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Blue baby syndrome

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502:. 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. 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. 332: 608:). 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. 276: 234: 42: 488: 297: 255: 318: 1151: 1280: 1217: 719:(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. The operation was published in the Journal of the American Medical Association in 1945 and impacted management of blue babies around the world. 198:
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.
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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
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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
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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
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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. 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
120:, or a bluish discoloration of skin or mucous membranes. Normally, oxygenated blood appears red and deoxygenated blood has more of a blue appearance. 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. 108:
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
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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
548:, episodes in which infants become cyanotic are called tet spells, typically occurring during feeding or crying. 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. 483:
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.
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standards in the United States and other countries. 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.
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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.
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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.
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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.
513:. While severe cyanosis can be easily noticed, an oxygen saturation as low as 80 % causes only mild clinical cyanosis that is difficult to see. Additionally an arterial blood gas is useful, for example in the case of methemoglobinemia the PO 565:
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.
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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
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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 %.
532:(fast breathing), a heart murmur, and decreased peripheral pulses. If congenital heart disease is suspected in a newborn, doctors will likely perform several tests to evaluate the heart, including a 83:
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:.
358:. 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 161:
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:
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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.
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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.
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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
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that results in low levels of oxygen in the blood. This can be caused by either reduced blood flow to the lungs or mixing of oxygenated and deoxygenated blood.
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to increase the percentage of oxygen in the blood. Most of these babies will require surgery during infancy to correct their structural heart defect.
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others. The blue baby syndrome or cyanosis occurs when absolute amount of deoxygenated hemoglobin > 3g/dL which is typically reflected with an O
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Hemoglobin oxygenation dissociation curve. In the case of methemoglobinemia the curve is shifted to the left given the higher affinity for oxygen.
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Ward, Mary H.; deKok, Theo M.; Levallois, Patrick; Brender, Jean; Gulis, Gabriel; Nolan, Bernard T.; VanDerslice, James (23 June 2005).
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The main sign of blue baby syndrome is cyanosis. Depending on the underlying cause of the cyanosis, additional symptoms may be:
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The heart valve connecting the right atrium to the right ventricle does not form properly, disrupting blood flow in the heart.
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On physical exam it is important to visualize where the cyanosis is present to differentiate between peripheral and central
177: 438:. Infants younger than 4 months are at greater risk given that they drink more water per body weight, they have a lower 2236: 2195: 2153: 1467: 731:
in 1944. In 1950, Anna's story was made into a movie, and the film has been shown to various schools and other groups.
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Methemoglobinemia is considered to be rare, with acquired methemoglobinemia encountered more than the congenital form.
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Khalil, Markus; Jux, Christian; Rueblinger, Lucie; Behrje, Johanna; Esmaeili, Anoosh; Schranz, Dietmar (April 2019).
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can be expected to be normal even with a low oxygen saturation. Additional work up includes a complete blood count,
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Oxygen saturation difference > 3 % between both extremities on 3 measurements separated by an hour each.
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Lin, Pei-Yi; Hagan, Katherine; Fenoglio, Angela; Grant, P. Ellen; Franceschini, Maria Angela (16 May 2016).
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The five most common cyanotic heart defects that may result in Blue Baby Syndrome include the following:
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Stack, Anne M (2006). "Cyanosis". In Fleisher, Gary Robert; Ludwig, Stephen; Henretig, Fred M (eds.).
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occurred at Johns Hopkins University in 1944. Through a collaboration between pediatric cardiologist
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Out of all the babies born with congenital heart defects, about 25 % have cyanosis as a result.
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shunt was created. Dr. Taussig had recognized that children with Tetralogy of Fallot who also had a
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However, in infants the most common cause of methemoglobinemia is acquired through the ingestion of
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The pulmonary veins returning oxygenated blood from the lungs do not connect properly to the heart.
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Instead of two separate major blood vessels leaving the heart, there is one common outgoing vessel.
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Oxygen saturation 90-94 % in both extremities on 3 measurements separated by an hour each,
100:, which is a disease defined by high levels of methemoglobin in the blood. Increased levels of 87: 924: 2465: 2368: 2358: 2177: 1917: 1591: 617: 2486: 2385: 2363: 2335: 2264: 2187: 1551: 1007: 8: 2218: 2124: 1990: 696: 668: 545: 541: 284: 1613:"Workgroup Report: Drinking-Water Nitrate and Health—Recent Findings and Research Needs" 1555: 1011: 1936:
Ossa Galvis, Maria M.; Bhakta, Rupal T.; Tarmahomed, Abdulla; Mendez, Magda D. (2024).
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success with Anna, Blalock and Thomas had the courage to perform the very first
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Disordered control of breathing (ex: cyanotic breath-holding spells, seizures)
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Bearl, David W.; Hill, Kevin (2019), Jain, Lucky; Suresh, Gautham K. (eds.),
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prevent oxygen from being released into the tissues and result in hypoxemia.
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Da-Silva, Shonola S.; Sajan, Imran S.; Underwood, Joseph P. (August 2003).
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American Academy of Pediatrics and American Heart Association (2016).
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Treatment for blue baby syndrome will depend on the underlying cause.
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Pulmonary/lung disorders (ex: pulmonary hypertension, cystic fibrosis)
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Partners of the Heart: Vivien Thomas and His Work with Alfred Blalock
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The first successful operation to treat blue baby syndrome caused by
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Clinical Methods: The History, Physical, and Laboratory Examinations
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methemoglobin in the blood. This is possible because methylene blue
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than nitrates and are the chemical agent really responsible of the
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Ludlow, John T.; Wilkerson, Richard G.; Nappe, Thomas M. (2024).
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Archives of Disease in Childhood - Fetal and Neonatal Edition
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McMullen, Sarah M.; Patrick, Ward (March 2013). "Cyanosis".
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pre-ductal oxygenation, referring to oxygenation before the
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Blue baby, cyanotic infant, cyanotic baby, cyanotic newborn
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saturation is measured in the right hand and either foot.
362:. The most common congenital cause is a deficiency in the 1736: 1610: 644:
The first-line treatment for severe methemoglobinemia is
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Majumdar Deepanjan (2003). "The Blue Baby Syndrome".
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Oxygen saturation < 90 % in either extremity,
1962: 1789:"Symptoms and Diagnosis of Congenital Heart Defects" 1321:"Congenital Heart Defects – Facts about TAVPR | CDC" 1931: 1929: 1538:van Grinsven, Hans JM; Ward, Mary H = 2006 (2006). 2011:. University of Pennsylvania Press, Incorporated. 1438:EPA: United States Environmental Protection Agency 1397: 671:is the most common cyanotic cardiac heart defect. 342:can be acquired or congenital. It occurs when the 1922:. American Academy of Pediatrics. pp. 33–65. 931:. Elsevier Health Sciences. pp. 910–913.e4. 2478: 1926: 1858:"US EPA Archive Document- Nitrates and Nitrites" 1537: 1690: 1152:"Orphanet: Transposition of the great arteries" 16:Two situations that lead to cyanosis in infants 923:Enlow, Elizabeth; Greenberg, James M. (2019). 922: 2101: 1732: 1730: 1660: 1658: 1656: 1434:"National Primary Drinking Water Regulations" 2461:Anomalous aortic origin of a coronary artery 2115: 869: 867: 865: 746: 744: 632:to be pumped to the body. Many also receive 222:1. Persistent (or patent) truncus arteriosus 1596:: CS1 maint: numeric names: authors list ( 1531: 1345:"Total Anomalous Pulmonary Venous Return". 1304:: CS1 maint: numeric names: authors list ( 1241:: CS1 maint: numeric names: authors list ( 1175:: CS1 maint: numeric names: authors list ( 1109:: CS1 maint: numeric names: authors list ( 639: 611: 466:used in agricultural lands, waste dumps or 58:Note the blue coloration of the fingertips. 2108: 2094: 1727: 1653: 1325:Centers for Disease Control and Prevention 1196:Centers for Disease Control and Prevention 1130:Centers for Disease Control and Prevention 750: 446:activity, and they have a higher level of 354:from Fe to Fe, leading to poor binding of 316: 295: 274: 253: 232: 40: 1833: 1764: 1754: 1664: 1636: 1573: 1563: 1514: 1347:The Pediatric Cardiac Anesthesia Handbook 1027: 899: 862: 741: 188: 1488: 1482: 954:Textbook of Pediatric Emergency Medicine 505:One of the key tools in diagnosing is a 486: 330: 1904:from the original on 11 September 2014. 573:A screening is considered positive if: 560: 2479: 2006: 983:Neonatal respiratory distress syndrome 873: 814: 751:Pahal, Parul; Goyal, Amandeep (2024). 2089: 1913: 1911: 1673:, New York, NY: McGraw-Hill Education 1378: 1376: 1374: 1050: 956:. Lippincott Williams & Wilkins. 951: 521:, blood culture, chest X-ray, and an 462:. The sources of nitrate can include 306:Anomalous pulmonary venous connection 123: 1604: 1279:Reserved, INSERM US14 – All Rights. 1278: 1216:Reserved, INSERM US14 – All Rights. 1215: 1150:Reserved, INSERM US14 – All Rights. 1149: 1084:Reserved, INSERM US14 – All Rights. 1083: 326: 1894:"Critical Congenital Heart Defects" 1387:. Elsevier Inc. pp. 2540–2558. 1318: 1189: 1123: 1055:. Elsevier Inc. pp. 2371–2373. 13: 2196:Sinus venosus atrial septal defect 2154:Transposition of the great vessels 2037:Johns Hopkins Medical Institutions 1920:Textbook of Neonatal Resuscitation 1908: 1874:from the original on 30 April 2022 1671:Clinical Guidelines in Neonatology 1371: 243:Transposition of the great vessels 14: 2503: 2061: 1667:"Approach to the Cyanotic Infant" 1617:Environmental Health Perspectives 1495:Environmental Health Perspectives 1262:"Facts about Tetralogy of Fallot" 753:"Central and Peripheral Cyanosis" 1693:The American Journal of Medicine 1489:Fewtrell, Lorna (22 July 2004). 1067:"Facts about Truncus Arteriosus" 2055: 2025: 2000: 1983: 1956: 1886: 1850: 1781: 1684: 1452: 1426: 1338: 1312: 1281:"Orphanet: Tetralogy of Fallot" 1272: 1209: 1183: 1143: 1117: 1077: 1059: 1044: 987: 662: 116:Both of these conditions cause 2229:Atrioventricular septal defect 1086:"Orphanet: Truncus arteriosus" 970: 945: 916: 858:Blue discoloration of the skin 845: 808: 791: 450:which converts more easily to 417:microbial reduction of nitrate 113:saturation of < 85 %. 1: 2492:Syndromes affecting the heart 2171:Persistent truncus arteriosus 2142:Double outlet right ventricle 1385:Nelson Textbook of Pediatrics 1218:"Orphanet: Tricuspid atresia" 1053:Nelson Textbook of Pediatrics 929:Nelson Textbook of Pediatrics 734: 587: 178:Respiratory distress syndrome 2134:Aortopulmonary septal defect 1705:10.1016/j.amjmed.2012.11.004 1383:Smith-Whitley, Kwiatkowski. 478: 183: 7: 677: 10: 2508: 2007:Thomas, Vivien T. (1998). 1355:10.1002/9781119095569.ch16 1349:. 2017. pp. 107–111. 707:, and surgical technician 690: 2400: 2346: 2286: 2277: 2245: 2227: 2211:Ventricular septal defect 2209: 2186: 2132: 2123: 1971:. StatPearls Publishing. 1944:. StatPearls Publishing. 1464:World Health Organization 759:. StatPearls Publishing. 648:, a medication that will 168:Decreased blood perfusion 154: 90:, which is a category of 62: 48: 39: 31: 26: 2117:Congenital heart defects 1938:"Cyanotic Heart Disease" 1743:Translational Pediatrics 1460:"Water-related diseases" 1319:CDC (19 November 2019). 1266:Congenital Heart Defects 1190:CDC (19 November 2019). 1124:CDC (15 November 2019). 1071:Congenital Heart Defects 978:MedlinePlus Encyclopedia 853:MedlinePlus Encyclopedia 799:MedlinePlus Encyclopedia 782:MedlinePlus Encyclopedia 717:patent ductus arteriosus 640:Severe methemoglobinemia 628:open and allow for more 624:after birth to keep the 2456:Coronary artery anomaly 1835:10.1542/peds.112.2.e158 444:cytochrome b5 reductase 367:cytochrome b5 reductase 92:congenital heart defect 2279:Valvular heart disease 2258:Cyanotic heart disease 2201:Lutembacher's syndrome 2033:"That First Operation" 1756:10.21037/tp.2019.04.06 1565:10.1186/1476-069X-5-26 815:Snider, H. L. (1990). 787:Cyanotic heart disease 713:Blalock-Thomas-Taussig 618:cyanotic heart disease 616:Some babies born with 612:Cyanotic heart disease 495: 472:drinking water quality 336: 189:Cyanotic heart disease 144:Rapid respiratory rate 88:Cyanotic heart disease 2466:Ventricular inversion 2178:Aortopulmonary window 2147:Taussig–Bing syndrome 2064:"Information on Anna" 490: 427:of Fe into Fe in the 334: 2265:Eisenmenger syndrome 2188:Atrial septal defect 874:Silove, E D (1994). 600:(connection between 561:Prevention/Screening 313:6-12/100,000 births 2219:Tetralogy of Fallot 2125:Heart septal defect 1965:"Methemoglobinemia" 1556:2006EnvHe...5...26V 1051:Bernstein, Daniel. 1012:2016NatSR...625903L 892:10.1136/fn.70.1.f71 697:tetralogy of Fallot 669:Tetralogy of Fallot 546:tetralogy of Fallot 285:Tetralogy of Fallot 271:1-9/100,000 births 229:3-5/100,000 births 150:Shortness of breath 1440:. 30 November 2015 1412:10.1007/BF02840703 1000:Scientific Reports 725:open heart surgery 496: 415:) produced by the 337: 292:1-5/10,000 births 250:1-5/10,000 births 210:Brief Description 165:Airway obstruction 124:Signs and symptoms 81:Blue baby syndrome 27:Blue baby syndrome 2474: 2473: 2396: 2395: 2336:Ebstein's anomaly 2273: 2272: 2018:978-0-8122-1634-9 1991:Methemoglobinemia 1623:(11): 1607–1614. 1501:(14): 1371–1374. 1364:978-1-119-09553-8 1020:10.1038/srep25903 963:978-0-7817-5074-5 938:978-0-323-56888-3 830:978-0-409-90077-4 804:Methemoglobinemia 626:ductus arteriosus 620:are treated with 598:ductus arteriosus 542:electrocardiogram 511:oxygen saturation 494:on infant's foot. 340:Methemoglobinemia 327:Methemoglobinemia 324: 323: 264:Tricuspid atresia 132:Failure to thrive 98:Methemoglobinemia 78: 77: 56:, or "blue baby". 21:Medical condition 2499: 2451:Brugada syndrome 2446:Crisscross heart 2316:tricuspid valves 2294:pulmonary valves 2284: 2283: 2130: 2129: 2110: 2103: 2096: 2087: 2086: 2080: 2079: 2077: 2075: 2070:on 31 March 2016 2066:. 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Index


cyanotic
newborn
Specialty
Pediatrics
cardiac surgery
Cyanotic heart disease
congenital heart defect
Methemoglobinemia
methemoglobin
cyanosis
Respiratory distress syndrome
1. Persistent (or patent) truncus arteriosus

Transposition of the great vessels

Tricuspid atresia

Tetralogy of Fallot

Anomalous pulmonary venous connection


Methemoglobinemia
iron
hemoglobin
oxidized
oxygen
cyanosis
enzyme

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