Advanced
Technical
IVT
Heterotaxy Syndrome
A rare congenital condition involving abnormal arrangement of thoracic and abdominal organs, often associated with complex congenital heart defects.
Full Definition
Heterotaxy syndrome, also known as isomerism, is a complex congenital malformation syndrome characterized by abnormal left-right axis development during embryogenesis. This results in improper positioning and orientation of thoracic and abdominal organs. The condition is frequently associated with severe congenital heart defects, splenic anomalies, and intestinal malrotation. Editors should note that this syndrome requires multidisciplinary care and is often described in case reports involving multiple organ systems.
Usage
Usage note: Always specify the type of isomerism (right or left) when known.
In Context
- "The patient presented with heterotaxy syndrome complicated by atrioventricular septal defect and bilateral superior vena cavae." — Clinical case report
- "Prenatal diagnosis of heterotaxy syndrome allows for coordinated delivery planning with pediatric cardiothoracic surgery." — Maternal-fetal medicine consultation
Also known as
Isomerism
Situs Ambiguus
Contrasted with
Situs Solitus
Don't confuse with
Dextrocardia
Situs Inversus