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.

Also Known As:

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 Syndromes; 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, Heterotaxy; Syndrome, Ivemark; Syndrome, Polysplenia; Syndromes, Asplenia; Syndromes, Heterotaxy; Syndromes, Polysplenia; Visceral Heterotaxies; Viscerum, Situs Ambiguus; Viscerums, Situs Ambiguus

Cardiovascular Diseases: 91362
- Heart Diseases: 26014
  - Congenital Heart Defects: 8917
    - Heterotaxy Syndrome: 205
- Cardiovascular Abnormalities: 452
  - Congenital Heart Defects: 8917
    - Heterotaxy Syndrome: 205
Hemic and Lymphatic Diseases: 6
- Lymphatic Diseases: 92
  - Splenic Diseases: 72
    - Heterotaxy Syndrome: 205
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Congenital Abnormalities: 25723
  - Cardiovascular Abnormalities: 452
    - Congenital Heart Defects: 8917
      - Heterotaxy Syndrome: 205
  - Multiple Abnormalities: 211
    - Heterotaxy Syndrome: 205

1.	activin receptor type II-BIBA 01/01/1999 - "Left-right axis malformations associated with mutations in ACVR2B, the gene for human activin receptor type IIB."

Left-Right Axis Malformations

Disease Context: Research Results

Drugs and Biologics