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

Nathalie Victoria Zacarías Mendoza; Milagros Elizabeth Napán Herrera; Diana Vanessa Carassa Rodríguez; Víctor Justo Robles Velarde

doi:10.47487/apcyccv.v6i4.522

Autores/as

Nathalie Victoria Zacarías Mendoza Facultad de Medicina «Alberto Hurtado», Universidad Peruana Cayetano Heredia, Lima, Perú. https://orcid.org/0000-0003-0343-1466
Milagros Elizabeth Napán Herrera Facultad de Medicina «Alberto Hurtado», Universidad Peruana Cayetano Heredia, Lima, Perú. https://orcid.org/0009-0009-8342-2594
Diana Vanessa Carassa Rodríguez Facultad de Medicina «Alberto Hurtado», Universidad Peruana Cayetano Heredia, Lima, Perú. https://orcid.org/0009-0005-4654-5430
Víctor Justo Robles Velarde Servicio de Cirugía Cardiovascular, Instituto Nacional Cardiovascular - INCOR, Lima, Perú. https://orcid.org/0000-0001-8840-3637

DOI:

https://doi.org/10.47487/apcyccv.v6i4.522

Palabras clave:

Congenitally Corrected Transposition of the Great Vessels, Congenital heart disease; Tricuspid Valve Stenoses, Ebstein´s Anomaly

Resumen

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 decisionmaking required in ccTGA with rare anatomical variants, highlighting the value of timely intervention, multidisciplinary care, and long-term follow-up to optimize outcomes.

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