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

Authors

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

Keywords:

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

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

Downloads

Download data is not yet available.

References

Karl TR, Jacobs JP. Congenitally corrected transposition of the great arteries [Internet]. In: Mavroudis C, Backer CL (ed). Pediatric Cardiac Surgery. John Wiley & Sons, Ltd; 2023 [cited 2024 Jan 16]. p. 581-600. Available from: https://onlinelibrary. wiley.com/doi/abs/10.1002/9781119282327.ch26

Kutty S, Danford DA, Diller GP, Tutarel O. Contemporary management and outcomes in congenitally corrected transposition of the great arteries. Heart. 2018;104(14):1148- 55. doi: 10.1136/heartjnl-2016-311032.

Kumar TKS. Congenitally corrected transposition of the great arteries. J Thorac Dis. 2020;12(3):1213. doi: 10.21037/ jtd.2019.10.15.

Spigel Z, Binsalamah ZM, Caldarone C. Congenitally corrected transposition of the great arteries: anatomic, physiologic repair, and palliation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2019;22:32-42. doi:10.1053/j. pcsu.2019.01.008.

Emani SM, Del Nido PJ. Systemic atrioventricular valve regurgitation in corrected transposition of the great arteries: where are we? J Am Coll Cardiol. 2023; 5;82(23):2209-11. doi: 10.1016/j.jacc.2023.10.022.

Mulla S, Asuka E, Bora V, Siddiqui WJ. Tricuspid regurgitation. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 [cited 2024 Jan 17]. Available from: http:// www.ncbi.nlm.nih.gov/books/NBK526121/

Abdelrehim AA, Stephens EH, Miranda WR, Todd AL, Connolly HM, Egbe AC, et al. Systemic atrioventricular valve surgery in patients with congenitally corrected transposition of the great vessels. J Am Coll Cardiol. 2023;5;82(23):2197-208. doi: 10.1016/j.jacc.2023.09.822.

Singh DP, Hussain K, Mahajan K. Ebstein anomaly and malformation. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 [cited 2024 Jan 17]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK534824/

Bini RM, Favilli S, Murzi B, Moschetti R, Santoro G, Traini AM. An unusual case of tricuspid lesion in congenital corrected transposition of the great arteries. J Cardiovasc Med. 2007;8(4):281-3. doi: 10.2459/01.JCM.0000263488.07339.d2.

Marino B, Sanders SP, Parness IA, Colan SD. Obstruction of right ventricular inflow and outflow in corrected transposition of the great arteries (S,L,L): two-dimensional echocardiographic diagnosis. J Am Coll Cardiol. 1986;8(2):407-11. doi: 10.1016/s0735-1097(86)80059-6.

Ritter M, Schneider J, Schmidlin D, Jenni R. Supravalvular tricuspid ring and Ebstein’s anomaly in congenitally corrected transposition. Am J Cardiol. 1992;15;70(20):1635-6. doi: 10.1016/0002-9149(92)90479-i.

Chesler E, Beck W, Barnard CN, Schrire V. Supravalvular stenosing ring of the left atrium associated with corrected transposition of the great vessels. Am J Cardiol. 1973;31(1):84- 8. doi: 10.1016/0002-9149(73)90815-1.

Allwork SP, Bentall HH, Becker AE, Cameron H, Gerlis LM, Wilkinson JL, et al. Congenitally corrected transposition of the great arteries: morphologic study of 32 cases. Am J Cardiol. 1976;38(7):910-23. doi: 10.1016/0002-9149(76)90804-3.

Kumar S, Phadke M, Kerkar P. Supratricuspid obstructive membrane in congenitally corrected transposition of the great arteries. Ann Pediatr Cardiol. 2013;6(2):204-5. doi: 10.4103/0974-2069.115287.

Henning RJ. Tricuspid valve regurgitation: current diagnosis and treatment. Am J Cardiovasc Dis. 2022;12(1):1-18.