Selective right ventricular unloading and novel technical concepts in Ebstein's anomaly

Area of Science:

• Cardiology
• Pediatric Cardiac Surgery
• Congenital Heart Disease

Background:

• Favorable outcomes in Ebstein's anomaly depend on tricuspid valve competence and right ventricular function.
• Valve repair is preferred over prosthetic replacement to minimize morbidity.
• This study reports experience with valve-sparing intracardiac repair and bidirectional Glenn (BDG) shunts.

Purpose of the Study:

• To evaluate the efficacy of valve-sparing intracardiac repair for Ebstein's anomaly.
• To assess the impact of selective bidirectional Glenn (BDG) shunts on outcomes.
• To present novel techniques for tricuspid valve repair in Ebstein's anomaly.

Main Methods:

• A cohort of 57 non-neonatal patients with Ebstein's anomaly underwent repair between 1993 and 2008.
• Patients presented with symptomatic disease (NYHA class II-IV) and some with cyanosis.
• Novel valve reconstructive techniques were employed, with selective bidirectional Glenn (BDG) performed in 55% of patients.

Main Results:

• No early or late mortality was observed.
• 91% of patients achieved freedom from prosthetic valves.
• At midterm follow-up, all patients were acyanotic, in NYHA class I, with mild or less tricuspid regurgitation in 86%.

Conclusions:

• A protocol utilizing BDG for ventricular unloading in selected Ebstein's anomaly patients facilitates durable valve-sparing repair.
• Excellent functional midterm outcomes are achievable with a selective one and a half ventricle approach.
• Novel valve repair techniques combined with BDG offer a promising strategy for Ebstein's anomaly.