972:
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.
1151:. 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
292:
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
128:
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
1178:
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
1166:
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.
971:
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
1167:
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.
1503:
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".
1134:
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
2102:
589:
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
939:
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.
1299:
2008:
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".
1209:
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.
1080:
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.
1787:
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).
561:, 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
537:). 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
2128:
1092:
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,
2154:
521:
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.
384:
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
101:, 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
2180:
388:, 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
336:
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
1582:
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".
97:, 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
921:
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:
466:
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
1468:
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".
1088:
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,
1951:
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).
2489:
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).
1837:
1175:
There have been vast amounts of research on the clinical features, racial disparities, and physiological mechanisms of heterotaxy syndrome dating back to 1973.
293:
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
93:
and arrangement of the visceral organs; however, classical heterotaxy requires multiple organs to be affected. This does not include the congenital defect
2401:
Lopez, Keila N.; Marengo, Lisa K.; Canfield, Mark A.; Belmont, John W.; Dickerson, Heather A. (2015-09-01). "Racial disparities in heterotaxy syndrome".
1652:
Kuehl KS, Loffredo CA (March 2003). "Population-based study of l-transposition of the great arteries: possible associations with environmental factors".
1260:
360:
Left atrial appendage isomerism, also called left atrial isomerism, is a cardiac development defect in which the heart has two bilateral left atria and
2768:
274:
1190:
descent, as well as female infants of non-Hispanic black and white mothers. Additional studies must be done to clarify the mechanisms behind
2444:
Kim, Soo-Jin; Kim, Woong-Han; Lim, Hong-Gook; Lee, Jae-Young (July 2008). "Outcome of 200 patients after an extracardiac Fontan procedure".
2626:
1896:"Characterization and mutation analysis of human LEFTY A and LEFTY B, homologues of murine genes implicated in left-right axis development"
2791:
541:. Rarely, left atrial isomeric patients have a single, normal, functional spleen. Patients lacking a functional spleen are in danger of
2679:
787:
573:
has an important role in positioning these cilia, and thus genes within this pathway are increasingly associated with situs ambiguus.
2786:
2309:
Hashmi, Aijaz; Abu-Sulaiman, Riyadh; McCrindle, Brian W; Smallhorn, Jeffrey F; Williams, William G; Freedom, Robert M (1998-04-01).
1194:
in heterotaxy syndrome. Individuals of Asian descent show a higher prevalence of heterotaxy syndrome in general than members of the
1191:
2686:
1687:
Sutherland, Mardi J.; Ware, Stephanie M. (15 November 2009). "Disorders of left-right asymmetry: Heterotaxy and situs inversus".
1560:
1953:"Identification and functional characterization of NODAL rare variants in heterotaxy and isolated cardiovascular malformations"
611:
591:
558:
2491:"Laterality defects in the national birth defects prevention study (1998–2007): Birth prevalence and descriptive epidemiology"
2805:
566:
1425:
Freedom, Robert M.; Treves, S. (1973-05-01). "Splenic
Scintigraphy and Radionuclide Venography in the Heterotaxy Syndrome".
2906:
1201:
The
National Birth Defects Prevention study (October 2014) attempted to link clinical presentations of situs ambiguus to
2256:"Polysplenia syndrome and sickle cell trait: extensive deep venous thrombosis from venous stasis and hypercoagulability"
456:
448:
364:
in the muscle wall. Left atrial isomerism can have varied clinical manifestations, including a later onset of symptoms.
266:
120:
Individuals with situs inversus or situs solitus do not experience fatal dysfunction of their organ systems, as general
996:
562:
2703:
792:
459:
of the body. Distribution of these organs largely dictates treatment, clinical outcomes, and further evaluation.
2619:
1851:
Chen, CM; et al. (April 2010). "Transcriptional
Control of Left–Right Patterning in Cardiac Development".
1120:
1001:
944:
425:
179:
754:
404:
largely depends on physiological looping of the ventricles. Abnormal looping of the ventricles contributes to
1547:
MartĂnez-FrĂas ML (March 2001). "Heterotaxia as an outcome of maternal diabetes: an epidemiological study".
1220:
325:
289:
2592:
1617:
Kuehl KS, Loffredo C (November 2002). "Risk factors for heart disease associated with abnormal sidedness".
1128:
1124:
1085:
960:
862:
509:
of the entire intestinal tract, or improper folding and bulging of the stomach and intestines, results in
506:
321:
in the muscle wall, as opposed to the normal right atrium and left atrium. In right atrial isomerism, the
285:
238:
187:
165:
494:
become a major concern. Biliary atresia is not usually observed in patients with right atrial isomerism.
486:, pale stools, dark urine, and abdominal swelling. If this condition continues without proper treatment,
182:
cases. Additional estimation of incidence and prevalence of isomerism proves difficult due to failure to
1831:
1089:
397:
269:. Poor positioning of the intestine also makes it more prone to blockage, which can result in numerous
233:
may raise further suspicion of a cardiac abnormality. Non-cardiac symptoms include impairments of the
1068:
Each of the symptoms of situs ambiguus must be managed with appropriate treatment dependent upon the
796:
570:
514:
502:
130:
125:
90:
622:
family transcription factor, is linked to a 50% risk of atrial isomerism in families. It is also an
2612:
1300:"Anatomy, clinical manifestations and diagnosis of heterotaxy (isomerism of the atrial appendages)"
1104:
991:
986:
932:
782:
607:
538:
389:
341:
153:
2207:""Contemporary outcomes after the Fontan procedure: A Pediatric Heart Network multicenter study.""
1107:
patients have an elevated lifetime risk of sepsis, as they have no functional spleen for fighting
213:
or blue skin coloration, primarily affecting the lips and fingernails, can indicate a systemic or
2911:
2383:
2291:
2034:
1876:
1769:
1712:
1529:
1148:
887:
405:
369:
345:
333:
301:, in which the apex of the heart is pointed toward the right side of the thorax, and 50–70% have
226:
214:
2698:
2528:
2510:
2471:
2426:
2418:
2375:
2367:
2332:
2283:
2275:
2236:
2083:
2075:
2026:
1990:
1972:
1933:
1915:
1868:
1819:
1761:
1704:
1669:
1634:
1599:
1564:
1521:
1485:
1450:
1442:
1407:
1355:
1347:
1140:
1098:
1069:
874:
510:
373:
270:
222:
218:
74:
49:
190:
is much more easily recognized than left isomerism, contributing to the failure to diagnose.
2715:
2518:
2502:
2461:
2453:
2410:
2359:
2350:
Mishra, Smita (2015-11-26). "Cardiac and Non-Cardiac
Abnormalities in Heterotaxy Syndrome".
2322:
2267:
2226:
2218:
2065:
2018:
1980:
1964:
1923:
1907:
1860:
1809:
1801:
1751:
1743:
1696:
1661:
1626:
1591:
1556:
1513:
1477:
1434:
1397:
1389:
1337:
1225:
1136:
981:
854:
202:
157:
1894:
Kosaki, K; Bassi, M T; Kosaki, R; Lewin, M; Belmont, J; Schauer, G; Casey, B (1999-03-01).
2756:
2749:
2732:
2693:
2070:
2053:
1116:
1057:
1034:
952:
901:
385:
329:
242:
161:
78:
2052:
Applegate, Kimberly E.; Goske, Marilyn J.; Pierce, Gregory; Murphy, Daniel (1999-07-01).
1073:
557:
use, suggesting both genetic and environmental factors play a role. Several genes in the
1256:
2854:
2523:
2490:
2231:
2206:
1985:
1952:
1928:
1895:
1814:
1789:
1756:
1731:
1402:
1377:
1230:
1042:
393:
178:
occurs in 1 out of every 10,000 live births and is associated with approximately 3% of
110:
94:
66:
2327:
2310:
629:
The most prevalent and best characterized genetic associations of heterotaxy include:
2900:
2844:
2667:
2387:
2295:
1773:
1533:
1481:
1235:
1195:
1111:. For this reason, asplenic patients are under constant observation for any signs of
823:
599:
526:
491:
483:
467:
401:
365:
98:
2255:
2038:
1716:
2834:
2271:
1206:
1028:
880:
318:
298:
246:
230:
137:
17:
1880:
284:
Due to abnormal cardiac development, patients with situs ambiguus usually develop
1790:"Imbalanced mitochondrial function provokes heterotaxy via aberrant ciliogenesis"
193:
Situs ambiguus is a growing field of research with findings dating back to 1973.
2457:
1152:
1010:
1005:
907:
897:
838:
623:
619:
553:
Situs ambiguus has been linked to family history of malformations and maternal
534:
475:
409:
361:
305:, in which the apex of the heart is pointed toward the left side of the thorax.
278:
2550:
2222:
2883:
2876:
2864:
2827:
2737:
2727:
2722:
2635:
2363:
1864:
1747:
1517:
1393:
1202:
1094:
1077:
870:
479:
424:
is not typically damaging and presents no significant clinical complications.
302:
54:
2514:
2422:
2371:
2279:
2079:
1976:
1919:
1446:
1351:
576:
Disrupted mitochondria function has also been recently linked to heterotaxy.
525:
Isomeric patients often experience disruptions to splenic development during
2555:
1342:
1325:
1163:
1108:
487:
250:
175:
141:
134:
2532:
2475:
2430:
2379:
2287:
2240:
2103:"Management and outcome of heterotaxy (isomerism of the atrial appendages)"
2087:
1994:
1937:
1872:
1823:
1765:
1732:"Planar cell polarity signaling in the development of left–right asymmetry"
1708:
1673:
1638:
1603:
1568:
1525:
1411:
1359:
896:
Intrahepatic interruption of the inferior vena cava with connection to the
478:
is functional and morphologically normal. This biliary atresia can lead to
313:
Right atrial appendage isomerism, also called right atrial isomerism, is a
2570:
2336:
2030:
1489:
1454:
1115:
or infection. In the case of infection, patients are placed on controlled
2587:
2506:
2311:"Management and Outcomes of Right Atrial Isomerism: A 26-Year Experience"
1968:
1700:
1689:
American
Journal of Medical Genetics Part C: Seminars in Medical Genetics
1187:
1183:
956:
926:
866:
848:
834:
530:
421:
337:
258:
254:
210:
183:
2466:
2414:
2022:
1665:
1630:
1561:
10.1002/1096-8628(2000)9999:999<00::AID-AJMG1139>3.0.CO;2-Z
808:
554:
498:
444:
436:
262:
149:
121:
86:
2254:
Shahrouki, Puja; Lee, Edward
Wolfgang; Ruehm, Stefan (February 2022).
1595:
1438:
929:(below the pulmonary artery): supplies blood to the bi-lobed left lung
2817:
1805:
842:
812:
759:
720:
626:, so testing for ZIC3 mutations is highly encouraged in male births.
542:
452:
392:, or irregular rapid heart beat, and abnormal heart rhythm, known as
294:
172:
102:
2181:"Clinical features and management of sepsis in the asplenic patient"
1182:
Recent studies have shown higher rates of heterotaxy syndrome among
935:(adjacent to the artery): supplies blood to the tri-lobed right lung
317:
development defect in which the heart has bilateral right atria and
89:. Clinically, heterotaxy spectrum generally refers to any defect of
1911:
857:
develop, which can lead to cancer, kidney stones, and/or infection.
1112:
819:
776:
740:
700:
595:
518:
463:
440:
349:
314:
234:
206:
201:
There are a variety of clinical manifestations of situs ambiguus.
145:
114:
106:
82:
1654:
Birth
Defects Research. Part A, Clinical and Molecular Teratology
1072:
involved. Intestinal malrotation is treated surgically using the
2403:
Birth Defects Research Part A: Clinical and Molecular Teratology
1324:
Zhu, Lirong; Belmont, John W.; Ware, Stephanie M. (2005-10-26).
1144:
948:
734:
714:
694:
680:
674:
660:
654:
638:
615:
603:
471:
322:
225:
may also be observed due to poor circulation. Upon examination,
2608:
1179:
evaluation in cases at risk for development of situs ambiguus.
186:
and underestimation of the disease by clinicians. Furthermore,
2604:
265:
region are features that suggest improper positioning of the
565:, likely due to the important role of cilia in establishing
501:
is often one of the first signals of situs ambiguus upon
372:
is disrupted due to the inappropriate morphology of the
2205:
Anderson PA, Sleeper LA, Mahony L, et al. (2008).
2101:
Lowenthal, Alexander; et al. (October 27, 2015).
2179:
Pasternack, Mark S; et al. (December 11, 2014).
2054:"Situs Revisited: Imaging of the Heterotaxy Syndrome"
27:
Abnormal placement of organs in the chest and abdomen
2560:
2578:
2564:
281:are also possible features of heterotaxy syndrome.
48:
40:
35:
2446:The Journal of Thoracic and Cardiovascular Surgery
168:considered to have a form of heterotaxy syndrome.
1298:Lowenthal, A.; et al. (September 26, 2015).
2153:Erlichman, Jessi; et al. (August 3, 2015).
348:also results due to improper positioning of the
2127:Brandt, Mary; et al. (October 24, 2015).
428:results in issues of blood flow to the lungs.
2620:
2315:Journal of the American College of Cardiology
2211:Journal of the American College of Cardiology
8:
1836:: CS1 maint: multiple names: authors list (
1017:Diagnostic techniques for non-cardiac causes
947:, there is a reduction in blood flow to the
152:with situs ambiguus lack lateralization and
2495:American Journal of Medical Genetics Part A
2627:
2613:
2605:
2561:
1261:United States National Library of Medicine
1139:routes blood through the patient's single
951:due to an obstruction of the heart at the
893:Bilateral superior or inferior venae cavae
32:
2522:
2465:
2326:
2230:
2069:
1984:
1927:
1813:
1755:
1401:
1341:
1123:. This therapy battles infection by both
529:, resulting in an overall lack a spleen (
2644:
976:Diagnostic techniques for cardiac causes
631:
455:may be randomly arranged throughout the
44:Situs ambiguous, heterotaxy, heterotaxia
1730:Axelrod, Jeffrey D. (1 February 2020).
1248:
1829:
1076:. This procedure widens a fold in the
826:are occasionally found in the midline.
81:are distributed abnormally within the
2071:10.1148/radiographics.19.4.g99jl31837
513:. This impairment leads to vomiting,
7:
1549:American Journal of Medical Genetics
1371:
1369:
1293:
1291:
1289:
1287:
1285:
1283:
1281:
1279:
1277:
1039:Evaluation of intestinal malrotation
804:Deranged abdominal organ asymmetry:
328:tract is damaged due to right-left
1900:American Journal of Human Genetics
1330:European Journal of Human Genetics
1033:Evaluation of biliary anatomy and
788:transposition of the great vessels
533:) or development of many spleens (
25:
2352:The Indian Journal of Pediatrics
1326:"Genetics of human heterotaxias"
585:Molecular and cellular mechanism
309:Right atrial appendage isomerism
1736:Current Opinion in Cell Biology
612:hypoplastic left heart syndrome
368:is often a concern because the
356:Left atrial appendage isomerism
2806:Edinburgh Handedness Inventory
2680:Geschwind–Galaburda hypothesis
2272:10.1016/j.clinimag.2021.11.017
873:attachments, which completely
815:are prone to isolated reversal
129:experience complications with
1:
2328:10.1016/S0735-1097(98)00062-X
1099:anti-inflammatory medications
847:More rarely, the head of the
1482:10.1016/0002-9149(90)90543-a
1054:Assessment of family history
380:Conductive nodes and tissues
205:symptoms can be due to both
77:in which the major visceral
69: 'ambiguous site'), or
2458:10.1016/j.jtcvs.2007.12.032
1084:Following cholangiogram, a
470:, which is responsible for
332:of blood. In addition, the
2928:
2223:10.1016/j.jacc.2008.01.074
1382:Korean Circulation Journal
1117:empiric antibiotic therapy
1025:Magnetic resonance imaging
997:Magnetic resonance imaging
563:primary ciliary dyskinesia
497:Random positioning of the
482:problems such as nutrient
474:production, even when the
376:to support the vena cava.
2872:Footedness in boardsports
2863:
2816:
2804:
2783:
2714:
2690:
2678:
2642:
2364:10.1007/s12098-015-1925-x
1865:10.1007/s00246-009-9610-3
1748:10.1016/j.ceb.2019.09.002
1518:10.1017/s104795110700114x
1394:10.4070/kcj.2011.41.5.227
1049:Other diagnostic features
1029:Splenic function analysis
209:and non-cardiac defects.
171:Heterotaxy syndrome with
2129:"Intestinal Malrotation"
1957:Human Molecular Genetics
1119:to avoid development of
945:pulmonary valve stenosis
917:Bronchial tree and lungs
426:Pulmonary valve stenosis
416:Bronchial tree and lungs
180:congenital heart disease
2676:In cognitive abilities
1506:Cardiology in the Young
1343:10.1038/sj.ejhg.5201506
1221:Chirality (mathematics)
1002:Cardiac catheterization
779:looping malformations:
545:and must be monitored.
249:and destruction of the
2801:Handedness measurement
961:pulmonary hypertension
955:. This contributes to
618:gene, which encodes a
606:, have been linked to
286:right atrial isomerism
239:gastrointestinal tract
2780:Handedness related to
1378:"Heterotaxy Syndrome"
1376:Kim, Soo-Jin (2011).
1257:"heterotaxy syndrome"
1121:antibiotic resistance
1105:Functionally asplenic
1090:liver transplantation
831:Organ malformations:
797:atrial septal defects
398:atrioventricular node
396:. Development of the
217:issue. Poor feeding,
2907:Congenital disorders
2792:Mathematical ability
2764:Handedness in people
2745:Handedness in boxing
2507:10.1002/ajmg.a.36695
1853:Pediatric Cardiology
1701:10.1002/ajmg.c.30228
1149:systemic circulation
571:planar cell polarity
567:left-right asymmetry
515:abdominal distension
346:systemic circulation
342:respiratory distress
261:and swelling of the
91:left-right asymmetry
2813:Handedness genetics
1022:Computed tomography
992:Computed tomography
987:Electrocardiography
933:Eparterial bronchus
771:Classical pathology
614:. Mutations in the
608:tetralogy of Fallot
539:functional asplenia
390:atrial fibrillation
18:Heterotaxy syndrome
2787:Sexual orientation
2579:External resources
2415:10.1002/bdra.23416
2023:10.1038/ng1197-305
1969:10.1093/hmg/ddn411
1666:10.1002/bdra.10015
1631:10.1002/tera.10099
1192:racial disparities
888:inferior vena cava
875:obstruct the bowel
783:Fallot's tetralogy
370:inferior vena cava
319:atrial attachments
288:consisting of two
197:Signs and symptoms
2894:
2893:
2889:
2888:
2841:In major viscera
2699:Dual brain theory
2602:
2601:
2501:(10): 2581–2591.
2358:(12): 1135–1146.
2155:"Biliary Atresia"
1596:10.1002/tera.1022
1439:10.1148/107.2.381
855:Horseshoe kidneys
768:
767:
624:X-linked disorder
511:bowel obstruction
420:Isomerism of the
362:atrial appendages
223:shallow breathing
219:failure to thrive
164:cavities and are
156:of organs in the
75:congenital defect
60:
59:
30:Medical condition
16:(Redirected from
2919:
2738:Right-handedness
2716:Ocular dominance
2645:
2629:
2622:
2615:
2606:
2562:
2537:
2536:
2526:
2486:
2480:
2479:
2469:
2441:
2435:
2434:
2398:
2392:
2391:
2347:
2341:
2340:
2330:
2321:(5): 1120–1126.
2306:
2300:
2299:
2260:Clinical Imaging
2251:
2245:
2244:
2234:
2202:
2196:
2195:
2193:
2191:
2176:
2170:
2169:
2167:
2165:
2150:
2144:
2143:
2141:
2139:
2124:
2118:
2117:
2115:
2113:
2098:
2092:
2091:
2073:
2049:
2043:
2042:
2005:
1999:
1998:
1988:
1948:
1942:
1941:
1931:
1891:
1885:
1884:
1848:
1842:
1841:
1835:
1827:
1817:
1806:10.1172/JCI98890
1800:(7): 2841–2855.
1784:
1778:
1777:
1759:
1727:
1721:
1720:
1684:
1678:
1677:
1649:
1643:
1642:
1614:
1608:
1607:
1579:
1573:
1572:
1544:
1538:
1537:
1500:
1494:
1493:
1465:
1459:
1458:
1422:
1416:
1415:
1405:
1373:
1364:
1363:
1345:
1321:
1315:
1314:
1312:
1310:
1295:
1272:
1271:
1269:
1267:
1253:
1226:Ivemark syndrome
1137:Fontan procedure
982:Echocardiography
902:hemiazygos veins
632:
592:TGF-beta pathway
559:TGF-beta pathway
449:intestinal tract
439:, including the
437:Abdominal organs
432:Abdominal organs
386:sinoatrial nodes
144:, and sometimes
33:
21:
2927:
2926:
2922:
2921:
2920:
2918:
2917:
2916:
2897:
2896:
2895:
2890:
2757:Orthodox stance
2750:Southpaw stance
2733:Cross-dominance
2728:Left-handedness
2694:Brain asymmetry
2638:
2633:
2603:
2598:
2597:
2574:
2573:
2546:
2541:
2540:
2488:
2487:
2483:
2443:
2442:
2438:
2409:(11): 941–950.
2400:
2399:
2395:
2349:
2348:
2344:
2308:
2307:
2303:
2253:
2252:
2248:
2204:
2203:
2199:
2189:
2187:
2178:
2177:
2173:
2163:
2161:
2152:
2151:
2147:
2137:
2135:
2126:
2125:
2121:
2111:
2109:
2100:
2099:
2095:
2051:
2050:
2046:
2011:Nature Genetics
2007:
2006:
2002:
1950:
1949:
1945:
1893:
1892:
1888:
1850:
1849:
1845:
1828:
1786:
1785:
1781:
1729:
1728:
1724:
1686:
1685:
1681:
1651:
1650:
1646:
1616:
1615:
1611:
1581:
1580:
1576:
1546:
1545:
1541:
1502:
1501:
1497:
1467:
1466:
1462:
1424:
1423:
1419:
1375:
1374:
1367:
1323:
1322:
1318:
1308:
1306:
1297:
1296:
1275:
1265:
1263:
1255:
1254:
1250:
1245:
1240:
1216:
1207:epidemiological
1173:
1161:
1086:Kasai procedure
1066:
1058:Genetic testing
1051:
1019:
978:
969:
919:
883:abnormalities:
822:, and a single
773:
587:
582:
580:Pathophysiology
551:
457:left-right axis
434:
418:
382:
358:
311:
273:health issues.
243:Biliary atresia
199:
188:right isomerism
31:
28:
23:
22:
15:
12:
11:
5:
2925:
2923:
2915:
2914:
2909:
2899:
2898:
2892:
2891:
2887:
2886:
2881:
2879:
2874:
2868:
2867:
2862:
2858:
2857:
2855:Situs inversus
2852:
2850:Situs ambiguus
2847:
2842:
2838:
2837:
2832:
2830:
2825:
2821:
2820:
2815:
2809:
2808:
2803:
2797:
2796:
2795:
2794:
2789:
2782:
2776:
2775:
2773:
2771:
2766:
2760:
2759:
2754:
2752:
2747:
2741:
2740:
2735:
2730:
2725:
2719:
2718:
2713:
2709:
2708:
2707:
2706:
2701:
2696:
2689:
2683:
2682:
2677:
2673:
2672:
2670:
2665:
2663:
2659:
2658:
2655:
2652:
2649:
2643:
2640:
2639:
2634:
2632:
2631:
2624:
2617:
2609:
2600:
2599:
2596:
2595:
2583:
2582:
2580:
2576:
2575:
2569:
2568:
2566:
2565:Classification
2559:
2558:
2545:
2544:External links
2542:
2539:
2538:
2481:
2452:(1): 108–116.
2436:
2393:
2342:
2301:
2246:
2197:
2171:
2145:
2119:
2093:
2064:(4): 837–852.
2044:
2017:(3): 305–308.
2000:
1963:(5): 861–871.
1943:
1912:10.1086/302289
1906:(3): 712–721.
1886:
1859:(3): 371–377.
1843:
1779:
1722:
1695:(4): 307–317.
1679:
1644:
1609:
1574:
1539:
1495:
1476:(20): 1504–6.
1460:
1433:(2): 381–386.
1417:
1365:
1316:
1273:
1247:
1246:
1244:
1241:
1239:
1238:
1233:
1231:Situs inversus
1228:
1223:
1217:
1215:
1212:
1172:
1169:
1160:
1157:
1074:Ladd procedure
1065:
1062:
1061:
1060:
1055:
1050:
1047:
1046:
1045:
1043:Pulse oximeter
1040:
1037:
1031:
1026:
1023:
1018:
1015:
1014:
1013:
1008:
999:
994:
989:
984:
977:
974:
968:
965:
953:pulmonic valve
937:
936:
930:
918:
915:
914:
913:
912:
911:
904:
894:
891:
878:
869:and/or faulty
860:
859:
858:
852:
845:
841:often lead to
829:
828:
827:
816:
802:
801:
800:
790:
785:
772:
769:
766:
765:
762:
757:
752:
748:
747:
744:
737:
732:
728:
727:
724:
717:
712:
708:
707:
704:
697:
692:
688:
687:
684:
677:
672:
668:
667:
664:
657:
652:
648:
647:
644:
641:
636:
586:
583:
581:
578:
550:
547:
433:
430:
422:bronchial tree
417:
414:
394:atrial flutter
381:
378:
374:left ventricle
357:
354:
310:
307:
297:, 25–50% have
253:, may lead to
198:
195:
140:, significant
95:situs inversus
63:Situs ambiguus
58:
57:
52:
46:
45:
42:
38:
37:
36:Situs ambiguus
29:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
2924:
2913:
2910:
2908:
2905:
2904:
2902:
2885:
2882:
2880:
2878:
2875:
2873:
2870:
2869:
2866:
2860:
2859:
2856:
2853:
2851:
2848:
2846:
2845:Situs solitus
2843:
2840:
2839:
2836:
2833:
2831:
2829:
2826:
2823:
2822:
2819:
2814:
2811:
2810:
2807:
2802:
2799:
2798:
2793:
2790:
2788:
2785:
2784:
2781:
2778:
2777:
2774:
2772:
2770:
2767:
2765:
2762:
2761:
2758:
2755:
2753:
2751:
2748:
2746:
2743:
2742:
2739:
2736:
2734:
2731:
2729:
2726:
2724:
2721:
2720:
2717:
2711:
2710:
2705:
2702:
2700:
2697:
2695:
2692:
2691:
2688:
2685:
2684:
2681:
2675:
2674:
2671:
2669:
2668:Ambidexterity
2666:
2664:
2661:
2660:
2656:
2653:
2650:
2647:
2646:
2641:
2637:
2630:
2625:
2623:
2618:
2616:
2611:
2610:
2607:
2594:
2590:
2589:
2585:
2584:
2581:
2577:
2572:
2567:
2563:
2557:
2553:
2552:
2548:
2547:
2543:
2534:
2530:
2525:
2520:
2516:
2512:
2508:
2504:
2500:
2496:
2492:
2485:
2482:
2477:
2473:
2468:
2463:
2459:
2455:
2451:
2447:
2440:
2437:
2432:
2428:
2424:
2420:
2416:
2412:
2408:
2404:
2397:
2394:
2389:
2385:
2381:
2377:
2373:
2369:
2365:
2361:
2357:
2353:
2346:
2343:
2338:
2334:
2329:
2324:
2320:
2316:
2312:
2305:
2302:
2297:
2293:
2289:
2285:
2281:
2277:
2273:
2269:
2265:
2261:
2257:
2250:
2247:
2242:
2238:
2233:
2228:
2224:
2220:
2216:
2212:
2208:
2201:
2198:
2186:
2182:
2175:
2172:
2160:
2156:
2149:
2146:
2134:
2130:
2123:
2120:
2108:
2104:
2097:
2094:
2089:
2085:
2081:
2077:
2072:
2067:
2063:
2059:
2058:RadioGraphics
2055:
2048:
2045:
2040:
2036:
2032:
2028:
2024:
2020:
2016:
2012:
2004:
2001:
1996:
1992:
1987:
1982:
1978:
1974:
1970:
1966:
1962:
1958:
1954:
1947:
1944:
1939:
1935:
1930:
1925:
1921:
1917:
1913:
1909:
1905:
1901:
1897:
1890:
1887:
1882:
1878:
1874:
1870:
1866:
1862:
1858:
1854:
1847:
1844:
1839:
1833:
1825:
1821:
1816:
1811:
1807:
1803:
1799:
1795:
1794:J Clin Invest
1791:
1783:
1780:
1775:
1771:
1767:
1763:
1758:
1753:
1749:
1745:
1741:
1737:
1733:
1726:
1723:
1718:
1714:
1710:
1706:
1702:
1698:
1694:
1690:
1683:
1680:
1675:
1671:
1667:
1663:
1659:
1655:
1648:
1645:
1640:
1636:
1632:
1628:
1624:
1620:
1613:
1610:
1605:
1601:
1597:
1593:
1590:(3): 119–26.
1589:
1585:
1578:
1575:
1570:
1566:
1562:
1558:
1554:
1550:
1543:
1540:
1535:
1531:
1527:
1523:
1519:
1515:
1512:(S2): 29–43.
1511:
1507:
1499:
1496:
1491:
1487:
1483:
1479:
1475:
1471:
1464:
1461:
1456:
1452:
1448:
1444:
1440:
1436:
1432:
1428:
1421:
1418:
1413:
1409:
1404:
1399:
1395:
1391:
1388:(5): 227–32.
1387:
1383:
1379:
1372:
1370:
1366:
1361:
1357:
1353:
1349:
1344:
1339:
1335:
1331:
1327:
1320:
1317:
1305:
1301:
1294:
1292:
1290:
1288:
1286:
1284:
1282:
1280:
1278:
1274:
1262:
1258:
1252:
1249:
1242:
1237:
1236:Situs solitus
1234:
1232:
1229:
1227:
1224:
1222:
1219:
1218:
1213:
1211:
1208:
1204:
1199:
1197:
1196:Western world
1193:
1189:
1185:
1180:
1176:
1170:
1168:
1165:
1158:
1156:
1154:
1150:
1146:
1142:
1138:
1132:
1130:
1129:gram-negative
1126:
1125:gram-positive
1122:
1118:
1114:
1110:
1106:
1102:
1100:
1096:
1091:
1087:
1082:
1079:
1075:
1071:
1063:
1059:
1056:
1053:
1052:
1048:
1044:
1041:
1038:
1036:
1035:cholangiogram
1032:
1030:
1027:
1024:
1021:
1020:
1016:
1012:
1009:
1007:
1003:
1000:
998:
995:
993:
990:
988:
985:
983:
980:
979:
975:
973:
966:
964:
962:
958:
954:
950:
946:
941:
934:
931:
928:
924:
923:
922:
916:
909:
905:
903:
899:
895:
892:
889:
885:
884:
882:
879:
876:
872:
868:
865:errors cause
864:
861:
856:
853:
851:fails to form
850:
846:
844:
840:
836:
833:
832:
830:
825:
824:adrenal gland
821:
818:The stomach,
817:
814:
810:
806:
805:
803:
798:
794:
791:
789:
786:
784:
781:
780:
778:
775:
774:
770:
763:
761:
758:
756:
753:
750:
749:
745:
743:
742:
738:
736:
733:
730:
729:
725:
723:
722:
718:
716:
713:
710:
709:
705:
703:
702:
698:
696:
693:
690:
689:
685:
683:
682:
678:
676:
673:
670:
669:
665:
663:
662:
658:
656:
653:
650:
649:
645:
642:
640:
637:
634:
633:
630:
627:
625:
621:
617:
613:
609:
605:
601:
597:
593:
584:
579:
577:
574:
572:
568:
564:
560:
556:
548:
546:
544:
540:
536:
532:
528:
527:embryogenesis
523:
520:
516:
512:
508:
504:
500:
495:
493:
492:liver failure
489:
485:
484:malabsorption
481:
477:
473:
469:
465:
460:
458:
454:
450:
446:
442:
438:
431:
429:
427:
423:
415:
413:
411:
407:
403:
402:bundle of His
399:
395:
391:
387:
379:
377:
375:
371:
367:
366:Heart failure
363:
355:
353:
351:
347:
343:
340:and possible
339:
335:
334:atrial septum
331:
327:
324:
320:
316:
308:
306:
304:
300:
296:
291:
287:
282:
280:
276:
272:
268:
264:
260:
256:
252:
248:
244:
240:
236:
232:
228:
224:
220:
216:
212:
208:
204:
196:
194:
191:
189:
185:
181:
177:
174:
169:
167:
163:
159:
155:
151:
147:
143:
139:
138:blood vessels
136:
132:
127:
123:
118:
116:
112:
108:
104:
100:
99:situs solitus
96:
92:
88:
84:
80:
76:
72:
68:
64:
56:
53:
51:
47:
43:
39:
34:
19:
2877:Regular foot
2871:
2849:
2835:Dextrocardia
2812:
2800:
2779:
2763:
2744:
2704:Bicameralism
2586:
2549:
2498:
2494:
2484:
2449:
2445:
2439:
2406:
2402:
2396:
2355:
2351:
2345:
2318:
2314:
2304:
2263:
2259:
2249:
2217:(2): 85–93.
2214:
2210:
2200:
2190:December 10,
2188:. Retrieved
2184:
2174:
2162:. Retrieved
2158:
2148:
2138:December 10,
2136:. Retrieved
2132:
2122:
2110:. Retrieved
2106:
2096:
2061:
2057:
2047:
2014:
2010:
2003:
1960:
1956:
1946:
1903:
1899:
1889:
1856:
1852:
1846:
1832:cite journal
1797:
1793:
1782:
1739:
1735:
1725:
1692:
1688:
1682:
1660:(3): 162–7.
1657:
1653:
1647:
1625:(5): 242–8.
1622:
1618:
1612:
1587:
1583:
1577:
1555:(2): 142–6.
1552:
1548:
1542:
1509:
1505:
1498:
1473:
1470:Am J Cardiol
1469:
1463:
1430:
1426:
1420:
1385:
1381:
1336:(1): 17–25.
1333:
1329:
1319:
1307:. Retrieved
1303:
1264:. Retrieved
1251:
1203:demographics
1200:
1181:
1177:
1174:
1162:
1133:
1103:
1083:
1070:organ system
1067:
970:
942:
938:
925:Hyparterial
920:
908:portal veins
886:Interrupted
739:
719:
699:
679:
659:
628:
594:, including
588:
575:
552:
524:
496:
476:gall bladder
468:biliary tree
461:
435:
419:
412:in fetuses.
383:
359:
326:blood oxygen
312:
299:dextrocardia
283:
247:inflammation
231:heart murmur
221:, and rapid
200:
192:
170:
119:
73:, is a rare
70:
62:
61:
2467:10371/60103
2266:: 127–131.
2164:December 9,
2112:December 8,
1309:November 4,
1186:infants of
1147:, and into
1095:choleretics
1011:Chest x-ray
1006:angiography
863:Malrotation
839:polysplenia
793:Ventricular
620:zinc finger
535:polysplenia
507:Malrotation
503:examination
410:heart block
290:bilaterally
279:polysplenia
215:circulatory
41:Other names
2901:Categories
2884:Goofy foot
2865:Footedness
2828:Levocardia
2636:Laterality
1619:Teratology
1584:Teratology
1304:Up To Date
1243:References
1131:bacteria.
1078:peritoneum
1064:Management
871:peritoneal
406:arrhythmia
303:levocardia
267:intestines
251:bile ducts
227:arrhythmia
166:clinically
126:morphology
71:heterotaxy
65:(from
55:Cardiology
2912:Syndromes
2824:In heart
2769:Musicians
2556:eMedicine
2551:radio/639
2515:1552-4833
2423:1542-0760
2388:207388492
2372:0019-5456
2296:244443675
2280:0899-7071
2080:0271-5333
1977:1460-2083
1920:0002-9297
1774:204918577
1742:: 61–69.
1534:206304327
1447:0033-8419
1427:Radiology
1352:1018-4813
1164:Prognosis
1159:Prognosis
1143:, to the
1141:ventricle
1109:infection
967:Diagnosis
906:Aberrant
488:cirrhosis
323:pulmonary
263:abdominal
176:isomerism
158:abdominal
142:morbidity
135:pulmonary
50:Specialty
2861:In feet
2723:In hands
2712:In eyes
2687:In brain
2662:General
2588:Orphanet
2533:25099286
2476:18603062
2431:26333177
2380:26612104
2288:34813990
2241:18598886
2185:UpToDate
2159:UpToDate
2133:UpToDate
2107:UpToDate
2088:10464794
2039:22916101
1995:19064609
1938:10053005
1873:20054532
1824:31094706
1766:31654871
1717:23490441
1709:19876930
1674:12797457
1639:12397632
1604:11283968
1569:11241474
1526:18039397
1412:21731561
1360:16251896
1214:See also
1184:Hispanic
1171:Research
957:cyanosis
927:bronchus
881:Vascular
867:volvulus
849:pancreas
835:asplenia
764:18q21.1
746:10q22.1
531:asplenia
338:cyanosis
330:shunting
275:Asplenia
259:Vomiting
255:jaundice
211:Cyanosis
184:diagnose
162:thoracic
154:symmetry
150:patients
131:systemic
2524:4462240
2337:9562017
2232:4385517
2031:9354794
1986:2722226
1929:1377788
1815:6597216
1757:9258637
1490:2252000
1455:4695908
1403:3116098
1188:Mexican
809:stomach
777:Cardiac
686:2q21.1
666:Xq26.2
555:cocaine
499:stomach
445:stomach
344:. Poor
315:cardiac
271:chronic
207:cardiac
122:anatomy
113:of the
87:abdomen
2818:LRRTM1
2657:Right
2593:157769
2531:
2521:
2513:
2474:
2429:
2421:
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2278:
2239:
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2078:
2037:
2029:
1993:
1983:
1975:
1936:
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1918:
1881:300003
1879:
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1672:
1637:
1602:
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1445:
1410:
1400:
1358:
1350:
1266:23 May
1205:in an
1153:valves
1097:, and
898:azygos
843:sepsis
813:spleen
760:CCDC11
755:614779
735:270100
721:ACVR2B
715:613751
695:606325
675:605376
655:306955
646:Locus
602:, and
600:NKX2-5
569:. The
549:Causes
543:sepsis
453:spleen
451:, and
295:thorax
173:atrial
148:. All
103:spleen
79:organs
2654:Both
2651:Left
2648:Side
2384:S2CID
2292:S2CID
2035:S2CID
1877:S2CID
1770:S2CID
1713:S2CID
1530:S2CID
1145:lungs
1113:fever
949:lungs
820:liver
751:HTX6
741:NODAL
731:HTX5
711:HTX4
706:6q21
691:HTX3
671:HTX2
651:HTX1
643:Gene
635:Type
596:NODAL
519:mucus
480:acute
464:liver
441:liver
350:aorta
245:, or
235:liver
203:Acute
146:death
115:heart
111:atria
107:lungs
83:chest
67:Latin
2529:PMID
2511:ISSN
2472:PMID
2427:PMID
2419:ISSN
2376:PMID
2368:ISSN
2333:PMID
2284:PMID
2276:ISSN
2237:PMID
2192:2015
2166:2015
2140:2015
2114:2015
2084:PMID
2076:ISSN
2027:PMID
1991:PMID
1973:ISSN
1934:PMID
1916:ISSN
1869:PMID
1838:link
1820:PMID
1762:PMID
1705:PMID
1693:151C
1670:PMID
1635:PMID
1600:PMID
1565:PMID
1522:PMID
1486:PMID
1451:PMID
1443:ISSN
1408:PMID
1356:PMID
1348:ISSN
1311:2015
1268:2016
1127:and
1004:and
959:and
837:and
811:and
807:The
795:and
726:992
701:PA26
681:CFC1
661:ZIC3
639:OMIM
616:ZIC3
610:and
604:ZIC3
490:and
472:bile
462:The
408:and
400:and
277:and
237:and
229:and
160:and
133:and
124:and
109:and
85:and
2554:at
2519:PMC
2503:doi
2499:164
2462:hdl
2454:doi
2450:136
2411:doi
2407:103
2360:doi
2323:doi
2268:doi
2227:PMC
2219:doi
2066:doi
2019:doi
1981:PMC
1965:doi
1924:PMC
1908:doi
1861:doi
1810:PMC
1802:doi
1798:129
1752:PMC
1744:doi
1697:doi
1662:doi
1627:doi
1592:doi
1557:doi
1514:doi
1478:doi
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