# Systemic atrioventricular valve replacement due to a supravalvular stenosing ring and ebsteinoid tricuspid valve in a patient with congenitally corrected transposition of the great arteries: a case report

## TL;DR

A 23-year-old with a rare heart defect underwent successful valve replacement surgery after developing severe valve issues.

## Contribution

This case report highlights the surgical management of a rare anatomical variant in congenitally corrected transposition of the great arteries.

## Key findings

- The patient had severe systemic atrioventricular valve stenosis and regurgitation due to a supravalvular stenosing ring and Ebsteinoid tricuspid valve.
- Bioprosthetic valve replacement and membrane excision led to preserved valve function and favorable recovery.
- The case underscores the importance of individualized surgical decisions and multidisciplinary care in complex congenital heart defects.

## Abstract

Congenitally corrected transposition of the great arteries (ccTGA) is a rare and complex cardiac malformation often associated with additional anomalies. We present a 23-year-old patient with ccTGA, a supravalvular stenosing ring, and an Ebsteinoid tricuspid valve who developed severe systemic atrioventricular valve (SAVV) stenosis and regurgitation. Preoperative evaluation revealed tricuspid valve abnormalities, a dilated systemic right ventricle (sRV) with a reduced sRV ejection fraction (42%). The patient underwent bioprosthetic SAVV replacement and excision of the supravalvular membrane. Postoperatively, valve function was preserved, and recovery was favorable despite transient complications. This case emphasizes the surgical challenges and individualized decision-making required in ccTGA with rare anatomical variants, highlighting the value of timely intervention, multidisciplinary care, and long-term follow-up to optimize outcomes.