Right ventricular unloading for heart failure related to Ebstein malformation

Area of Science:

• Cardiovascular Surgery
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Ebstein malformation (EM) with severe right ventricular (RV) dilatation and dysfunction poses high operative risks.
• The bidirectional cavopulmonary shunt (BCPS) has shown promise in unloading the RV during EM repair.
• This study evaluates the 1.5-ventricle repair strategy for high-risk EM patients.

Purpose of the Study:

• To assess the efficacy and outcomes of the 1.5-ventricle repair strategy using BCPS in patients with advanced Ebstein malformation and severe RV dysfunction.
• To determine the early and intermediate-term results, including mortality, reintervention rates, and functional status.

Main Methods:

• A cohort of 62 patients with severe EM underwent tricuspid valve repair/replacement with concomitant BCPS between 1999 and 2013.
• Patients had severe RV dilatation (100%) and dysfunction (72.5%), with some requiring prior heart transplant evaluation.
• Concomitant procedures included atrial septal defect closure, maze, and mitral valve repair.

Main Results:

• Early mortality was low at 1.6%.
• Patients with preoperative low left ventricular ejection fraction (LVEF) showed significant improvement postoperatively (p=0.001).
• At a mean follow-up of 3.6 years, 88% of survivors were in New York Heart Association functional class I/II, with 8% requiring late reintervention.

Conclusions:

• Concomitant BCPS is a valuable adjunct for Ebstein malformation repair in patients with severe RV issues.
• The 1.5-ventricle repair strategy allows for low early mortality and good intermediate-term survival and quality of life.
• This approach can effectively delay or obviate the need for heart transplantation in many patients.