Diseases

Heterotaxy Syndrome

Abnormal thoracoabdominal VISCERA arrangement (visceral heterotaxy) or malformation that involves additional CONGENITAL HEART DEFECTS (e.g., heart isomerism; DEXTROCARDIA) and/or abnormal SPLEEN (e.g., asplenia and polysplenia). Irregularities with the central nervous system, the skeleton and urinary tract are often associated with the syndrome.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

Abnormal thoracoabdominal VISCERA arrangement (visceral heterotaxy) or malformation that involves additional CONGENITAL HEART DEFECTS (e.g., heart isomerism; DEXTROCARDIA) and/or abnormal SPLEEN (e.g., asplenia and polysplenia). Irregularities with the central nervous system, the skeleton and urinary tract are often associated with the syndrome.

MeSH Record

Classification

Broader headings

Related Concepts

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MeSH Record

Synonyms

42 entry terms
  • Heterotaxy Syndromes
  • Syndrome, Heterotaxy
  • Syndromes, Heterotaxy
  • Asplenia Syndrome
  • Asplenia with Cardiovascular Anomalies
  • Ivemark Syndrome
  • Left Atrial Isomerism
  • Left Atrial Isomerism with Polysplenia
  • Polysplenia Syndrome
  • Right Atrial Isomerism
  • Right Atrial Isomerism with Asplenia
  • Situs Ambiguus
  • Situs Ambiguus Viscerum
  • Situs Ambiguus with Asplenia
  • Situs Ambiguus with Polysplenia
  • Visceral Heterotaxy
  • Ambiguus Viscerum, Situs
  • Ambiguus Viscerums, Situs
  • Ambiguus, Situs
  • Asplenia Syndromes
  • Atrial Isomerism, Left
  • Atrial Isomerism, Right
  • Atrial Isomerisms, Left
  • Atrial Isomerisms, Right
  • Heterotaxies, Visceral
  • Heterotaxy, Visceral
  • Isomerism, Left Atrial
  • Isomerism, Right Atrial
  • Isomerisms, Left Atrial
  • Isomerisms, Right Atrial
  • Left Atrial Isomerisms
  • Polysplenia Syndromes
  • Right Atrial Isomerisms
  • Situs Ambiguus Viscerums
  • Syndrome, Asplenia
  • Syndrome, Ivemark
  • Syndrome, Polysplenia
  • Syndromes, Asplenia
  • Syndromes, Polysplenia
  • Visceral Heterotaxies
  • Viscerum, Situs Ambiguus
  • Viscerums, Situs Ambiguus

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2012

MeSH Record

Previous Indexing

  • Heart Defects, Congenital (1976-2011)
  • Spleen/abnormalities (1966-2011)
  • Viscera/abnormalities (1994-2011)

MeSH Hierarchy

Tree Numbers

  1. C14w.240.400w.592
  2. C14w.280w.400w.592
  3. C15.604w.744w.146
  4. C16w.131w.077w.401
  5. C16w.131w.240.400w.592

AMA Style

References

  1. National Library of Medicine. Heterotaxy Syndrome. Medical Subject Headings (MeSH). 2026. Unique ID D059446. http://id.nlm.nih.gov/mesh/2026/D059446
  2. Heterotaxy Syndrome. In: Wikidata. https://www.wikidata.org/wiki/Q203115