8th March 2024

Management

The treatment and prognosis depend on the severity of the disease. Asymptomatic patients require routine monitoring with surgery indicated for symptomatic adults. Medical therapy is used as a bridge towards surgery or for symptomatic control, which includes diuretics for volume overload, anticoagulation for arrhythmias and standard heart failure treatment. Antibiotic prophylaxis is indicated in cyanotic patients with Ebstein anomaly, patients with previous infective endocarditis; six months after surgical repair or in those with prosthetic material used for repair.

Indications for surgery include progressive RV failure; cyanosis with resting oxygen saturation < 90%, paradoxical embolism (ASD), arrhythmias, progressive cardiomegaly and severe tricuspid regurgitation with NYHA III-IV.