Common atrioventricular valve failure during single ventricle palliation†

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Single ventricle physiology presents complex challenges in congenital heart disease management.
• The common atrioventricular valve is a frequent anomaly in these patients, impacting long-term outcomes.
• Understanding the natural history of valve function is crucial for risk stratification and management.

Purpose of the Study:

• To determine the lifetime risk of atrioventricular valve failure in patients with single ventricle physiology and a common atrioventricular valve.
• To evaluate the incidence of valve intervention or moderate-to-greater regurgitation.
• To assess the long-term valve function trajectory in this specific patient population.

Main Methods:

• Retrospective analysis of a bi-national, population-based registry of Fontan survivors.
• Inclusion of 136 patients with common atrioventricular valve and available echocardiographic follow-up.
• Assessment of atrioventricular valve repair/replacement or moderate-to-greater regurgitation incidence.

Main Results:

• The cumulative incidence of atrioventricular valve failure by 28 years was 62%.
• Twenty-eight patients required initial valve repair or replacement, with significant rates of re-intervention or persistent regurgitation.
• An additional 30 patients developed moderate-to-greater regurgitation, impacting functional status and Fontan circulation.

Conclusions:

• Patients with single ventricle physiology and a common atrioventricular valve experience progressive valve dysfunction.
• The majority of these patients will encounter atrioventricular valve failure within the first three decades of life.
• Continuous monitoring and timely intervention are essential for managing valve complications in this high-risk group.