Heterotaxy
Pronunciation: HET-er-oh-tak-see
A complex condition involving abnormal arrangement of thoracic and abdominal organs, often associated with congenital heart disease.
Full Definition
Heterotaxy refers to a spectrum of congenital anomalies characterized by abnormal lateralization and positioning of normally asymmetric organs within the thoracic and abdominal cavities. This condition includes both right and left atrial isomerism, each associated with specific patterns of cardiac and extracardiac malformations. Common cardiac defects include atrioventricular canal defects, pulmonary stenosis or atresia, and abnormalities of systemic and pulmonary venous connections. Splenic abnormalities, intestinal malrotation, and ciliary dysfunction may also be present.
Usage
Usage note: Single word, not hyphenated; encompasses both right and left isomerism.
In Context
- "The fetal echocardiogram revealed heterotaxy with multiple cardiac defects." — Prenatal diagnosis
- "Patients with heterotaxy require multidisciplinary evaluation for associated anomalies." — Management guideline