shaun White">Shaun White</a>,<sup id="cite_ref-Yen_2003_90-0" class="reference"><a href="#cite_note-Yen_2003-90"><span class="cite-bracket">[</span>90<span class="cite-bracket">]</span></a></sup> American professional snowboarder and skateboarder</li> <li><a href="/wiki/Beau_Casson" title="Beau Casson">Beau Casson</a>,<sup id="cite_ref-smh.com.au_91-0" class="reference"><a href="#cite_note-smh.com.au-91"><span class="cite-bracket">[</span>91<span class="cite-bracket">]</span></a></sup> Australian cricketer</li> <li><a href="/wiki/Dennis_McEldowney" title="Dennis McEldowney">Dennis McEldowney</a>,<sup id="cite_ref-92" class="reference"><a href="#cite_note-92"><span class="cite-bracket">[</span>92<span class="cite-bracket">]</span></a></sup> New Zealand author and publisher</li> <li><a href="/wiki/The_Force_(advertisement)" title="The Force (advertisement)">Max Page</a>, Volkswagen's "Little Darth Vader" from the 2011 Super Bowl commercial<sup id="cite_ref-93" class="reference"><a href="#cite_note-93"><span class="cite-bracket">[</span>93<span class="cite-bracket">]</span></a></sup></li> <li>Billy Kimmel, the son of talk show host <a href="/wiki/Jimmy_Kimmel" title="Jimmy Kimmel">Jimmy Kimmel</a>; Billy's diagnosis led Kimmel to discuss access to health care on his show <i><a href="/wiki/Jimmy_Kimmel_Live!" title="Jimmy Kimmel Live!">Jimmy Kimmel Live!</a></i><sup id="cite_ref-94" class="reference"><a href="#cite_note-94"><span class="cite-bracket">[</span>94<span class="cite-bracket">]</span></a></sup></li></ul> <div class="mw-heading mw-heading2"><h2 id="See_also">See also</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=31" title="Edit section: See also"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><a href="/wiki/Trilogy_of_Fallot" title="Trilogy of Fallot">Trilogy of Fallot</a></li> <li><a href="/wiki/Congenital_rubella_syndrome" title="Congenital rubella syndrome">Congenital rubella syndrome</a></li></ul> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=32" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output .reflist-lower-alpha{list-style-type:lower-alpha}.mw-parser-output .reflist-lower-greek{list-style-type:lower-greek}.mw-parser-output .reflist-lower-roman{list-style-type:lower-roman}</style><div class="reflist"> <div class="mw-references-wrap mw-references-columns"><ol class="references"> <li id="cite_note-Name2016-1"><span class="mw-cite-backlink">^ <a href="#cite_ref-Name2016_1-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Name2016_1-1"><sup><i><b>b</b></i></sup></a> <a href="#cite_ref-Name2016_1-2"><sup><i><b>c</b></i></sup></a> <a href="#cite_ref-Name2016_1-3"><sup><i><b>d</b></i></sup></a> <a href="#cite_ref-Name2016_1-4"><sup><i><b>e</b></i></sup></a></span> <span class="reference-text"><style data-mw-deduplicate="TemplateStyles:r1238218222">.mw-parser-output cite.citation{font-style:inherit;word-wrap:break-word}.mw-parser-output .citation q{quotes:"\"""\"""'""'"}.mw-parser-output .citation:target{background-color:rgba(0,127,255,0.133)}.mw-parser-output .id-lock-free.id-lock-free a{background:url("//upload.wikimedia.org/wikipedia/commons/6/65/Lock-green.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-limited.id-lock-limited a,.mw-parser-output .id-lock-registration.id-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/d/d6/Lock-gray-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-subscription.id-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/a/aa/Lock-red-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/4/4c/Wikisource-logo.svg")right 0.1em center/12px no-repeat}body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-free a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-limited a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-registration a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-subscription a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .cs1-ws-icon a{background-size:contain;padding:0 1em 0 0}.mw-parser-output .cs1-code{color:inherit;background:inherit;border:none;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;color:var(--color-error,#d33)}.mw-parser-output .cs1-visible-error{color:var(--color-error,#d33)}.mw-parser-output .cs1-maint{display:none;color:#085;margin-left:0.3em}.mw-parser-output .cs1-kern-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right{padding-right:0.2em}.mw-parser-output .citation .mw-selflink{font-weight:inherit}@media screen{.mw-parser-output .cs1-format{font-size:95%}html.skin-theme-clientpref-night .mw-parser-output .cs1-maint{color:#18911f}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .cs1-maint{color:#18911f}}</style><cite id="CITEREFLehn" class="citation web cs1">Lehn M. <a rel="nofollow" class="external text" href="http://www.whonamedit.com/synd.cfm/2281.html">"Fallot's tetralogy"</a>. <i>Whonamedit?</i>. <a rel="nofollow" class="external text" href="https://web.archive.org/web/20161003083817/http://www.whonamedit.com/synd.cfm/2281.html">Archived</a> from the original on 3 October 2016<span class="reference-accessdate">. Retrieved <span class="nowrap">2 October</span> 2016</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=Whonamedit%3F&rft.atitle=Fallot%27s+tetralogy&rft.aulast=Lehn&rft.aufirst=M&rft_id=http%3A%2F%2Fwww.whonamedit.com%2Fsynd.cfm%2F2281.html&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-NIH2011Sym-2"><span class="mw-cite-backlink">^ <a href="#cite_ref-NIH2011Sym_2-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-NIH2011Sym_2-1"><sup><i><b>b</b></i></sup></a> <a href="#cite_ref-NIH2011Sym_2-2"><sup><i><b>c</b></i></sup></a> <a href="#cite_ref-NIH2011Sym_2-3"><sup><i><b>d</b></i></sup></a> <a href="#cite_ref-NIH2011Sym_2-4"><sup><i><b>e</b></i></sup></a> <a href="#cite_ref-NIH2011Sym_2-5"><sup><i><b>f</b></i></sup></a> <a href="#cite_ref-NIH2011Sym_2-6"><sup><i><b>g</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite class="citation web cs1"><a rel="nofollow" class="external text" href="http://www.nhlbi.nih.gov/health/health-topics/topics/tof/signs">"What Are the Signs and Symptoms of Tetralogy of Fallot?"</a>. <i>NHLBI</i>. 1 July 2011. <a rel="nofollow" class="external text" href="https://web.archive.org/web/20161005191311/http://www.nhlbi.nih.gov/health/health-topics/topics/tof/signs">Archived</a> from the original on 5 October 2016<span class="reference-accessdate">. Retrieved <span class="nowrap">2 October</span> 2016</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=NHLBI&rft.atitle=What+Are+the+Signs+and+Symptoms+of+Tetralogy+of+Fallot%3F&rft.date=2011-07-01&rft_id=http%3A%2F%2Fwww.nhlbi.nih.gov%2Fhealth%2Fhealth-topics%2Ftopics%2Ftof%2Fsigns&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-War2005-3"><span class="mw-cite-backlink">^ <a href="#cite_ref-War2005_3-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-War2005_3-1"><sup><i><b>b</b></i></sup></a> <a href="#cite_ref-War2005_3-2"><sup><i><b>c</b></i></sup></a> <a href="#cite_ref-War2005_3-3"><sup><i><b>d</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFWarnes2005" class="citation journal cs1">Warnes CA (July 2005). <a rel="nofollow" class="external text" href="https://doi.org/10.1016%2Fj.jacc.2005.02.083">"The adult with congenital heart disease: born to be bad?"</a>. <i>Journal of the American College of Cardiology</i>. <b>46</b> (1): 1–8. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1016%2Fj.jacc.2005.02.083">10.1016/j.jacc.2005.02.083</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/15992627">15992627</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Journal+of+the+American+College+of+Cardiology&rft.atitle=The+adult+with+congenital+heart+disease%3A+born+to+be+bad%3F&rft.volume=46&rft.issue=1&rft.pages=1-8&rft.date=2005-07&rft_id=info%3Adoi%2F10.1016%2Fj.jacc.2005.02.083&rft_id=info%3Apmid%2F15992627&rft.aulast=Warnes&rft.aufirst=CA&rft_id=https%3A%2F%2Fdoi.org%2F10.1016%252Fj.jacc.2005.02.083&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-NIH2011What-4"><span class="mw-cite-backlink">^ <a href="#cite_ref-NIH2011What_4-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-NIH2011What_4-1"><sup><i><b>b</b></i></sup></a> <a href="#cite_ref-NIH2011What_4-2"><sup><i><b>c</b></i></sup></a> <a href="#cite_ref-NIH2011What_4-3"><sup><i><b>d</b></i></sup></a> <a href="#cite_ref-NIH2011What_4-4"><sup><i><b>e</b></i></sup></a> <a href="#cite_ref-NIH2011What_4-5"><sup><i><b>f</b></i></sup></a> <a href="#cite_ref-NIH2011What_4-6"><sup><i><b>g</b></i></sup></a> <a href="#cite_ref-NIH2011What_4-7"><sup><i><b>h</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite class="citation web cs1"><a rel="nofollow" class="external text" href="http://www.nhlbi.nih.gov/health/health-topics/topics/tof/">"What Is Tetralogy of Fallot?"</a>. <i>NHLBI</i>. 1 July 2011. <a rel="nofollow" class="external text" href="https://web.archive.org/web/20161004225713/http://www.nhlbi.nih.gov/health/health-topics/topics/tof">Archived</a> from the original on 4 October 2016<span class="reference-accessdate">. Retrieved <span class="nowrap">2 October</span> 2016</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=NHLBI&rft.atitle=What+Is+Tetralogy+of+Fallot%3F&rft.date=2011-07-01&rft_id=http%3A%2F%2Fwww.nhlbi.nih.gov%2Fhealth%2Fhealth-topics%2Ftopics%2Ftof%2F&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-Roos-Hesselink_2017-5"><span class="mw-cite-backlink">^ <a href="#cite_ref-Roos-Hesselink_2017_5-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Roos-Hesselink_2017_5-1"><sup><i><b>b</b></i></sup></a> <a href="#cite_ref-Roos-Hesselink_2017_5-2"><sup><i><b>c</b></i></sup></a> <a href="#cite_ref-Roos-Hesselink_2017_5-3"><sup><i><b>d</b></i></sup></a> <a href="#cite_ref-Roos-Hesselink_2017_5-4"><sup><i><b>e</b></i></sup></a> <a href="#cite_ref-Roos-Hesselink_2017_5-5"><sup><i><b>f</b></i></sup></a> <a href="#cite_ref-Roos-Hesselink_2017_5-6"><sup><i><b>g</b></i></sup></a> <a href="#cite_ref-Roos-Hesselink_2017_5-7"><sup><i><b>h</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFRoos-HesselinkJohnson2017" class="citation book cs1">Roos-Hesselink JW, Johnson MR (2017). <i>Pregnancy and congenital heart disease</i>. Cham: Springer. p. 62. <a href="/wiki/ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <a href="/wiki/Special:BookSources/9783319389134" title="Special:BookSources/9783319389134"><bdi>9783319389134</bdi></a>. <a href="/wiki/OCLC_(identifier)" class="mw-redirect" title="OCLC (identifier)">OCLC</a> <a rel="nofollow" class="external text" href="https://search.worldcat.org/oclc/969644876">969644876</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=book&rft.btitle=Pregnancy+and+congenital+heart+disease&rft.place=Cham&rft.pages=62&rft.pub=Springer&rft.date=2017&rft_id=info%3Aoclcnum%2F969644876&rft.isbn=9783319389134&rft.aulast=Roos-Hesselink&rft.aufirst=JW&rft.au=Johnson%2C+MR&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-NIH2011Diag-6"><span class="mw-cite-backlink"><b><a href="#cite_ref-NIH2011Diag_6-0">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://www.nhlbi.nih.gov/health/health-topics/topics/tof/diagnosis">"How Is Tetralogy of Fallot Diagnosed?"</a>. <i>NHLBI</i>. 1 July 2011. <a rel="nofollow" class="external text" href="https://web.archive.org/web/20170429160101/https://www.nhlbi.nih.gov/health/health-topics/topics/tof/diagnosis">Archived</a> from the original on 29 April 2017<span class="reference-accessdate">. Retrieved <span class="nowrap">7 May</span> 2017</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=NHLBI&rft.atitle=How+Is+Tetralogy+of+Fallot+Diagnosed%3F&rft.date=2011-07-01&rft_id=https%3A%2F%2Fwww.nhlbi.nih.gov%2Fhealth%2Fhealth-topics%2Ftopics%2Ftof%2Fdiagnosis&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-7"><span class="mw-cite-backlink"><b><a href="#cite_ref-7">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFPrasadKahanMohan2007" class="citation book cs1">Prasad R, Kahan S, Mohan P (2007). <a rel="nofollow" class="external text" href="https://books.google.com/books?id=7ODciw8IIF8C&pg=PA134"><i>In a Page: Cardiology</i></a>. Lippincott Williams & Wilkins. <a href="/wiki/ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <a href="/wiki/Special:BookSources/9780781764964" title="Special:BookSources/9780781764964"><bdi>9780781764964</bdi></a>. <a rel="nofollow" class="external text" href="https://web.archive.org/web/20210515074550/https://books.google.com/books?id=7ODciw8IIF8C&pg=PA134">Archived</a> from the original on 2021-05-15<span class="reference-accessdate">. Retrieved <span class="nowrap">2017-09-15</span></span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=book&rft.btitle=In+a+Page%3A+Cardiology&rft.pub=Lippincott+Williams+%26+Wilkins&rft.date=2007&rft.isbn=9780781764964&rft.aulast=Prasad&rft.aufirst=R&rft.au=Kahan%2C+S&rft.au=Mohan%2C+P&rft_id=https%3A%2F%2Fbooks.google.com%2Fbooks%3Fid%3D7ODciw8IIF8C%26pg%3DPA134&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-NIH2011Tx-8"><span class="mw-cite-backlink">^ <a href="#cite_ref-NIH2011Tx_8-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-NIH2011Tx_8-1"><sup><i><b>b</b></i></sup></a> <a href="#cite_ref-NIH2011Tx_8-2"><sup><i><b>c</b></i></sup></a> <a href="#cite_ref-NIH2011Tx_8-3"><sup><i><b>d</b></i></sup></a> <a href="#cite_ref-NIH2011Tx_8-4"><sup><i><b>e</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite class="citation web cs1"><a rel="nofollow" class="external text" href="http://www.nhlbi.nih.gov/health/health-topics/topics/tof/treatment">"How Is Tetralogy of Fallot Treated?"</a>. <i>NHLBI</i>. July 1, 2011. <a rel="nofollow" class="external text" href="https://web.archive.org/web/20161005191332/http://www.nhlbi.nih.gov/health/health-topics/topics/tof/treatment">Archived</a> from the original on 5 October 2016<span class="reference-accessdate">. Retrieved <span class="nowrap">2 October</span> 2016</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=NHLBI&rft.atitle=How+Is+Tetralogy+of+Fallot+Treated%3F&rft.date=2011-07-01&rft_id=http%3A%2F%2Fwww.nhlbi.nih.gov%2Fhealth%2Fhealth-topics%2Ftopics%2Ftof%2Ftreatment&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-Diaz-Frias_2021-9"><span class="mw-cite-backlink">^ <a href="#cite_ref-Diaz-Frias_2021_9-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-1"><sup><i><b>b</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-2"><sup><i><b>c</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-3"><sup><i><b>d</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-4"><sup><i><b>e</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-5"><sup><i><b>f</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-6"><sup><i><b>g</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-7"><sup><i><b>h</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-8"><sup><i><b>i</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-9"><sup><i><b>j</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-10"><sup><i><b>k</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-11"><sup><i><b>l</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-12"><sup><i><b>m</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-13"><sup><i><b>n</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-14"><sup><i><b>o</b></i></sup></a> <a href="#cite_ref-Diaz-Frias_2021_9-15"><sup><i><b>p</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFDiaz-FriasGuillaume2021" class="citation book cs1">Diaz-Frias J, Guillaume M (2021). <a rel="nofollow" class="external text" href="http://www.ncbi.nlm.nih.gov/books/NBK513288/">"Tetralogy of Fallot"</a>. <i>StatPearls</i>. Treasure Island (FL): StatPearls Publishing. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/30020660">30020660</a>. <a rel="nofollow" class="external text" href="https://web.archive.org/web/20200812103313/https://www.ncbi.nlm.nih.gov/books/NBK513288/">Archived</a> from the original on 2020-08-12<span class="reference-accessdate">. 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Retrieved <span class="nowrap">2009-01-02</span></span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=The+Sydney+Morning+Herald&rft.atitle=New+twist+in+Casson%27s+amazing+journey+%E2%80%93+Cricket+%E2%80%93+Sport&rft.date=2008-06-06&rft.aulast=Brown&rft.aufirst=A&rft.au=Saltau%2C+C&rft_id=http%3A%2F%2Fwww.smh.com.au%2Fnews%2Fcricket%2Fnew-twist-in-cassons-amazing-journey%2F2008%2F06%2F05%2F1212259004804.html&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-92"><span class="mw-cite-backlink"><b><a href="#cite_ref-92">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://web.archive.org/web/20081016082559/http://www.bookcouncil.org.nz/writers/mceldowney.html">"McEldowney, Dennis"</a>. <i>New Zealand Book Council</i>. Archived from <a rel="nofollow" class="external text" href="http://www.bookcouncil.org.nz/writers/mceldowney.html">the original</a> on 2008-10-16<span class="reference-accessdate">. Retrieved <span class="nowrap">2009-02-28</span></span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=New+Zealand+Book+Council&rft.atitle=McEldowney%2C+Dennis&rft_id=http%3A%2F%2Fwww.bookcouncil.org.nz%2Fwriters%2Fmceldowney.html&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-93"><span class="mw-cite-backlink"><b><a href="#cite_ref-93">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFInbar2011" class="citation web cs1">Inbar M (7 February 2011). <a rel="nofollow" class="external text" href="https://web.archive.org/web/20130825162531/http://www.nbcnews.com/id/41455377/">"<span class="cs1-kern-left"></span>'Little Darth Vader' reveals face behind the Force"</a>. <i><a href="/wiki/NBC_News" title="NBC News">NBC News</a></i>. Archived from <a rel="nofollow" class="external text" href="http://www.nbcnews.com/id/41455377">the original</a> on 25 August 2013<span class="reference-accessdate">. Retrieved <span class="nowrap">17 November</span> 2019</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=NBC+News&rft.atitle=%27Little+Darth+Vader%27+reveals+face+behind+the+Force&rft.date=2011-02-07&rft.aulast=Inbar&rft.aufirst=M&rft_id=http%3A%2F%2Fwww.nbcnews.com%2Fid%2F41455377&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> <li id="cite_note-94"><span class="mw-cite-backlink"><b><a href="#cite_ref-94">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFMcCluskey2017" class="citation magazine cs1">McCluskey M (26 December 2017). <a rel="nofollow" class="external text" href="https://time.com/5072384/jimmy-kimmel-health-care-politics-timeline/">"Aide to All the Times Jimmy Kimmel Has Gotten Political"</a>. <i>Time</i>. <a rel="nofollow" class="external text" href="https://web.archive.org/web/20180227211956/http://time.com/5072384/jimmy-kimmel-health-care-politics-timeline/">Archived</a> from the original on 2018-02-27<span class="reference-accessdate">. Retrieved <span class="nowrap">2018-02-23</span></span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Time&rft.atitle=Aide+to+All+the+Times+Jimmy+Kimmel+Has+Gotten+Political&rft.date=2017-12-26&rft.aulast=McCluskey&rft.aufirst=M&rft_id=https%3A%2F%2Ftime.com%2F5072384%2Fjimmy-kimmel-health-care-politics-timeline%2F&rfr_id=info%3Asid%2Fen.wikipedia.org%3ATetralogy+of+Fallot" class="Z3988"></span></span> </li> </ol></div></div> <div class="mw-heading mw-heading2"><h2 id="External_links">External links</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Tetralogy_of_Fallot&action=edit&section=33" title="Edit section: External links"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1235681985">.mw-parser-output .side-box{margin:4px 0;box-sizing:border-box;border:1px solid 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data-file-height="1376" /></span></span></div> <div class="side-box-text plainlist">Wikimedia Commons has media related to <span style="font-weight: bold; font-style: italic;"><a href="https://commons.wikimedia.org/wiki/Category:Tetralogy_of_Fallot" class="extiw" title="commons:Category:Tetralogy of Fallot">Tetralogy of Fallot</a></span>.</div></div> </div> <ul><li><a rel="nofollow" class="external text" href="https://www.nhlbi.nih.gov/health/congenital-heart-defects/types">What Is Tetralogy of Fallot?</a> at the <a href="/wiki/National_Institutes_of_Health" title="National Institutes of Health">National Institutes of Health</a></li> <li><a rel="nofollow" class="external text" href="https://www.bhf.org.uk/~/media/files/publications/children-and-young-people/c5understanding-your-childs-heart-tetralogy-of-fallot1208.pdf">Understanding your child's heart: Tetralogy of Fallot</a> by the <a href="/wiki/British_Heart_Foundation" title="British Heart Foundation">British Heart 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style="width:1%;background: #EAECF0;color:black;">Classification</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><div style="position:relative; float:right; font-size:0.8em;"><a href="https://www.wikidata.org/wiki/Q1126831" class="extiw" title="d:Q1126831">D</a></div><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/ICD-11" title="ICD-11">11</a></b>: <a rel="nofollow" class="external text" href="https://icd.who.int/browse/latest-release/mms/en#90973426">LA88.2</a></li><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/ICD-10" title="ICD-10">10</a></b>: <a rel="nofollow" class="external text" href="https://icd.who.int/browse10/2019/en#/Q21.3">Q21.3</a></li><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/List_of_ICD-9_codes" title="List of ICD-9 codes">9-CM</a></b>: <a rel="nofollow" class="external text" href="http://www.icd9data.com/getICD9Code.ashx?icd9=745.2">745.2</a></li><li><b><a href="/wiki/Online_Mendelian_Inheritance_in_Man" title="Online Mendelian Inheritance in Man">OMIM</a></b>: <a rel="nofollow" class="external text" href="https://omim.org/entry/187500">187500</a></li><li><b><a href="/wiki/Medical_Subject_Headings" title="Medical Subject Headings">MeSH</a></b>: <a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?ui=D013771">D013771</a></li><li><b><a href="/wiki/Diseases_Database" title="Diseases Database">DiseasesDB</a></b>: <a rel="nofollow" class="external text" href="http://www.diseasesdatabase.com/ddb4660.htm">4660</a></li></ul></div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">External resources</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/MedlinePlus" title="MedlinePlus">MedlinePlus</a></b>: <a rel="nofollow" class="external text" href="https://www.nlm.nih.gov/medlineplus/ency/article/001567.htm">001567</a></li><li><b><a href="/wiki/EMedicine" title="EMedicine">eMedicine</a></b>: <a rel="nofollow" class="external text" href="https://emedicine.medscape.com/emerg/575-overview">emerg/575</a></li><li><b><a href="/wiki/Patient_UK" title="Patient UK">Patient UK</a></b>: <a rel="nofollow" class="external text" href="https://patient.info/doctor/fallots-tetralogy">Tetralogy of Fallot</a></li><li><b><a href="/wiki/Orphanet" title="Orphanet">Orphanet</a></b>: <a rel="nofollow" class="external text" href="https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=3303">3303</a></li></ul></div></div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Congenital_heart_defects" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><style 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heart defects</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Heart_septal_defect" title="Heart septal defect">Heart septal defect</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Aortopulmonary_septal_defect" title="Aortopulmonary septal defect">Aortopulmonary septal defect</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Double_outlet_right_ventricle" title="Double outlet right ventricle">Double outlet right ventricle</a> <ul><li><a href="/wiki/Taussig%E2%80%93Bing_syndrome" title="Taussig–Bing syndrome">Taussig–Bing syndrome</a></li></ul></li> <li><a href="/wiki/Transposition_of_the_great_vessels" title="Transposition of the great vessels">Transposition of the great vessels</a> <ul><li><a href="/wiki/Dextro-Transposition_of_the_great_arteries" title="Dextro-Transposition of the great arteries">dextro</a></li> <li><a href="/wiki/Levo-Transposition_of_the_great_arteries" title="Levo-Transposition of the great arteries">levo</a></li></ul></li> <li><a href="/wiki/Persistent_truncus_arteriosus" title="Persistent truncus arteriosus">Persistent truncus arteriosus</a></li></ul> <ul><li><a href="/wiki/Aortopulmonary_window" title="Aortopulmonary window">Aortopulmonary window</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Atrial_septal_defect" title="Atrial septal defect">Atrial septal defect</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Sinus_venosus_atrial_septal_defect" title="Sinus venosus atrial septal defect">Sinus venosus atrial septal defect</a></li> <li><a href="/wiki/Lutembacher%27s_syndrome" title="Lutembacher's syndrome">Lutembacher's syndrome</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Ventricular_septal_defect" title="Ventricular septal defect">Ventricular septal defect</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a class="mw-selflink selflink">Tetralogy of Fallot</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Atrioventricular_septal_defect" title="Atrioventricular septal defect">Atrioventricular septal defect</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Ostium_primum_atrial_septal_defect" title="Ostium primum atrial septal defect">Ostium primum</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Consequences</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Cardiac_shunt" title="Cardiac shunt">Cardiac shunt</a> <ul><li><a href="/wiki/Cyanotic_heart_disease" class="mw-redirect" title="Cyanotic heart disease">Cyanotic heart disease</a></li></ul></li> <li><a href="/wiki/Eisenmenger_syndrome" title="Eisenmenger syndrome">Eisenmenger syndrome</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Valvular_heart_disease" title="Valvular heart disease">Valvular heart disease</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%">Right</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Pulmonary_valve" title="Pulmonary valve">pulmonary valves</a> <ul><li><a href="/wiki/Pulmonary_valve_stenosis" title="Pulmonary valve stenosis">stenosis</a></li> <li><a href="/wiki/Pulmonary_valve_insufficiency" class="mw-redirect" title="Pulmonary valve insufficiency">insufficiency</a></li> <li><a href="/wiki/Absent_pulmonary_valve_syndrome" title="Absent pulmonary valve syndrome">absence</a></li></ul></li> <li><a href="/wiki/Tricuspid_valve" title="Tricuspid valve">tricuspid valves</a> <ul><li><a href="/wiki/Tricuspid_atresia" title="Tricuspid atresia">atresia</a></li> <li><a href="/wiki/Tricuspid_regurgitation" title="Tricuspid regurgitation">regurgitation</a></li> <li><a href="/wiki/Tricuspid_valve_stenosis" title="Tricuspid valve stenosis">stenosis</a></li> <li><a href="/wiki/Ebstein%27s_anomaly" title="Ebstein's anomaly">Ebstein's anomaly</a></li></ul></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Left</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Aortic_valve" title="Aortic valve">aortic valves</a> <ul><li><a href="/wiki/Aortic_valve_stenosis" class="mw-redirect" title="Aortic valve stenosis">stenosis</a></li> <li><a href="/wiki/Aortic_insufficiency" class="mw-redirect" title="Aortic insufficiency">insufficiency</a></li> <li><a href="/wiki/Bicuspid_aortic_valve" title="Bicuspid aortic valve">bicuspid</a></li></ul></li> <li><a href="/wiki/Mitral_valve" title="Mitral valve">mitral valves</a> <ul><li><a href="/wiki/Mitral_stenosis" title="Mitral stenosis">stenosis</a></li> <li><a href="/wiki/Mitral_regurgitation" title="Mitral regurgitation">regurgitation</a></li></ul></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li>Underdeveloped <a href="/wiki/Heart_chamber" class="mw-redirect" title="Heart chamber">heart chambers</a> <ul><li><a href="/wiki/Hypoplastic_right_heart_syndrome" title="Hypoplastic right heart syndrome">right</a></li> <li><a href="/wiki/Hypoplastic_left_heart_syndrome" title="Hypoplastic left heart syndrome">left</a></li> <li><a href="/wiki/Uhl_anomaly" title="Uhl anomaly">Uhl anomaly</a></li></ul></li> <li><a href="/wiki/Dextrocardia" title="Dextrocardia">Dextrocardia</a></li> <li><a href="/wiki/Levocardia" title="Levocardia">Levocardia</a></li> <li><a href="/wiki/Cor_triatriatum" title="Cor triatriatum">Cor triatriatum</a></li> <li><a href="/wiki/Crisscross_heart" title="Crisscross heart">Crisscross heart</a></li> <li><a href="/wiki/Brugada_syndrome" title="Brugada syndrome">Brugada syndrome</a></li> <li><a href="/wiki/Coronary_artery_anomaly" title="Coronary artery anomaly">Coronary artery anomaly</a></li> <li><a href="/wiki/Anomalous_aortic_origin_of_a_coronary_artery" title="Anomalous aortic origin of a coronary artery">Anomalous aortic origin of a coronary artery</a></li> <li><a href="/wiki/Ventricular_inversion" title="Ventricular inversion">Ventricular inversion</a></li></ul> </div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"><style data-mw-deduplicate="TemplateStyles:r1038841319">.mw-parser-output .tooltip-dotted{border-bottom:1px dotted;cursor:help}</style><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1038841319"></div><div role="navigation" class="navbox authority-control" aria-label="Navbox" style="padding:3px"><table class="nowraplinks hlist navbox-inner" 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