Overview
Definition
A rare congenital heart defect characterized by the downward displacement of the septal and posterior leaflets of the tricuspid valve into the right ventricle.
Epidemiology
Occurs in ~1 in 200,000 live births. Strongly associated with maternal Lithium use in pregnancy.
Etiology & Risk Factors
- Failure of proper delamination of the tricuspid valve leaflets during embryonic development
Clinical Symptoms
- Cyanosis
- Right-sided heart failure (hepatomegaly, ascites, edema)
- Arrhythmias (SVT, associated with WPW syndrome)
- Fatigue on exertion
Clinical Approach
Diagnosis
- Echocardiogram showing atrialization of the right ventricle and severe tricuspid regurgitation
Management
- Tricuspid valve repair or replacement
- Treatment of heart failure and control of tachyarrhythmias
Complications
- Right Heart Failure
- Paradoxical embolization / Stroke
- Atrial Fibrillation
- Sudden Death