961:
location across the left-right axis. Additional radiographic and cross-sectional imaging may be obtained to evaluate both cardiac and non-cardiac manifestations of situs ambiguus. In addition, a series of gastrointestinal tests can be conducted for observation of intestinal malrotation, as well as a scan of the liver and spleen for biliary function.
1140:. This process is favorable in patients aged 2 to 5 years old. About 20–30% of patients will require a heart transplant. Left-atrial isomeric patients have less severe complications, as they typically have 2 functional ventricles. In this case, they can undergo biventricular repair to form 2 separate ventricles and functional associated
281:
paired right atria, or left atrial isomerism consisting of two bilaterally paired left atria. Clinical features and symptoms can vary dependent upon assignment of left versus right atrial isomerism. In either instance, the apex of the heart will be poorly positioned, which should alert a clinician of
117:
of the abdominothoracic organ-vessel systems are conserved. Due to abnormal arrangement of organs in situs ambiguus, orientation across the left-right axis of the body is disrupted early in fetal development, resulting in severely flawed cardiac development and function in 50–80% of cases. They also
1167:
Mishra et al. published a review in
November 2015 describing current knowledge of cardiac and non-cardiac abnormalities associated with situs ambiguus. The author stresses the importance of genetic testing prior to deciding a prognosis for affected patients. She also proposes prenatal screening and
1155:
for patients with situs ambiguus is quite varied, owed to the spectrum of clinical manifestations. Infants who experience severe cyanosis at birth may die within hours of delivery if medical intervention is not immediate. Alternatively, longevity of neonates with mild cardiac lesions is unaffected.
960:
For proper diagnosis of situs ambiguus, cardiac and non-cardiac features must be evaluated. Diagnostic criteria for atrial isomerism includes observation of symmetry of thoracic visceral organs upon echocardiogram, arrhythmia upon electrocardiogram, and chest x-ray for confirmation of the heart's
1156:
Ten percent of patients born with right atrial isomerism die by the age of 5 without intervention. Improvements in therapies has increased the 5-year survival to 30–74% for right atrial isomeric patients and 65–84% for left atrial isomeric patients based on the cause of their disease.
1492:
Cohen, Meryl S.; Anderson, Robert H.; Cohen, Mitchell I.; Atz, Andrew M.; Fogel, Mark; Gruber, Peter J.; Lopez, Leo; Rome, Jonathan J.; Weinberg, Paul M. (2007). "Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome".
1123:
Right-atrial and left-atrial isomerism and associated pulmonary issues are treated in a series of steps based on the severity of symptoms. Isomeric patients are first treated by inserting a shunt that will move incoming blood through the pulmonary circuit. The
2091:
578:
Several genes have been identified in normal development of the right-left axis. These genes have been extensively researched. Gene mutations that lead to atrial isomerism is a growing area of research. Mutations in genes that encode proteins in the
928:
In situs ambiguus, there is a duplication of either the hyparterial or eparterial bronchus. These features are not associated with any significant clinical complications. Mechanisms leading to bronchial duplication are not thoroughly understood.
1288:
1997:
Gebbia, Marinella; Ferrero, Giovanni B.; Pilia, Giuseppe; Bassi, Maria T.; Aylsworth, Arthur S.; Penman-Splitt, Miranda; Bird, Lynne M.; Bamforth, John S.; Burn, John (1997-11-01). "X-linked situs abnormalities result from mutations in ZIC3".
1198:
study. This proved a difficult task due to the vast differences in presentation of this disorder. However, the authors are hopeful that finding a link can help inform clinical decision-making, predictive analyses, and future outcomes.
1069:
so that the intestines can be placed in non-rotated formation. It is not possible to return the bowel to a normal morphology
However, 89% of patients that undergo the Ladd surgery experience a complete resolution of symptoms.
1776:
Burkhalter MD, Sridhar A, Sampaio P, Jacinto R, Burczyk MS, Donow C, Angenendt M, Competence
Network for Congenital Heart Defects Investigators, Hempel M, Walther P, Pennekamp P, Omran H, Lopes SS, Ware SM, Philipp M (2019).
550:, which controls left-right patterning of visceral organs across the body axis, have been indicated in sporadic and familial cases of atrial isomerism. There is also overlap between genes associated with situs ambiguus and
526:). Asplenia is most often observed in patients with right atrial isomerism. Polysplenia results in 90% of patients with left atrial isomerism. Although they have many spleens, each is usually ineffective resulting in
2117:
1081:
can be considered based on the overall health of the patient. The Kasai procedure is successful in approximately 80% of patients. Following the operation, patients are advised to take fat-soluble vitamins,
2143:
510:
and blood in the stool. Patients may also experience abdominal pain. Intestinal malrotation is more commonly identified in patients with right atrial isomerism than in those with left atrial isomerism.
373:
Abnormal development of the heart results in impaired doubles of conductive nodes, as well as faulty electrical fibers throughout the ventricles. Individuals with right atrial isomerism develop two
90:, which is normal asymmetric distribution of the abdominothoracic visceral organs. Situs ambiguus can also be subdivided into left-isomerism and right isomerism based on the defects observed in the
2169:
377:, as they have 2 mirrored right atria, whereas those with left atrial isomerism fail to develop a sinus node at all. Thus, patients with left atrial isomerism are more likely to experience
325:
which distinguishes the 2 atria is absent. These impairments, in addition to congestion in the pulmonary tract, allows deoxygenated blood to mix with oxygenated blood, contributing to
1571:
Maeyama K, Kosaki R, Yoshihashi H, Casey B, Kosaki K (March 2001). "Mutation analysis of left-right axis determining genes in NOD and ICR, strains susceptible to maternal diabetes".
86:, which results when arrangement of all the organs in the abdomen and chest are mirrored, so the positions are opposite the normal placement. Situs inversus is the mirror image of
910:
Pathophysiology in the bronchial tree can be observed by radiography. Under normal development, the bronchial tree consists of two main bronchi that are anatomically different:
455:
is typically symmetrical across the left-right axis in patients with situs ambiguus, which is abnormal. A majority of left atrial isomeric patients have defects throughout the
1457:
Van Praagh, R; et al. (December 15, 1990). "Atrial isomerism in the heterotaxy syndromes with asplenia, or polysplenia, or normally formed spleen: an erroneous concept".
1077:
is usually performed in cases of biliary atresia. In this surgery, a Y-shaped shunt is used to passage bile from the liver directly to the intestine. If this is unsuccessful,
1940:
Mohapatra, Bhagyalaxmi; Casey, Brett; Li, Hua; Ho-Dawson, Trang; Smith, Liana; Fernbach, Susan D.; Molinari, Laura; Niesh, Stephen R.; Jefferies, John Lynn (2009-03-01).
2478:
Lin, Angela E.; Krikov, Sergey; Riehle-Colarusso, Tiffany; FrĂas, Jaime L.; Belmont, John; Anderka, Marlene; Geva, Tal; Getz, Kelly D.; Botto, Lorenzo D. (2014-10-01).
1826:
1164:
There have been vast amounts of research on the clinical features, racial disparities, and physiological mechanisms of heterotaxy syndrome dating back to 1973.
282:
the likelihood of atrial isomerism. It is estimated that 5–10% of isomeric patients have mesocardia, in which the heart is positioned at the center of the
82:
and arrangement of the visceral organs; however, classical heterotaxy requires multiple organs to be affected. This does not include the congenital defect
2390:
Lopez, Keila N.; Marengo, Lisa K.; Canfield, Mark A.; Belmont, John W.; Dickerson, Heather A. (2015-09-01). "Racial disparities in heterotaxy syndrome".
1641:
Kuehl KS, Loffredo CA (March 2003). "Population-based study of l-transposition of the great arteries: possible associations with environmental factors".
1249:
349:
Left atrial appendage isomerism, also called left atrial isomerism, is a cardiac development defect in which the heart has two bilateral left atria and
2757:
263:
1179:
descent, as well as female infants of non-Hispanic black and white mothers. Additional studies must be done to clarify the mechanisms behind
2433:
Kim, Soo-Jin; Kim, Woong-Han; Lim, Hong-Gook; Lee, Jae-Young (July 2008). "Outcome of 200 patients after an extracardiac Fontan procedure".
2615:
1885:"Characterization and mutation analysis of human LEFTY A and LEFTY B, homologues of murine genes implicated in left-right axis development"
2780:
530:. Rarely, left atrial isomeric patients have a single, normal, functional spleen. Patients lacking a functional spleen are in danger of
2668:
776:
562:
has an important role in positioning these cilia, and thus genes within this pathway are increasingly associated with situs ambiguus.
2775:
2298:
Hashmi, Aijaz; Abu-Sulaiman, Riyadh; McCrindle, Brian W; Smallhorn, Jeffrey F; Williams, William G; Freedom, Robert M (1998-04-01).
1183:
in heterotaxy syndrome. Individuals of Asian descent show a higher prevalence of heterotaxy syndrome in general than members of the
1180:
2675:
1676:
Sutherland, Mardi J.; Ware, Stephanie M. (15 November 2009). "Disorders of left-right asymmetry: Heterotaxy and situs inversus".
1549:
1942:"Identification and functional characterization of NODAL rare variants in heterotaxy and isolated cardiovascular malformations"
600:
580:
547:
2480:"Laterality defects in the national birth defects prevention study (1998–2007): Birth prevalence and descriptive epidemiology"
2794:
555:
1414:
Freedom, Robert M.; Treves, S. (1973-05-01). "Splenic
Scintigraphy and Radionuclide Venography in the Heterotaxy Syndrome".
2895:
1190:
The
National Birth Defects Prevention study (October 2014) attempted to link clinical presentations of situs ambiguus to
2245:"Polysplenia syndrome and sickle cell trait: extensive deep venous thrombosis from venous stasis and hypercoagulability"
445:
437:
353:
in the muscle wall. Left atrial isomerism can have varied clinical manifestations, including a later onset of symptoms.
255:
109:
Individuals with situs inversus or situs solitus do not experience fatal dysfunction of their organ systems, as general
985:
551:
2692:
781:
448:
of the body. Distribution of these organs largely dictates treatment, clinical outcomes, and further evaluation.
2608:
1840:
Chen, CM; et al. (April 2010). "Transcriptional
Control of Left–Right Patterning in Cardiac Development".
1109:
990:
933:
414:
168:
743:
393:
largely depends on physiological looping of the ventricles. Abnormal looping of the ventricles contributes to
1536:
MartĂnez-FrĂas ML (March 2001). "Heterotaxia as an outcome of maternal diabetes: an epidemiological study".
1209:
314:
278:
2581:
1606:
Kuehl KS, Loffredo C (November 2002). "Risk factors for heart disease associated with abnormal sidedness".
1117:
1113:
1074:
949:
851:
498:
of the entire intestinal tract, or improper folding and bulging of the stomach and intestines, results in
495:
310:
in the muscle wall, as opposed to the normal right atrium and left atrium. In right atrial isomerism, the
274:
227:
176:
154:
483:
become a major concern. Biliary atresia is not usually observed in patients with right atrial isomerism.
475:, pale stools, dark urine, and abdominal swelling. If this condition continues without proper treatment,
171:
cases. Additional estimation of incidence and prevalence of isomerism proves difficult due to failure to
1820:
1078:
386:
258:. Poor positioning of the intestine also makes it more prone to blockage, which can result in numerous
222:
may raise further suspicion of a cardiac abnormality. Non-cardiac symptoms include impairments of the
1057:
Each of the symptoms of situs ambiguus must be managed with appropriate treatment dependent upon the
785:
559:
503:
491:
119:
114:
79:
611:
family transcription factor, is linked to a 50% risk of atrial isomerism in families. It is also an
2601:
1289:"Anatomy, clinical manifestations and diagnosis of heterotaxy (isomerism of the atrial appendages)"
1093:
980:
975:
921:
771:
596:
527:
378:
330:
142:
2196:""Contemporary outcomes after the Fontan procedure: A Pediatric Heart Network multicenter study.""
1096:
patients have an elevated lifetime risk of sepsis, as they have no functional spleen for fighting
202:
or blue skin coloration, primarily affecting the lips and fingernails, can indicate a systemic or
2900:
2372:
2280:
2023:
1865:
1758:
1701:
1518:
1137:
876:
394:
358:
334:
322:
290:, in which the apex of the heart is pointed toward the right side of the thorax, and 50–70% have
215:
203:
2687:
2517:
2499:
2460:
2415:
2407:
2364:
2356:
2321:
2272:
2264:
2225:
2072:
2064:
2015:
1979:
1961:
1922:
1904:
1857:
1808:
1750:
1693:
1658:
1623:
1588:
1553:
1510:
1474:
1439:
1431:
1396:
1344:
1336:
1129:
1087:
1058:
863:
499:
362:
259:
211:
207:
63:
38:
179:
is much more easily recognized than left isomerism, contributing to the failure to diagnose.
2704:
2507:
2491:
2450:
2442:
2399:
2348:
2339:
Mishra, Smita (2015-11-26). "Cardiac and Non-Cardiac
Abnormalities in Heterotaxy Syndrome".
2311:
2256:
2215:
2207:
2054:
2007:
1969:
1953:
1912:
1896:
1849:
1798:
1790:
1740:
1732:
1685:
1650:
1615:
1580:
1545:
1502:
1466:
1423:
1386:
1378:
1326:
1214:
1125:
970:
843:
191:
146:
1883:
Kosaki, K; Bassi, M T; Kosaki, R; Lewin, M; Belmont, J; Schauer, G; Casey, B (1999-03-01).
2745:
2738:
2721:
2682:
2059:
2042:
1105:
1046:
1023:
941:
890:
374:
318:
231:
150:
67:
2041:
Applegate, Kimberly E.; Goske, Marilyn J.; Pierce, Gregory; Murphy, Daniel (1999-07-01).
1062:
546:
use, suggesting both genetic and environmental factors play a role. Several genes in the
1245:
2843:
2512:
2479:
2220:
2195:
1974:
1941:
1917:
1884:
1803:
1778:
1745:
1720:
1391:
1366:
1219:
1031:
382:
167:
occurs in 1 out of every 10,000 live births and is associated with approximately 3% of
99:
83:
55:
2316:
2299:
618:
The most prevalent and best characterized genetic associations of heterotaxy include:
2889:
2833:
2656:
2376:
2284:
1762:
1522:
1470:
1224:
1184:
1100:. For this reason, asplenic patients are under constant observation for any signs of
812:
588:
515:
480:
472:
456:
390:
354:
87:
2244:
2027:
1705:
2823:
2260:
1195:
1017:
869:
307:
287:
235:
219:
126:
1869:
273:
Due to abnormal cardiac development, patients with situs ambiguus usually develop
1779:"Imbalanced mitochondrial function provokes heterotaxy via aberrant ciliogenesis"
182:
Situs ambiguus is a growing field of research with findings dating back to 1973.
2446:
1141:
999:
994:
896:
886:
827:
612:
608:
542:
Situs ambiguus has been linked to family history of malformations and maternal
523:
464:
398:
350:
294:, in which the apex of the heart is pointed toward the left side of the thorax.
267:
2539:
2211:
2872:
2865:
2853:
2816:
2726:
2716:
2711:
2624:
2352:
1853:
1736:
1506:
1382:
1191:
1083:
1066:
859:
468:
413:
is not typically damaging and presents no significant clinical complications.
291:
43:
2503:
2411:
2360:
2268:
2068:
1965:
1908:
1435:
1340:
565:
Disrupted mitochondria function has also been recently linked to heterotaxy.
514:
Isomeric patients often experience disruptions to splenic development during
2544:
1331:
1314:
1152:
1097:
476:
239:
164:
130:
123:
2521:
2464:
2419:
2368:
2276:
2229:
2092:"Management and outcome of heterotaxy (isomerism of the atrial appendages)"
2076:
1983:
1926:
1861:
1812:
1754:
1721:"Planar cell polarity signaling in the development of left–right asymmetry"
1697:
1662:
1627:
1592:
1557:
1514:
1400:
1348:
885:
Intrahepatic interruption of the inferior vena cava with connection to the
467:
is functional and morphologically normal. This biliary atresia can lead to
302:
Right atrial appendage isomerism, also called right atrial isomerism, is a
2559:
2325:
2019:
1478:
1443:
1104:
or infection. In the case of infection, patients are placed on controlled
2576:
2495:
2300:"Management and Outcomes of Right Atrial Isomerism: A 26-Year Experience"
1957:
1689:
1678:
American
Journal of Medical Genetics Part C: Seminars in Medical Genetics
1176:
1172:
945:
915:
855:
837:
823:
519:
410:
326:
247:
243:
199:
172:
2455:
2403:
2011:
1654:
1619:
1550:
10.1002/1096-8628(2000)9999:999<00::AID-AJMG1139>3.0.CO;2-Z
797:
543:
487:
433:
425:
251:
138:
110:
75:
2243:
Shahrouki, Puja; Lee, Edward
Wolfgang; Ruehm, Stefan (February 2022).
1584:
1427:
918:(below the pulmonary artery): supplies blood to the bi-lobed left lung
2806:
1794:
831:
801:
748:
709:
615:, so testing for ZIC3 mutations is highly encouraged in male births.
531:
441:
381:, or irregular rapid heart beat, and abnormal heart rhythm, known as
283:
161:
91:
2170:"Clinical features and management of sepsis in the asplenic patient"
1171:
Recent studies have shown higher rates of heterotaxy syndrome among
924:(adjacent to the artery): supplies blood to the tri-lobed right lung
306:
development defect in which the heart has bilateral right atria and
78:. Clinically, heterotaxy spectrum generally refers to any defect of
1900:
846:
develop, which can lead to cancer, kidney stones, and/or infection.
1101:
808:
765:
729:
689:
584:
507:
452:
429:
338:
303:
223:
195:
190:
There are a variety of clinical manifestations of situs ambiguus.
134:
103:
95:
71:
1643:
Birth
Defects Research. Part A, Clinical and Molecular Teratology
1061:
involved. Intestinal malrotation is treated surgically using the
2392:
Birth Defects Research Part A: Clinical and Molecular Teratology
1313:
Zhu, Lirong; Belmont, John W.; Ware, Stephanie M. (2005-10-26).
1133:
937:
723:
703:
683:
669:
663:
649:
643:
627:
604:
592:
460:
311:
214:
may also be observed due to poor circulation. Upon examination,
2597:
1168:
evaluation in cases at risk for development of situs ambiguus.
175:
and underestimation of the disease by clinicians. Furthermore,
2593:
254:
region are features that suggest improper positioning of the
554:, likely due to the important role of cilia in establishing
490:
is often one of the first signals of situs ambiguus upon
361:
is disrupted due to the inappropriate morphology of the
2194:
Anderson PA, Sleeper LA, Mahony L, et al. (2008).
2090:
Lowenthal, Alexander; et al. (October 27, 2015).
2168:
Pasternack, Mark S; et al. (December 11, 2014).
2043:"Situs Revisited: Imaging of the Heterotaxy Syndrome"
16:
Abnormal placement of organs in the chest and abdomen
2549:
2567:
2553:
270:are also possible features of heterotaxy syndrome.
37:
29:
24:
2435:The Journal of Thoracic and Cardiovascular Surgery
157:considered to have a form of heterotaxy syndrome.
1287:Lowenthal, A.; et al. (September 26, 2015).
2142:Erlichman, Jessi; et al. (August 3, 2015).
337:also results due to improper positioning of the
2116:Brandt, Mary; et al. (October 24, 2015).
417:results in issues of blood flow to the lungs.
2609:
2304:Journal of the American College of Cardiology
2200:Journal of the American College of Cardiology
8:
1825:: CS1 maint: multiple names: authors list (
1006:Diagnostic techniques for non-cardiac causes
936:, there is a reduction in blood flow to the
141:with situs ambiguus lack lateralization and
2484:American Journal of Medical Genetics Part A
2616:
2602:
2594:
2550:
1250:United States National Library of Medicine
1128:routes blood through the patient's single
940:due to an obstruction of the heart at the
882:Bilateral superior or inferior venae cavae
21:
2511:
2454:
2315:
2219:
2058:
1973:
1916:
1802:
1744:
1390:
1330:
1112:. This therapy battles infection by both
518:, resulting in an overall lack a spleen (
2633:
965:Diagnostic techniques for cardiac causes
620:
444:may be randomly arranged throughout the
33:Situs ambiguous, heterotaxy, heterotaxia
1719:Axelrod, Jeffrey D. (1 February 2020).
1237:
1818:
1065:. This procedure widens a fold in the
815:are occasionally found in the midline.
70:are distributed abnormally within the
2060:10.1148/radiographics.19.4.g99jl31837
502:. This impairment leads to vomiting,
7:
1538:American Journal of Medical Genetics
1360:
1358:
1282:
1280:
1278:
1276:
1274:
1272:
1270:
1268:
1266:
1028:Evaluation of intestinal malrotation
793:Deranged abdominal organ asymmetry:
317:tract is damaged due to right-left
1889:American Journal of Human Genetics
1319:European Journal of Human Genetics
1022:Evaluation of biliary anatomy and
777:transposition of the great vessels
522:) or development of many spleens (
14:
2341:The Indian Journal of Pediatrics
1315:"Genetics of human heterotaxias"
574:Molecular and cellular mechanism
298:Right atrial appendage isomerism
1725:Current Opinion in Cell Biology
601:hypoplastic left heart syndrome
357:is often a concern because the
345:Left atrial appendage isomerism
2795:Edinburgh Handedness Inventory
2669:Geschwind–Galaburda hypothesis
2261:10.1016/j.clinimag.2021.11.017
862:attachments, which completely
804:are prone to isolated reversal
118:experience complications with
1:
2317:10.1016/S0735-1097(98)00062-X
1088:anti-inflammatory medications
836:More rarely, the head of the
1471:10.1016/0002-9149(90)90543-a
1043:Assessment of family history
369:Conductive nodes and tissues
194:symptoms can be due to both
66:in which the major visceral
58: 'ambiguous site'), or
2447:10.1016/j.jtcvs.2007.12.032
1073:Following cholangiogram, a
459:, which is responsible for
321:of blood. In addition, the
2917:
2212:10.1016/j.jacc.2008.01.074
1371:Korean Circulation Journal
1106:empiric antibiotic therapy
1014:Magnetic resonance imaging
986:Magnetic resonance imaging
552:primary ciliary dyskinesia
486:Random positioning of the
471:problems such as nutrient
463:production, even when the
365:to support the vena cava.
2861:Footedness in boardsports
2852:
2805:
2793:
2772:
2703:
2679:
2667:
2631:
2353:10.1007/s12098-015-1925-x
1854:10.1007/s00246-009-9610-3
1737:10.1016/j.ceb.2019.09.002
1507:10.1017/s104795110700114x
1383:10.4070/kcj.2011.41.5.227
1038:Other diagnostic features
1018:Splenic function analysis
198:and non-cardiac defects.
160:Heterotaxy syndrome with
2118:"Intestinal Malrotation"
1946:Human Molecular Genetics
1108:to avoid development of
934:pulmonary valve stenosis
906:Bronchial tree and lungs
415:Pulmonary valve stenosis
405:Bronchial tree and lungs
169:congenital heart disease
2665:In cognitive abilities
1495:Cardiology in the Young
1332:10.1038/sj.ejhg.5201506
1210:Chirality (mathematics)
991:Cardiac catheterization
768:looping malformations:
534:and must be monitored.
238:and destruction of the
2790:Handedness measurement
950:pulmonary hypertension
944:. This contributes to
607:gene, which encodes a
595:, have been linked to
275:right atrial isomerism
228:gastrointestinal tract
2769:Handedness related to
1367:"Heterotaxy Syndrome"
1365:Kim, Soo-Jin (2011).
1246:"heterotaxy syndrome"
1110:antibiotic resistance
1094:Functionally asplenic
1079:liver transplantation
820:Organ malformations:
786:atrial septal defects
387:atrioventricular node
385:. Development of the
206:issue. Poor feeding,
2896:Congenital disorders
2781:Mathematical ability
2753:Handedness in people
2734:Handedness in boxing
2496:10.1002/ajmg.a.36695
1842:Pediatric Cardiology
1690:10.1002/ajmg.c.30228
1138:systemic circulation
560:planar cell polarity
556:left-right asymmetry
504:abdominal distension
335:systemic circulation
331:respiratory distress
250:and swelling of the
80:left-right asymmetry
2802:Handedness genetics
1011:Computed tomography
981:Computed tomography
976:Electrocardiography
922:Eparterial bronchus
760:Classical pathology
603:. Mutations in the
597:tetralogy of Fallot
528:functional asplenia
379:atrial fibrillation
2776:Sexual orientation
2568:External resources
2404:10.1002/bdra.23416
2012:10.1038/ng1197-305
1958:10.1093/hmg/ddn411
1655:10.1002/bdra.10015
1620:10.1002/tera.10099
1181:racial disparities
877:inferior vena cava
864:obstruct the bowel
772:Fallot's tetralogy
359:inferior vena cava
308:atrial attachments
277:consisting of two
186:Signs and symptoms
2883:
2882:
2878:
2877:
2830:In major viscera
2688:Dual brain theory
2591:
2590:
2490:(10): 2581–2591.
2347:(12): 1135–1146.
2144:"Biliary Atresia"
1585:10.1002/tera.1022
1428:10.1148/107.2.381
844:Horseshoe kidneys
757:
756:
613:X-linked disorder
500:bowel obstruction
409:Isomerism of the
351:atrial appendages
212:shallow breathing
208:failure to thrive
153:cavities and are
145:of organs in the
64:congenital defect
49:
48:
19:Medical condition
2908:
2727:Right-handedness
2705:Ocular dominance
2634:
2618:
2611:
2604:
2595:
2551:
2526:
2525:
2515:
2475:
2469:
2468:
2458:
2430:
2424:
2423:
2387:
2381:
2380:
2336:
2330:
2329:
2319:
2310:(5): 1120–1126.
2295:
2289:
2288:
2249:Clinical Imaging
2240:
2234:
2233:
2223:
2191:
2185:
2184:
2182:
2180:
2165:
2159:
2158:
2156:
2154:
2139:
2133:
2132:
2130:
2128:
2113:
2107:
2106:
2104:
2102:
2087:
2081:
2080:
2062:
2038:
2032:
2031:
1994:
1988:
1987:
1977:
1937:
1931:
1930:
1920:
1880:
1874:
1873:
1837:
1831:
1830:
1824:
1816:
1806:
1795:10.1172/JCI98890
1789:(7): 2841–2855.
1773:
1767:
1766:
1748:
1716:
1710:
1709:
1673:
1667:
1666:
1638:
1632:
1631:
1603:
1597:
1596:
1568:
1562:
1561:
1533:
1527:
1526:
1489:
1483:
1482:
1454:
1448:
1447:
1411:
1405:
1404:
1394:
1362:
1353:
1352:
1334:
1310:
1304:
1303:
1301:
1299:
1284:
1261:
1260:
1258:
1256:
1242:
1215:Ivemark syndrome
1126:Fontan procedure
971:Echocardiography
891:hemiazygos veins
621:
581:TGF-beta pathway
548:TGF-beta pathway
438:intestinal tract
428:, including the
426:Abdominal organs
421:Abdominal organs
375:sinoatrial nodes
133:, and sometimes
22:
2916:
2915:
2911:
2910:
2909:
2907:
2906:
2905:
2886:
2885:
2884:
2879:
2746:Orthodox stance
2739:Southpaw stance
2722:Cross-dominance
2717:Left-handedness
2683:Brain asymmetry
2627:
2622:
2592:
2587:
2586:
2563:
2562:
2535:
2530:
2529:
2477:
2476:
2472:
2432:
2431:
2427:
2398:(11): 941–950.
2389:
2388:
2384:
2338:
2337:
2333:
2297:
2296:
2292:
2242:
2241:
2237:
2193:
2192:
2188:
2178:
2176:
2167:
2166:
2162:
2152:
2150:
2141:
2140:
2136:
2126:
2124:
2115:
2114:
2110:
2100:
2098:
2089:
2088:
2084:
2040:
2039:
2035:
2000:Nature Genetics
1996:
1995:
1991:
1939:
1938:
1934:
1882:
1881:
1877:
1839:
1838:
1834:
1817:
1775:
1774:
1770:
1718:
1717:
1713:
1675:
1674:
1670:
1640:
1639:
1635:
1605:
1604:
1600:
1570:
1569:
1565:
1535:
1534:
1530:
1491:
1490:
1486:
1456:
1455:
1451:
1413:
1412:
1408:
1364:
1363:
1356:
1312:
1311:
1307:
1297:
1295:
1286:
1285:
1264:
1254:
1252:
1244:
1243:
1239:
1234:
1229:
1205:
1196:epidemiological
1162:
1150:
1075:Kasai procedure
1055:
1047:Genetic testing
1040:
1008:
967:
958:
908:
872:abnormalities:
811:, and a single
762:
576:
571:
569:Pathophysiology
540:
446:left-right axis
423:
407:
371:
347:
300:
262:health issues.
232:Biliary atresia
188:
177:right isomerism
20:
17:
12:
11:
5:
2914:
2912:
2904:
2903:
2898:
2888:
2887:
2881:
2880:
2876:
2875:
2870:
2868:
2863:
2857:
2856:
2851:
2847:
2846:
2844:Situs inversus
2841:
2839:Situs ambiguus
2836:
2831:
2827:
2826:
2821:
2819:
2814:
2810:
2809:
2804:
2798:
2797:
2792:
2786:
2785:
2784:
2783:
2778:
2771:
2765:
2764:
2762:
2760:
2755:
2749:
2748:
2743:
2741:
2736:
2730:
2729:
2724:
2719:
2714:
2708:
2707:
2702:
2698:
2697:
2696:
2695:
2690:
2685:
2678:
2672:
2671:
2666:
2662:
2661:
2659:
2654:
2652:
2648:
2647:
2644:
2641:
2638:
2632:
2629:
2628:
2623:
2621:
2620:
2613:
2606:
2598:
2589:
2588:
2585:
2584:
2572:
2571:
2569:
2565:
2564:
2558:
2557:
2555:
2554:Classification
2548:
2547:
2534:
2533:External links
2531:
2528:
2527:
2470:
2441:(1): 108–116.
2425:
2382:
2331:
2290:
2235:
2186:
2160:
2134:
2108:
2082:
2053:(4): 837–852.
2033:
2006:(3): 305–308.
1989:
1952:(5): 861–871.
1932:
1901:10.1086/302289
1895:(3): 712–721.
1875:
1848:(3): 371–377.
1832:
1768:
1711:
1684:(4): 307–317.
1668:
1633:
1598:
1563:
1528:
1484:
1465:(20): 1504–6.
1449:
1422:(2): 381–386.
1406:
1354:
1305:
1262:
1236:
1235:
1233:
1230:
1228:
1227:
1222:
1220:Situs inversus
1217:
1212:
1206:
1204:
1201:
1161:
1158:
1149:
1146:
1063:Ladd procedure
1054:
1051:
1050:
1049:
1044:
1039:
1036:
1035:
1034:
1032:Pulse oximeter
1029:
1026:
1020:
1015:
1012:
1007:
1004:
1003:
1002:
997:
988:
983:
978:
973:
966:
963:
957:
954:
942:pulmonic valve
926:
925:
919:
907:
904:
903:
902:
901:
900:
893:
883:
880:
867:
858:and/or faulty
849:
848:
847:
841:
834:
830:often lead to
818:
817:
816:
805:
791:
790:
789:
779:
774:
761:
758:
755:
754:
751:
746:
741:
737:
736:
733:
726:
721:
717:
716:
713:
706:
701:
697:
696:
693:
686:
681:
677:
676:
673:
666:
661:
657:
656:
653:
646:
641:
637:
636:
633:
630:
625:
575:
572:
570:
567:
539:
536:
422:
419:
411:bronchial tree
406:
403:
383:atrial flutter
370:
367:
363:left ventricle
346:
343:
299:
296:
286:, 25–50% have
242:, may lead to
187:
184:
129:, significant
84:situs inversus
52:Situs ambiguus
47:
46:
41:
35:
34:
31:
27:
26:
25:Situs ambiguus
18:
15:
13:
10:
9:
6:
4:
3:
2:
2913:
2902:
2899:
2897:
2894:
2893:
2891:
2874:
2871:
2869:
2867:
2864:
2862:
2859:
2858:
2855:
2849:
2848:
2845:
2842:
2840:
2837:
2835:
2834:Situs solitus
2832:
2829:
2828:
2825:
2822:
2820:
2818:
2815:
2812:
2811:
2808:
2803:
2800:
2799:
2796:
2791:
2788:
2787:
2782:
2779:
2777:
2774:
2773:
2770:
2767:
2766:
2763:
2761:
2759:
2756:
2754:
2751:
2750:
2747:
2744:
2742:
2740:
2737:
2735:
2732:
2731:
2728:
2725:
2723:
2720:
2718:
2715:
2713:
2710:
2709:
2706:
2700:
2699:
2694:
2691:
2689:
2686:
2684:
2681:
2680:
2677:
2674:
2673:
2670:
2664:
2663:
2660:
2658:
2657:Ambidexterity
2655:
2653:
2650:
2649:
2645:
2642:
2639:
2636:
2635:
2630:
2626:
2619:
2614:
2612:
2607:
2605:
2600:
2599:
2596:
2583:
2579:
2578:
2574:
2573:
2570:
2566:
2561:
2556:
2552:
2546:
2542:
2541:
2537:
2536:
2532:
2523:
2519:
2514:
2509:
2505:
2501:
2497:
2493:
2489:
2485:
2481:
2474:
2471:
2466:
2462:
2457:
2452:
2448:
2444:
2440:
2436:
2429:
2426:
2421:
2417:
2413:
2409:
2405:
2401:
2397:
2393:
2386:
2383:
2378:
2374:
2370:
2366:
2362:
2358:
2354:
2350:
2346:
2342:
2335:
2332:
2327:
2323:
2318:
2313:
2309:
2305:
2301:
2294:
2291:
2286:
2282:
2278:
2274:
2270:
2266:
2262:
2258:
2254:
2250:
2246:
2239:
2236:
2231:
2227:
2222:
2217:
2213:
2209:
2205:
2201:
2197:
2190:
2187:
2175:
2171:
2164:
2161:
2149:
2145:
2138:
2135:
2123:
2119:
2112:
2109:
2097:
2093:
2086:
2083:
2078:
2074:
2070:
2066:
2061:
2056:
2052:
2048:
2047:RadioGraphics
2044:
2037:
2034:
2029:
2025:
2021:
2017:
2013:
2009:
2005:
2001:
1993:
1990:
1985:
1981:
1976:
1971:
1967:
1963:
1959:
1955:
1951:
1947:
1943:
1936:
1933:
1928:
1924:
1919:
1914:
1910:
1906:
1902:
1898:
1894:
1890:
1886:
1879:
1876:
1871:
1867:
1863:
1859:
1855:
1851:
1847:
1843:
1836:
1833:
1828:
1822:
1814:
1810:
1805:
1800:
1796:
1792:
1788:
1784:
1783:J Clin Invest
1780:
1772:
1769:
1764:
1760:
1756:
1752:
1747:
1742:
1738:
1734:
1730:
1726:
1722:
1715:
1712:
1707:
1703:
1699:
1695:
1691:
1687:
1683:
1679:
1672:
1669:
1664:
1660:
1656:
1652:
1648:
1644:
1637:
1634:
1629:
1625:
1621:
1617:
1613:
1609:
1602:
1599:
1594:
1590:
1586:
1582:
1579:(3): 119–26.
1578:
1574:
1567:
1564:
1559:
1555:
1551:
1547:
1543:
1539:
1532:
1529:
1524:
1520:
1516:
1512:
1508:
1504:
1501:(S2): 29–43.
1500:
1496:
1488:
1485:
1480:
1476:
1472:
1468:
1464:
1460:
1453:
1450:
1445:
1441:
1437:
1433:
1429:
1425:
1421:
1417:
1410:
1407:
1402:
1398:
1393:
1388:
1384:
1380:
1377:(5): 227–32.
1376:
1372:
1368:
1361:
1359:
1355:
1350:
1346:
1342:
1338:
1333:
1328:
1324:
1320:
1316:
1309:
1306:
1294:
1290:
1283:
1281:
1279:
1277:
1275:
1273:
1271:
1269:
1267:
1263:
1251:
1247:
1241:
1238:
1231:
1226:
1225:Situs solitus
1223:
1221:
1218:
1216:
1213:
1211:
1208:
1207:
1202:
1200:
1197:
1193:
1188:
1186:
1185:Western world
1182:
1178:
1174:
1169:
1165:
1159:
1157:
1154:
1147:
1145:
1143:
1139:
1135:
1131:
1127:
1121:
1119:
1118:gram-negative
1115:
1114:gram-positive
1111:
1107:
1103:
1099:
1095:
1091:
1089:
1085:
1080:
1076:
1071:
1068:
1064:
1060:
1052:
1048:
1045:
1042:
1041:
1037:
1033:
1030:
1027:
1025:
1024:cholangiogram
1021:
1019:
1016:
1013:
1010:
1009:
1005:
1001:
998:
996:
992:
989:
987:
984:
982:
979:
977:
974:
972:
969:
968:
964:
962:
955:
953:
951:
947:
943:
939:
935:
930:
923:
920:
917:
913:
912:
911:
905:
898:
894:
892:
888:
884:
881:
878:
874:
873:
871:
868:
865:
861:
857:
854:errors cause
853:
850:
845:
842:
840:fails to form
839:
835:
833:
829:
825:
822:
821:
819:
814:
813:adrenal gland
810:
807:The stomach,
806:
803:
799:
795:
794:
792:
787:
783:
780:
778:
775:
773:
770:
769:
767:
764:
763:
759:
752:
750:
747:
745:
742:
739:
738:
734:
732:
731:
727:
725:
722:
719:
718:
714:
712:
711:
707:
705:
702:
699:
698:
694:
692:
691:
687:
685:
682:
679:
678:
674:
672:
671:
667:
665:
662:
659:
658:
654:
652:
651:
647:
645:
642:
639:
638:
634:
631:
629:
626:
623:
622:
619:
616:
614:
610:
606:
602:
598:
594:
590:
586:
582:
573:
568:
566:
563:
561:
557:
553:
549:
545:
537:
535:
533:
529:
525:
521:
517:
516:embryogenesis
512:
509:
505:
501:
497:
493:
489:
484:
482:
481:liver failure
478:
474:
473:malabsorption
470:
466:
462:
458:
454:
449:
447:
443:
439:
435:
431:
427:
420:
418:
416:
412:
404:
402:
400:
396:
392:
391:bundle of His
388:
384:
380:
376:
368:
366:
364:
360:
356:
355:Heart failure
352:
344:
342:
340:
336:
332:
329:and possible
328:
324:
323:atrial septum
320:
316:
313:
309:
305:
297:
295:
293:
289:
285:
280:
276:
271:
269:
265:
261:
257:
253:
249:
245:
241:
237:
233:
229:
225:
221:
217:
213:
209:
205:
201:
197:
193:
185:
183:
180:
178:
174:
170:
166:
163:
158:
156:
152:
148:
144:
140:
136:
132:
128:
127:blood vessels
125:
121:
116:
112:
107:
105:
101:
97:
93:
89:
88:situs solitus
85:
81:
77:
73:
69:
65:
61:
57:
53:
45:
42:
40:
36:
32:
28:
23:
2866:Regular foot
2860:
2838:
2824:Dextrocardia
2801:
2789:
2768:
2752:
2733:
2693:Bicameralism
2575:
2538:
2487:
2483:
2473:
2438:
2434:
2428:
2395:
2391:
2385:
2344:
2340:
2334:
2307:
2303:
2293:
2252:
2248:
2238:
2206:(2): 85–93.
2203:
2199:
2189:
2179:December 10,
2177:. Retrieved
2173:
2163:
2151:. Retrieved
2147:
2137:
2127:December 10,
2125:. Retrieved
2121:
2111:
2099:. Retrieved
2095:
2085:
2050:
2046:
2036:
2003:
1999:
1992:
1949:
1945:
1935:
1892:
1888:
1878:
1845:
1841:
1835:
1821:cite journal
1786:
1782:
1771:
1728:
1724:
1714:
1681:
1677:
1671:
1649:(3): 162–7.
1646:
1642:
1636:
1614:(5): 242–8.
1611:
1607:
1601:
1576:
1572:
1566:
1544:(2): 142–6.
1541:
1537:
1531:
1498:
1494:
1487:
1462:
1459:Am J Cardiol
1458:
1452:
1419:
1415:
1409:
1374:
1370:
1325:(1): 17–25.
1322:
1318:
1308:
1296:. Retrieved
1292:
1253:. Retrieved
1240:
1192:demographics
1189:
1170:
1166:
1163:
1151:
1122:
1092:
1072:
1059:organ system
1056:
959:
931:
927:
914:Hyparterial
909:
897:portal veins
875:Interrupted
728:
708:
688:
668:
648:
617:
583:, including
577:
564:
541:
513:
485:
465:gall bladder
457:biliary tree
450:
424:
408:
401:in fetuses.
372:
348:
315:blood oxygen
301:
288:dextrocardia
272:
236:inflammation
220:heart murmur
210:, and rapid
189:
181:
159:
108:
62:, is a rare
59:
51:
50:
2456:10371/60103
2255:: 127–131.
2153:December 9,
2101:December 8,
1298:November 4,
1175:infants of
1136:, and into
1084:choleretics
1000:Chest x-ray
995:angiography
852:Malrotation
828:polysplenia
782:Ventricular
609:zinc finger
524:polysplenia
496:Malrotation
492:examination
399:heart block
279:bilaterally
268:polysplenia
204:circulatory
30:Other names
2890:Categories
2873:Goofy foot
2854:Footedness
2817:Levocardia
2625:Laterality
1608:Teratology
1573:Teratology
1293:Up To Date
1232:References
1120:bacteria.
1067:peritoneum
1053:Management
860:peritoneal
395:arrhythmia
292:levocardia
256:intestines
240:bile ducts
216:arrhythmia
155:clinically
115:morphology
60:heterotaxy
54:(from
44:Cardiology
2901:Syndromes
2813:In heart
2758:Musicians
2545:eMedicine
2540:radio/639
2504:1552-4833
2412:1542-0760
2377:207388492
2361:0019-5456
2285:244443675
2269:0899-7071
2069:0271-5333
1966:1460-2083
1909:0002-9297
1763:204918577
1731:: 61–69.
1523:206304327
1436:0033-8419
1416:Radiology
1341:1018-4813
1153:Prognosis
1148:Prognosis
1132:, to the
1130:ventricle
1098:infection
956:Diagnosis
895:Aberrant
477:cirrhosis
312:pulmonary
252:abdominal
165:isomerism
147:abdominal
131:morbidity
124:pulmonary
39:Specialty
2850:In feet
2712:In hands
2701:In eyes
2676:In brain
2651:General
2577:Orphanet
2522:25099286
2465:18603062
2420:26333177
2369:26612104
2277:34813990
2230:18598886
2174:UpToDate
2148:UpToDate
2122:UpToDate
2096:UpToDate
2077:10464794
2028:22916101
1984:19064609
1927:10053005
1862:20054532
1813:31094706
1755:31654871
1706:23490441
1698:19876930
1663:12797457
1628:12397632
1593:11283968
1558:11241474
1515:18039397
1401:21731561
1349:16251896
1203:See also
1173:Hispanic
1160:Research
946:cyanosis
916:bronchus
870:Vascular
856:volvulus
838:pancreas
824:asplenia
753:18q21.1
735:10q22.1
520:asplenia
327:cyanosis
319:shunting
264:Asplenia
248:Vomiting
244:jaundice
200:Cyanosis
173:diagnose
151:thoracic
143:symmetry
139:patients
120:systemic
2513:4462240
2326:9562017
2221:4385517
2020:9354794
1975:2722226
1918:1377788
1804:6597216
1746:9258637
1479:2252000
1444:4695908
1392:3116098
1177:Mexican
798:stomach
766:Cardiac
675:2q21.1
655:Xq26.2
544:cocaine
488:stomach
434:stomach
333:. Poor
304:cardiac
260:chronic
196:cardiac
111:anatomy
102:of the
76:abdomen
2807:LRRTM1
2646:Right
2582:157769
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1982:
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1255:23 May
1194:in an
1142:valves
1086:, and
887:azygos
832:sepsis
802:spleen
749:CCDC11
744:614779
724:270100
710:ACVR2B
704:613751
684:606325
664:605376
644:306955
635:Locus
591:, and
589:NKX2-5
558:. The
538:Causes
532:sepsis
442:spleen
440:, and
284:thorax
162:atrial
137:. All
92:spleen
68:organs
2643:Both
2640:Left
2637:Side
2373:S2CID
2281:S2CID
2024:S2CID
1866:S2CID
1759:S2CID
1702:S2CID
1519:S2CID
1134:lungs
1102:fever
938:lungs
809:liver
740:HTX6
730:NODAL
720:HTX5
700:HTX4
695:6q21
680:HTX3
660:HTX2
640:HTX1
632:Gene
624:Type
585:NODAL
508:mucus
469:acute
453:liver
430:liver
339:aorta
234:, or
224:liver
192:Acute
135:death
104:heart
100:atria
96:lungs
72:chest
56:Latin
2518:PMID
2500:ISSN
2461:PMID
2416:PMID
2408:ISSN
2365:PMID
2357:ISSN
2322:PMID
2273:PMID
2265:ISSN
2226:PMID
2181:2015
2155:2015
2129:2015
2103:2015
2073:PMID
2065:ISSN
2016:PMID
1980:PMID
1962:ISSN
1923:PMID
1905:ISSN
1858:PMID
1827:link
1809:PMID
1751:PMID
1694:PMID
1682:151C
1659:PMID
1624:PMID
1589:PMID
1554:PMID
1511:PMID
1475:PMID
1440:PMID
1432:ISSN
1397:PMID
1345:PMID
1337:ISSN
1300:2015
1257:2016
1116:and
993:and
948:and
826:and
800:and
796:The
784:and
715:992
690:PA26
670:CFC1
650:ZIC3
628:OMIM
605:ZIC3
599:and
593:ZIC3
479:and
461:bile
451:The
397:and
389:and
266:and
226:and
218:and
149:and
122:and
113:and
98:and
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2492:doi
2488:164
2451:hdl
2443:doi
2439:136
2400:doi
2396:103
2349:doi
2312:doi
2257:doi
2216:PMC
2208:doi
2055:doi
2008:doi
1970:PMC
1954:doi
1913:PMC
1897:doi
1850:doi
1799:PMC
1791:doi
1787:129
1741:PMC
1733:doi
1686:doi
1651:doi
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1581:doi
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