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class="vector-toc-numb">2</span> <span>Causes</span> </div> </a> <ul id="toc-Causes-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Mechanism" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Mechanism"> <div class="vector-toc-text"> <span class="vector-toc-numb">3</span> <span>Mechanism</span> </div> </a> <button aria-controls="toc-Mechanism-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 Mechanism subsection</span> </button> <ul id="toc-Mechanism-sublist" class="vector-toc-list"> <li id="toc-Cyanotic_heart_disease" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Cyanotic_heart_disease"> <div class="vector-toc-text"> <span class="vector-toc-numb">3.1</span> <span>Cyanotic heart disease</span> </div> </a> <ul id="toc-Cyanotic_heart_disease-sublist" 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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/Mavi_k%C3%B6rp%C9%99_sindromu" title="Mavi körpə sindromu – Azerbaijani" lang="az" hreflang="az" data-title="Mavi körpə sindromu" data-language-autonym="Azərbaycanca" data-language-local-name="Azerbaijani" class="interlanguage-link-target"><span>Azərbaycanca</span></a></li><li class="interlanguage-link interwiki-ca mw-list-item"><a href="https://ca.wikipedia.org/wiki/S%C3%ADndrome_del_nad%C3%B3_blau" title="Síndrome del nadó blau – Catalan" lang="ca" hreflang="ca" data-title="Síndrome del nadó blau" data-language-autonym="Català" data-language-local-name="Catalan" class="interlanguage-link-target"><span>Català</span></a></li><li class="interlanguage-link interwiki-es mw-list-item"><a href="https://es.wikipedia.org/wiki/S%C3%ADndrome_del_beb%C3%A9_azul" title="Síndrome del bebé azul – Spanish" lang="es" hreflang="es" 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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">Blue baby syndrome</th></tr><tr><th scope="row" class="infobox-label">Other names</th><td class="infobox-data">Blue baby, cyanotic infant, cyanotic baby, cyanotic newborn</td></tr><tr style="background-color: #f8f9fa;"><td colspan="2" class="infobox-full-data"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:Cyanotic_neonate.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/e/e1/Cyanotic_neonate.jpg/220px-Cyanotic_neonate.jpg" decoding="async" width="220" height="281" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/e/e1/Cyanotic_neonate.jpg/330px-Cyanotic_neonate.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/e/e1/Cyanotic_neonate.jpg/440px-Cyanotic_neonate.jpg 2x" data-file-width="555" data-file-height="710" /></a></span></td></tr><tr><td colspan="2" class="infobox-full-data">A <a href="/wiki/Cyanotic" class="mw-redirect" title="Cyanotic">cyanotic</a> <a href="/wiki/Neonate" class="mw-redirect" title="Neonate">newborn</a>, or "blue baby".<br />Note the blue coloration of the fingertips.</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/Pediatrics" title="Pediatrics">Pediatrics</a>, <a href="/wiki/Cardiac_surgery" title="Cardiac surgery">cardiac surgery</a></td></tr></tbody></table> <p><b>Blue baby syndrome</b> can refer to conditions that cause cyanosis, or blueness of the skin, in babies as a result of low oxygen levels in the blood. This term has traditionally been applied to cyanosis as a result of:.<sup id="cite_ref-Pahal_2022_1-0" class="reference"><a href="#cite_note-Pahal_2022-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> </p> <ol><li><a href="/wiki/Cyanotic_heart_disease" class="mw-redirect" title="Cyanotic heart disease">Cyanotic heart disease</a>, which is a category of <a href="/wiki/Congenital_heart_defect" title="Congenital heart defect">congenital heart defect</a> that results in low levels of oxygen in the blood.<sup id="cite_ref-MedlinePlusEncyclopedia_Cyanotic_heart_disease_2-0" class="reference"><a href="#cite_note-MedlinePlusEncyclopedia_Cyanotic_heart_disease-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> This can be caused by either reduced blood flow to the lungs or mixing of oxygenated and deoxygenated blood.<sup id="cite_ref-MedlinePlusEncyclopedia_Cyanotic_heart_disease_2-1" class="reference"><a href="#cite_note-MedlinePlusEncyclopedia_Cyanotic_heart_disease-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Methemoglobinemia" title="Methemoglobinemia">Methemoglobinemia</a>, which is a disease defined by high levels of methemoglobin in the blood. Increased levels of <a href="/wiki/Methemoglobin" title="Methemoglobin">methemoglobin</a> prevent oxygen from being released into the tissues and result in hypoxemia.<sup id="cite_ref-3" class="reference"><a href="#cite_note-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup></li></ol> <p>Although these are the most common causes of cyanosis, there are other potential factors that can cause a blue tint to a baby's skin or mucous membranes. These factors include hypoventilation, perfusion or ventilation differences in the lungs, and poor cardiac output of oxygenated blood, among others. The blue baby syndrome or cyanosis occurs when absolute amount of deoxygenated hemoglobin > 3g/dL which is typically reflected with an O<sub>2</sub> saturation of < 85 %.<sup id="cite_ref-Pahal_2022_1-1" class="reference"><a href="#cite_note-Pahal_2022-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> </p><p>Both of these conditions cause <a href="/wiki/Cyanosis" title="Cyanosis">cyanosis</a>, or a bluish discoloration of skin or mucous membranes.<sup id="cite_ref-4" class="reference"><a href="#cite_note-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Normally, oxygenated blood appears red and deoxygenated blood has more of a blue appearance.<sup id="cite_ref-5" class="reference"><a href="#cite_note-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> In babies with low levels of oxygen or mixing of oxygenated and deoxygenated blood, the blood can have a blue or purple color, causing cyanosis.<sup id="cite_ref-Silove1994_6-0" class="reference"><a href="#cite_note-Silove1994-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p> <meta property="mw:PageProp/toc" /> <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=Blue_baby_syndrome&action=edit&section=1" title="Edit section: Signs and symptoms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The main sign of blue baby syndrome is cyanosis. Depending on the underlying cause of the cyanosis, additional symptoms may be:<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> </p> <ul><li>Failure to thrive</li> <li>Lethargy</li> <li>Nasal flaring</li> <li>Rapid heartbeat</li> <li>Rapid respiratory rate</li> <li>Seizures</li> <li>Shortness of breath</li></ul> <div class="mw-heading mw-heading2"><h2 id="Causes">Causes</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Blue_baby_syndrome&action=edit&section=2" title="Edit section: Causes"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><span class="anchor" id="Cause"></span> </p><p>Blue baby syndrome has been attributed to cyanotic congenital heart diseases and methemoglobinemia, however there are additional causes that could result in a baby becoming cyanotic, such as:<sup id="cite_ref-8" class="reference"><a href="#cite_note-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p> <ul><li>Airway obstruction</li> <li>Decreased blood perfusion</li> <li>Disordered control of breathing (ex: cyanotic breath-holding spells, seizures)</li> <li>Pulmonary/lung disorders (ex: pulmonary hypertension, cystic fibrosis)</li> <li><a href="/wiki/Acute_respiratory_distress_syndrome" title="Acute respiratory distress syndrome">Respiratory distress syndrome</a><sup id="cite_ref-9" class="reference"><a href="#cite_note-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup></li></ul> <div class="mw-heading mw-heading2"><h2 id="Mechanism">Mechanism</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Blue_baby_syndrome&action=edit&section=3" title="Edit section: Mechanism"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="mw-heading mw-heading3"><h3 id="Cyanotic_heart_disease">Cyanotic heart disease</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Blue_baby_syndrome&action=edit&section=4" title="Edit section: Cyanotic heart disease"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Specific types of congenital heart defects that cause blood to pass directly from the right side of the heart to the left side result in cyanosis.<sup id="cite_ref-10" class="reference"><a href="#cite_note-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> In these defects, some of the blood that is pumped to the body has not been oxygenated by the lungs and therefore will appear more blue. Infants with these types of heart defects may have a constant bluish tint to their skin, or they may have temporary episodes of cyanosis. The degree of cyanosis is dependent on how much deoxygenated blood is mixed with oxygenated blood before being pumped to the body. </p><p>Cardiac conditions in which there is decreased blood flow to the lungs such as, tetralogy of Fallot or pulmonary valve atresia, result in less blood becoming oxygenated. There are also cardiac conditions such as transposition of the great arteries or truncus arteriosus, that results in an overall increase in blood flow to the lungs but with limited flow of the oxygenated blood to the rest of the body. Conditions in which there is poor blood flow to the systemic circulation, such as coarctation of the aorta suggests that the body does not receive the oxygenated blood it requires with resultant cyanosis.<sup id="cite_ref-11" class="reference"><a href="#cite_note-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> </p><p>The five most common cyanotic heart defects that may result in Blue Baby Syndrome include the following: </p> <table class="wikitable" style="width: 75%; text-align:center;"> <tbody><tr> <th style="width: 25%">Pathology </th> <th style="width: 25%">Brief Description </th> <th style="width: 15%">Epidemiology </th> <th style="width: 35%">Diagram </th></tr> <tr> <td><a href="/wiki/Persistent_truncus_arteriosus" title="Persistent truncus arteriosus">1. Persistent (or patent) truncus arteriosus</a> </td> <td>Instead of two separate major blood vessels leaving the heart, there is one common outgoing vessel.<sup id="cite_ref-12" class="reference"><a href="#cite_note-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </td> <td>3-5/100,000 births<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> </td> <td><span typeof="mw:File/Frameless"><a href="/wiki/File:Truncus_arteriosus.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/b/bf/Truncus_arteriosus.jpg/200px-Truncus_arteriosus.jpg" decoding="async" width="200" height="256" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/b/bf/Truncus_arteriosus.jpg/299px-Truncus_arteriosus.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/b/bf/Truncus_arteriosus.jpg/399px-Truncus_arteriosus.jpg 2x" data-file-width="575" data-file-height="737" /></a></span> </td></tr> <tr> <td>2. <a href="/wiki/Transposition_of_the_great_vessels" title="Transposition of the great vessels">Transposition of the great vessels</a> </td> <td>The positions of the pulmonary artery and the aorta are switched, with the aorta connecting to the right ventricle and the pulmonary artery connecting to the left ventricle.<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> </td> <td>1-5/10,000 births<sup id="cite_ref-15" class="reference"><a href="#cite_note-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> </td> <td><span typeof="mw:File/Frameless"><a href="/wiki/File:D-tga-575px.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/6/66/D-tga-575px.jpg/188px-D-tga-575px.jpg" decoding="async" width="188" height="256" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/6/66/D-tga-575px.jpg/282px-D-tga-575px.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/6/66/D-tga-575px.jpg/376px-D-tga-575px.jpg 2x" data-file-width="575" data-file-height="782" /></a></span> </td></tr> <tr> <td>3. <a href="/wiki/Tricuspid_atresia" title="Tricuspid atresia">Tricuspid atresia</a> </td> <td>The heart valve connecting the right atrium to the right ventricle does not form properly, disrupting blood flow in the heart.<sup id="cite_ref-16" class="reference"><a href="#cite_note-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> </td> <td>1-9/100,000 births<sup id="cite_ref-17" class="reference"><a href="#cite_note-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </td> <td><span typeof="mw:File/Frameless"><a href="/wiki/File:Tricuspid_atresia.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/8/86/Tricuspid_atresia.svg/256px-Tricuspid_atresia.svg.png" decoding="async" width="256" height="256" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/8/86/Tricuspid_atresia.svg/384px-Tricuspid_atresia.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/8/86/Tricuspid_atresia.svg/512px-Tricuspid_atresia.svg.png 2x" data-file-width="650" data-file-height="650" /></a></span> </td></tr> <tr> <td>4. <a href="/wiki/Tetralogy_of_Fallot" title="Tetralogy of Fallot">Tetralogy of Fallot</a> </td> <td>A heart defect with 4 features including a narrow pulmonary artery, a thick right ventricle, an aorta that connects to both the right and left ventricles, and a hole in the ventricular wall.<sup id="cite_ref-CDC_Facts_about_Tetralogy_of_Fallot_18-0" class="reference"><a href="#cite_note-CDC_Facts_about_Tetralogy_of_Fallot-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> </td> <td>1-5/10,000 births<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> </td> <td><span typeof="mw:File/Frameless"><a href="/wiki/File:Teralogy_web.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/6/6d/Teralogy_web.jpg/198px-Teralogy_web.jpg" decoding="async" width="198" height="256" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/6/6d/Teralogy_web.jpg/297px-Teralogy_web.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/6/6d/Teralogy_web.jpg/396px-Teralogy_web.jpg 2x" data-file-width="500" data-file-height="647" /></a></span> </td></tr> <tr> <td>5. <a href="/wiki/Anomalous_pulmonary_venous_connection" title="Anomalous pulmonary venous connection">Anomalous pulmonary venous connection</a> </td> <td>The pulmonary veins returning oxygenated blood from the lungs do not connect properly to the heart.<sup id="cite_ref-20" class="reference"><a href="#cite_note-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup> </td> <td>6-12/100,000 births<sup id="cite_ref-21" class="reference"><a href="#cite_note-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup> </td> <td><span typeof="mw:File/Frameless"><a href="/wiki/File:Tapv-575px.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/a/a0/Tapv-575px.jpg/221px-Tapv-575px.jpg" decoding="async" width="221" height="256" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/a/a0/Tapv-575px.jpg/331px-Tapv-575px.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/a/a0/Tapv-575px.jpg/441px-Tapv-575px.jpg 2x" data-file-width="575" data-file-height="667" /></a></span> </td></tr></tbody></table> <div class="mw-heading mw-heading3"><h3 id="Methemoglobinemia">Methemoglobinemia</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Blue_baby_syndrome&action=edit&section=5" title="Edit section: Methemoglobinemia"><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:Oxyhaemoglobin_dissociation_curve.png" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/8/8a/Oxyhaemoglobin_dissociation_curve.png/220px-Oxyhaemoglobin_dissociation_curve.png" decoding="async" width="220" height="207" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/8/8a/Oxyhaemoglobin_dissociation_curve.png/330px-Oxyhaemoglobin_dissociation_curve.png 1.5x, //upload.wikimedia.org/wikipedia/commons/8/8a/Oxyhaemoglobin_dissociation_curve.png 2x" data-file-width="439" data-file-height="413" /></a><figcaption>Hemoglobin oxygenation dissociation curve. In the case of methemoglobinemia the curve is shifted to the left given the higher affinity for oxygen.<sup id="cite_ref-:1_22-0" class="reference"><a href="#cite_note-:1-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup></figcaption></figure> <p><a href="/wiki/Methemoglobinemia" title="Methemoglobinemia">Methemoglobinemia</a> can be acquired or congenital. It occurs when the <a href="/wiki/Iron" title="Iron">iron</a> in <a href="/wiki/Hemoglobin" title="Hemoglobin">hemoglobin</a> is <a href="/wiki/Redox" title="Redox">oxidized</a> from Fe<sup>2+</sup> to Fe<sup>3+</sup>, leading to poor binding of <a href="/wiki/Oxygen" title="Oxygen">oxygen</a>. Additionally, the oxygen that is already bound is held more tightly to the hemoglobin due to a higher affinity, resulting in less oxygen delivery. A methemoglobin level > 1.5 g/dL causes <a href="/wiki/Cyanosis" title="Cyanosis">cyanosis</a>. The most common congenital cause is a deficiency in the <a href="/wiki/Enzyme" title="Enzyme">enzyme</a> <a href="/wiki/Cytochrome_b5_reductase" title="Cytochrome b5 reductase">cytochrome b5 reductase</a> which <a href="/wiki/Redox" title="Redox">reduces</a> <a href="/wiki/Methemoglobin" title="Methemoglobin">methemoglobin</a> in the blood.<sup id="cite_ref-:1_22-1" class="reference"><a href="#cite_note-:1-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup> </p><p>However, in infants the most common cause of methemoglobinemia is acquired through the ingestion of <a href="/wiki/Nitrate" title="Nitrate">nitrates</a> (<style data-mw-deduplicate="TemplateStyles:r1123817410">.mw-parser-output .template-chem2-su{display:inline-block;font-size:80%;line-height:1;vertical-align:-0.35em}.mw-parser-output .template-chem2-su>span{display:block;text-align:left}.mw-parser-output sub.template-chem2-sub{font-size:80%;vertical-align:-0.35em}.mw-parser-output sup.template-chem2-sup{font-size:80%;vertical-align:0.65em}</style><span class="chemf nowrap">NO<span class="template-chem2-su"><span>−</span><span>3</span></span></span>) through <a href="/wiki/Well_water" class="mw-redirect" title="Well water">well water</a> or foods. <a href="/wiki/Nitrite" title="Nitrite">Nitrites</a> (<link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1123817410"><span class="chemf nowrap">NO<span class="template-chem2-su"><span>−</span><span>2</span></span></span>) produced by the <a href="/wiki/Denitrification" title="Denitrification">microbial reduction of nitrate</a> (directly in the drinkwater, or after ingestion by the infant, in his digestive system) are more powerful <a href="/wiki/Oxidizing_agent" title="Oxidizing agent">oxidizers</a> than nitrates and are the chemical agent really responsible of the <a href="/wiki/Redox" title="Redox">oxidation</a> of Fe<sup>2+</sup> into Fe<sup>3+</sup> in the <a href="/wiki/Tetrapyrrole" title="Tetrapyrrole">tetrapyrrole</a> <a href="/wiki/Heme" title="Heme">heme</a> of <a href="/wiki/Hemoglobin" title="Hemoglobin">hemoglobin</a>. Infants younger than 4 months are at greater risk given that they drink more water per body weight, they have a lower <a href="/wiki/Nicotinamide_adenine_dinucleotide" title="Nicotinamide adenine dinucleotide">NADH</a>- <a href="/wiki/Cytochrome_b5_reductase" title="Cytochrome b5 reductase">cytochrome b5 reductase</a> activity, and they have a higher level of <a href="/wiki/Fetal_hemoglobin" title="Fetal hemoglobin">fetal hemoglobin</a> which converts more easily to <a href="/wiki/Methemoglobin" title="Methemoglobin">methemoglobin</a>. Additionally, infants are at an increased risk after an episode of gastroenteritis due to the production of <a href="/wiki/Nitrite" title="Nitrite">nitrites</a> by <a href="/wiki/Bacteria" title="Bacteria">bacteria</a>.<sup id="cite_ref-:1_22-2" class="reference"><a href="#cite_note-:1-22"><span class="cite-bracket">[</span>22<span class="cite-bracket">]</span></a></sup> The sources of nitrate can include <a href="/wiki/Fertilizer" title="Fertilizer">fertilizers</a> used in agricultural lands, waste dumps or <a href="/wiki/Pit_latrine" title="Pit latrine">pit latrines</a>.<sup id="cite_ref-The_Blud_Baby_Syndrome_23-0" class="reference"><a href="#cite_note-The_Blud_Baby_Syndrome-23"><span class="cite-bracket">[</span>23<span class="cite-bracket">]</span></a></sup> For example, nitrate levels are subject to monitoring to comply with <a href="/wiki/Water_quality" title="Water quality">drinking water quality</a> standards in the United States and other countries.<sup id="cite_ref-24" class="reference"><a href="#cite_note-24"><span class="cite-bracket">[</span>24<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-25" class="reference"><a href="#cite_note-25"><span class="cite-bracket">[</span>25<span class="cite-bracket">]</span></a></sup> The link between blue baby syndrome and nitrates in drinking water is widely accepted, but as of 2006 some studies indicated that other contaminants or dietary nitrate sources, might also play a role in the syndrome.<sup id="cite_ref-Fewtrell_26-0" class="reference"><a href="#cite_note-Fewtrell-26"><span class="cite-bracket">[</span>26<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-vanGrinsven_27-0" class="reference"><a href="#cite_note-vanGrinsven-27"><span class="cite-bracket">[</span>27<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Ward_28-0" class="reference"><a href="#cite_note-Ward-28"><span class="cite-bracket">[</span>28<span class="cite-bracket">]</span></a></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=Blue_baby_syndrome&action=edit&section=6" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>When diagnosing blue baby syndrome, it is important to perform a thorough history and physical exam. When obtaining the history, it is important to determine the timing of symptoms and to ask about risk factors/exposures, such as prenatal history or access to well-water.<sup id="cite_ref-Bearl_2019_29-0" class="reference"><a href="#cite_note-Bearl_2019-29"><span class="cite-bracket">[</span>29<span class="cite-bracket">]</span></a></sup> </p> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:Pulse_Oximeter.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/6/6f/Pulse_Oximeter.jpg/220px-Pulse_Oximeter.jpg" decoding="async" width="220" height="293" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/6/6f/Pulse_Oximeter.jpg/330px-Pulse_Oximeter.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/6/6f/Pulse_Oximeter.jpg/440px-Pulse_Oximeter.jpg 2x" data-file-width="3024" data-file-height="4032" /></a><figcaption><a href="/wiki/Pulse_oximetry" title="Pulse oximetry">Pulse oximeter</a> on infant's foot.</figcaption></figure> <p>On physical exam it is important to visualize where the cyanosis is present to differentiate between peripheral and central <a href="/wiki/Cyanosis" title="Cyanosis">cyanosis</a>. Central cyanosis is typically visible as a blueish discoloration over the entire body and mucous membranes. In contrast, peripheral cyanosis typically has a blueish discoloration over the extremities. Cyanosis can be noted in babies around the lips, tongue, and sublingual area, where the skin is thinnest.<sup id="cite_ref-30" class="reference"><a href="#cite_note-30"><span class="cite-bracket">[</span>30<span class="cite-bracket">]</span></a></sup> In addition, it is important to observe the infant for signs of respiratory distress, visualized as nasal flaring, subcostal retractions, etc. Examination should include a respiratory and cardiac assessment.<sup id="cite_ref-Bearl_2019_29-1" class="reference"><a href="#cite_note-Bearl_2019-29"><span class="cite-bracket">[</span>29<span class="cite-bracket">]</span></a></sup> </p><p>One of the key tools in diagnosing is a <a href="/wiki/Pulse_oximetry" title="Pulse oximetry">pulse oximeter</a> to determine <a href="/wiki/Oxygen_saturation_(medicine)" title="Oxygen saturation (medicine)">oxygen saturation</a>. While severe cyanosis can be easily noticed, an oxygen saturation as low as 80 % causes only mild clinical cyanosis that is difficult to see.<sup id="cite_ref-Silove1994_6-1" class="reference"><a href="#cite_note-Silove1994-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> Additionally an arterial blood gas is useful, for example in the case of methemoglobinemia the PO<sub>2</sub> can be expected to be normal even with a low oxygen saturation. Additional work up includes a complete blood count, <a href="/wiki/Blood_sugar_level" title="Blood sugar level">blood glucose</a>, blood culture, chest X-ray, and an <a href="/wiki/Echocardiography" title="Echocardiography">echocardiography</a>.<sup id="cite_ref-Bearl_2019_29-2" class="reference"><a href="#cite_note-Bearl_2019-29"><span class="cite-bracket">[</span>29<span class="cite-bracket">]</span></a></sup> </p><p>Babies with cyanosis due to congenital heart disease usually present with symptoms hours to days after birth. In addition to cyanosis, they often show signs of <a href="/wiki/Tachypnea" title="Tachypnea">tachypnea</a> (fast breathing), a heart murmur, and decreased peripheral pulses.<sup id="cite_ref-Silove1994_6-2" class="reference"><a href="#cite_note-Silove1994-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-ReferenceA_31-0" class="reference"><a href="#cite_note-ReferenceA-31"><span class="cite-bracket">[</span>31<span class="cite-bracket">]</span></a></sup> If congenital heart disease is suspected in a newborn, doctors will likely perform several tests to evaluate the heart, including a <a href="/wiki/Chest_radiograph" title="Chest radiograph">chest x-ray</a>, <a href="/wiki/Echocardiogram" class="mw-redirect" title="Echocardiogram">echocardiogram</a>, and <a href="/wiki/Electrocardiography" title="Electrocardiography">electrocardiogram</a>.<sup id="cite_ref-32" class="reference"><a href="#cite_note-32"><span class="cite-bracket">[</span>32<span class="cite-bracket">]</span></a></sup> In <a href="/wiki/Tetralogy_of_Fallot" title="Tetralogy of Fallot">tetralogy of Fallot</a>, episodes in which infants become cyanotic are called tet spells, typically occurring during feeding or crying.<sup id="cite_ref-CDC_Facts_about_Tetralogy_of_Fallot_18-1" class="reference"><a href="#cite_note-CDC_Facts_about_Tetralogy_of_Fallot-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> When older, children may squat to feel relief since this increases the systemic vascular resistance causing more blood to go towards the lungs, resulting in increased oxygenation.<sup id="cite_ref-CDC_Facts_about_Tetralogy_of_Fallot_18-2" class="reference"><a href="#cite_note-CDC_Facts_about_Tetralogy_of_Fallot-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> </p><p>Babies with cyanosis due to methemoglobinemia also usually present with cyanosis in the neonatal period, but pulse oximetry may be falsely elevated and does not always reveal low blood oxygen saturation. A <a href="/wiki/CO-oximeter" title="CO-oximeter">CO-oximeter</a> can be used to detect levels of methemoglobin in the blood if methemoglobinemia is suspected, by seeing the difference between oxygen saturation on an arterial blood gas and the measurement on a co-oximeter. Additionally, a direct methemoglobin level can be obtained.<sup id="cite_ref-Da-Silva_Sajan_Underwood_2003_33-0" class="reference"><a href="#cite_note-Da-Silva_Sajan_Underwood_2003-33"><span class="cite-bracket">[</span>33<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Prevention/Screening"><span id="Prevention.2FScreening"></span>Prevention/Screening</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Blue_baby_syndrome&action=edit&section=7" title="Edit section: Prevention/Screening"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>As of 22 May 2007, the United States Environmental Protection Agency has established a maximum contaminant level of 10 mg/L for nitrate and 1 mg/L for nitrite in drinking water due to the potential harmful effects in infants.<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> </p><p>A screening tool has been developed to screen for critical cardiac defects, which refers to cardiac lesions that require surgery or intervention in the 1st year of life. Screening for critical congenital heart defects should be done on all newborns after 24hours or shortly before discharge. Oxygen saturation is measured in the right hand and either foot.<sup id="cite_ref-35" class="reference"><a href="#cite_note-35"><span class="cite-bracket">[</span>35<span class="cite-bracket">]</span></a></sup> </p><p>A screening is considered positive if: </p> <ul><li>Oxygen saturation < 90 % in either extremity,</li> <li>Oxygen saturation 90-94 % in both extremities on 3 measurements separated by an hour each,</li> <li>Oxygen saturation difference > 3 % between both extremities on 3 measurements separated by an hour each.</li></ul> <div class="mw-heading mw-heading2"><h2 id="Management">Management</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Blue_baby_syndrome&action=edit&section=8" title="Edit section: Management"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Treatment for blue baby syndrome will depend on the underlying cause. </p><p>When evaluating a patient for cyanosis or respiratory distress, vital signs should be monitored, especially the patient's heart rate and oxygen saturation. It is beneficial to have vascular access established. In newborns, the pulse oximeter is typically placed on the right hand to determine pre-ductal oxygenation, referring to oxygenation before the <a href="/wiki/Ductus_arteriosus" title="Ductus arteriosus">ductus arteriosus</a> (connection between <a href="/wiki/Aorta" title="Aorta">aorta</a> and <a href="/wiki/Pulmonary_artery" title="Pulmonary artery">pulmonary artery</a>).<sup id="cite_ref-:2_36-0" class="reference"><a href="#cite_note-:2-36"><span class="cite-bracket">[</span>36<span class="cite-bracket">]</span></a></sup> This gives the oxygenation level the heart and brain receive. Traditionally, supplemental oxygen is given in an escalating manner beginning with free-flowing oxygen, progressing to positive pressure ventilation or continuous positive airway pressure, and ending with mechanical intubation. The goal oxygen saturation is between 85 and 95 %. If an infant requires supplemental oxygen for a prolonged time it should be heated and humidified to avoid heat loss.<sup id="cite_ref-:2_36-1" class="reference"><a href="#cite_note-:2-36"><span class="cite-bracket">[</span>36<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Cyanotic_heart_disease_2">Cyanotic heart disease</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Blue_baby_syndrome&action=edit&section=9" title="Edit section: Cyanotic heart disease"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Some babies born with <a href="/wiki/Cyanotic_heart_defect" title="Cyanotic heart defect">cyanotic heart disease</a> are treated with <a href="/wiki/Prostaglandin_E1" title="Prostaglandin E1">prostaglandin E1</a> after birth to keep the <a href="/wiki/Ductus_arteriosus" title="Ductus arteriosus">ductus arteriosus</a> open and allow for more <a href="/wiki/Oxygenated_blood" class="mw-redirect" title="Oxygenated blood">oxygenated blood</a> to be pumped to the body. Many also receive <a href="/wiki/Oxygen_therapy" title="Oxygen therapy">oxygen therapy</a> to increase the percentage of oxygen in the blood. Most of these babies will require surgery during infancy to correct their structural heart defect.<sup id="cite_ref-ReferenceA_31-1" class="reference"><a href="#cite_note-ReferenceA-31"><span class="cite-bracket">[</span>31<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Severe_methemoglobinemia">Severe methemoglobinemia</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Blue_baby_syndrome&action=edit&section=10" title="Edit section: Severe methemoglobinemia"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The first-line treatment for severe methemoglobinemia is <a href="/wiki/Methylene_blue" title="Methylene blue">methylene blue</a>, a medication that will <a href="/wiki/Redox" title="Redox">reduce</a> methemoglobin in the blood. This is possible because methylene blue <a href="/wiki/Redox" title="Redox">oxidizes</a> <a href="/wiki/Nicotinamide_adenine_dinucleotide_phosphate" title="Nicotinamide adenine dinucleotide phosphate">NADPH</a>, which in turn can convert methemoglobin back to hemoglobin.<sup id="cite_ref-Da-Silva_Sajan_Underwood_2003_33-1" class="reference"><a href="#cite_note-Da-Silva_Sajan_Underwood_2003-33"><span class="cite-bracket">[</span>33<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=Blue_baby_syndrome&action=edit&section=11" title="Edit section: Epidemiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Out of all the babies born with congenital heart defects, about 25 % have cyanosis as a result. <a href="/wiki/Tetralogy_of_Fallot" title="Tetralogy of Fallot">Tetralogy of Fallot</a> is the most common cyanotic cardiac heart defect.<sup id="cite_ref-Ossa_Galvis_2022_37-0" class="reference"><a href="#cite_note-Ossa_Galvis_2022-37"><span class="cite-bracket">[</span>37<span class="cite-bracket">]</span></a></sup> </p><p>Methemoglobinemia is considered to be rare, with acquired methemoglobinemia encountered more than the congenital form.<sup id="cite_ref-38" class="reference"><a href="#cite_note-38"><span class="cite-bracket">[</span>38<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Outcomes">Outcomes</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Blue_baby_syndrome&action=edit&section=12" title="Edit section: Outcomes"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>In the case of cyanotic causing heart defects, about 75 % of infants survive to 1 year of age and 69 % survive to 18 years of age. These individuals have an increased risk of developmental delay, heart failure, or heart rhythm disorders.<sup id="cite_ref-Ossa_Galvis_2022_37-1" class="reference"><a href="#cite_note-Ossa_Galvis_2022-37"><span class="cite-bracket">[</span>37<span class="cite-bracket">]</span></a></sup> </p><p>Methemoglobinemia responds well to treatment, its prognosis is associated with the level of methemoglobinemia and the degree of end organ damage it can cause. Death can occur when levels reach 70 %.<sup id="cite_ref-39" class="reference"><a href="#cite_note-39"><span class="cite-bracket">[</span>39<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=Blue_baby_syndrome&action=edit&section=13" title="Edit section: History"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The first successful operation to treat blue baby syndrome caused by <a href="/wiki/Tetralogy_of_Fallot" title="Tetralogy of Fallot">tetralogy of Fallot</a> occurred at Johns Hopkins University in 1944. Through a collaboration between pediatric cardiologist <a href="/wiki/Helen_B._Taussig" title="Helen B. Taussig">Helen Taussig</a>, surgeon <a href="/wiki/Alfred_Blalock" title="Alfred Blalock">Alfred Blalock</a>, and surgical technician <a href="/wiki/Vivien_Thomas" title="Vivien Thomas">Vivien Thomas</a>, the <a href="/wiki/Blalock%E2%80%93Taussig_shunt" class="mw-redirect" title="Blalock–Taussig shunt">Blalock-Thomas-Taussig</a> shunt was created. Dr. Taussig had recognized that children with Tetralogy of Fallot who also had a <a href="/wiki/Patent_ductus_arteriosus" title="Patent ductus arteriosus">patent ductus arteriosus</a> (PDA) typically lived longer, so the trio tried to create the same effect as a PDA by joining the subclavian artery to the pulmonary artery, relieving the child's cyanosis.<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> The operation was published in the Journal of the American Medical Association in 1945 and impacted management of blue babies around the world.<sup id="cite_ref-41" class="reference"><a href="#cite_note-41"><span class="cite-bracket">[</span>41<span class="cite-bracket">]</span></a></sup> </p><p>Anna was the name of the dog who was the first survivor of the surgery, considered an experimental procedure at the time. Anna survived the first pulmonary bypass after having been operated on twice. The second operation was required to replace the original stitches with flexible ones. After their success with Anna, Blalock and Thomas had the courage to perform the very first <a href="/wiki/Open_heart_surgery" class="mw-redirect" title="Open heart surgery">open heart surgery</a> on <a href="/wiki/Eileen_Saxon" title="Eileen Saxon">Eileen Saxon</a> in 1944. In 1950, Anna's story was made into a movie, and the film has been shown to various schools and other groups.<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> </p> <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=Blue_baby_syndrome&action=edit&section=14" 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-Pahal_2022-1"><span class="mw-cite-backlink">^ <a href="#cite_ref-Pahal_2022_1-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Pahal_2022_1-1"><sup><i><b>b</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="CITEREFPahalGoyal2024" class="citation book cs1">Pahal, Parul; Goyal, Amandeep (2024). <a rel="nofollow" class="external text" href="http://www.ncbi.nlm.nih.gov/books/NBK559167/">"Central and Peripheral Cyanosis"</a>. <i>StatPearls</i>. 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/32644593">32644593</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=bookitem&rft.atitle=Central+and+Peripheral+Cyanosis&rft.btitle=StatPearls&rft.pub=StatPearls+Publishing&rft.date=2024&rft_id=info%3Apmid%2F32644593&rft.aulast=Pahal&rft.aufirst=Parul&rft.au=Goyal%2C+Amandeep&rft_id=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fbooks%2FNBK559167%2F&rfr_id=info%3Asid%2Fen.wikipedia.org%3ABlue+baby+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-MedlinePlusEncyclopedia_Cyanotic_heart_disease-2"><span class="mw-cite-backlink">^ <a href="#cite_ref-MedlinePlusEncyclopedia_Cyanotic_heart_disease_2-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-MedlinePlusEncyclopedia_Cyanotic_heart_disease_2-1"><sup><i><b>b</b></i></sup></a></span> <span class="reference-text"><i><a href="/wiki/MedlinePlus" title="MedlinePlus">MedlinePlus Encyclopedia</a></i>: <a rel="nofollow" class="external text" href="https://medlineplus.gov/ency/article/001104.htm">Cyanotic heart disease</a></span> </li> <li id="cite_note-3"><span class="mw-cite-backlink"><b><a href="#cite_ref-3">^</a></b></span> <span class="reference-text"><i><a href="/wiki/MedlinePlus" title="MedlinePlus">MedlinePlus Encyclopedia</a></i>: <a rel="nofollow" class="external text" href="https://medlineplus.gov/ency/article/000562.htm">Methemoglobinemia</a></span> </li> <li id="cite_note-4"><span class="mw-cite-backlink"><b><a href="#cite_ref-4">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFSnider1990" class="citation book cs1">Snider, H. L. (1990). <a rel="nofollow" class="external text" href="http://www.ncbi.nlm.nih.gov/books/NBK367/">"Cyanosis"</a>. <i>Clinical Methods: The History, Physical, and Laboratory Examinations</i>. Butterworths. <a href="/wiki/ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <a href="/wiki/Special:BookSources/978-0-409-90077-4" title="Special:BookSources/978-0-409-90077-4"><bdi>978-0-409-90077-4</bdi></a>. <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/21250208">21250208</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=bookitem&rft.atitle=Cyanosis&rft.btitle=Clinical+Methods%3A+The+History%2C+Physical%2C+and+Laboratory+Examinations&rft.pub=Butterworths&rft.date=1990&rft_id=info%3Apmid%2F21250208&rft.isbn=978-0-409-90077-4&rft.aulast=Snider&rft.aufirst=H.+L.&rft_id=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fbooks%2FNBK367%2F&rfr_id=info%3Asid%2Fen.wikipedia.org%3ABlue+baby+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-5"><span class="mw-cite-backlink"><b><a href="#cite_ref-5">^</a></b></span> <span class="reference-text"><i><a href="/wiki/MedlinePlus" title="MedlinePlus">MedlinePlus Encyclopedia</a></i>: <a rel="nofollow" class="external text" href="https://medlineplus.gov/ency/article/003215.htm">Blue discoloration of the skin</a></span> </li> <li id="cite_note-Silove1994-6"><span class="mw-cite-backlink">^ <a href="#cite_ref-Silove1994_6-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Silove1994_6-1"><sup><i><b>b</b></i></sup></a> <a href="#cite_ref-Silove1994_6-2"><sup><i><b>c</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFSilove1994" class="citation journal cs1">Silove, E D (1994). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1060995">"Assessment and management of congenital heart disease in the newborn by the district paediatrician"</a>. <i>Archives of Disease in Childhood - Fetal and Neonatal Edition</i>. <b>70</b> (1): <span class="nowrap">F71 –</span> <span class="nowrap">F74</span>. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1136%2Ffn.70.1.f71">10.1136/fn.70.1.f71</a>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1060995">1060995</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/8117134">8117134</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Archives+of+Disease+in+Childhood+-+Fetal+and+Neonatal+Edition&rft.atitle=Assessment+and+management+of+congenital+heart+disease+in+the+newborn+by+the+district+paediatrician.&rft.volume=70&rft.issue=1&rft.pages=%3Cspan+class%3D%22nowrap%22%3EF71+-%3C%2Fspan%3E+%3Cspan+class%3D%22nowrap%22%3EF74%3C%2Fspan%3E&rft.date=1994&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC1060995%23id-name%3DPMC&rft_id=info%3Apmid%2F8117134&rft_id=info%3Adoi%2F10.1136%2Ffn.70.1.f71&rft.aulast=Silove&rft.aufirst=E+D&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC1060995&rfr_id=info%3Asid%2Fen.wikipedia.org%3ABlue+baby+syndrome" 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="CITEREFEnlowGreenberg2019" class="citation book cs1">Enlow, Elizabeth; Greenberg, James M. (2019). <a rel="nofollow" class="external text" href="https://books.google.com/books?id=LJuRDwAAQBAJ&pg=PA910">"Clinical Manifestations of Diseases in the Newborn Period"</a>. In Kliegman, Robert M.; St Geme, Joseph W. (eds.). <i>Nelson Textbook of Pediatrics</i>. 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a>span,.mw-parser-output .navbar a>abbr{text-decoration:inherit}.mw-parser-output .navbar-mini abbr{font-variant:small-caps;border-bottom:none;text-decoration:none;cursor:inherit}.mw-parser-output .navbar-ct-full{font-size:114%;margin:0 7em}.mw-parser-output .navbar-ct-mini{font-size:114%;margin:0 4em}html.skin-theme-clientpref-night .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}@media(prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}}@media print{.mw-parser-output .navbar{display:none!important}}</style><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Congenital_heart_defects" title="Template:Congenital heart defects"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Congenital_heart_defects" title="Template talk:Congenital heart defects"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Congenital_heart_defects" title="Special:EditPage/Template:Congenital heart defects"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Congenital_heart_defects82" style="font-size:114%;margin:0 4em"><a href="/wiki/Congenital_heart_defect" title="Congenital heart defect">Congenital 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 href="/wiki/Tetralogy_of_Fallot" title="Tetralogy of Fallot">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><noscript><img src="https://login.wikimedia.org/wiki/Special:CentralAutoLogin/start?useformat=desktop&type=1x1&usesul3=0" alt="" width="1" height="1" style="border: none; position: 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