Neoaortic Dilation and Polyvalvar Dysfunction: The Coexistence of Marfan Syndrome with Hypoplastic Left Heart Syndrome

Sruti Rao; Rukmini Komarlu; Hani K. Najm; Brittany Hansen; Rene E. Rodriguez; Kenneth G. Zahka

doi:10.1177/21501351241313314

Neoaortic Dilation and Polyvalvar Dysfunction: The Coexistence of Marfan Syndrome with Hypoplastic Left Heart Syndrome

Sruti Rao, Rukmini Komarlu, Hani K. Najm, Brittany Hansen, Rene E. Rodriguez, Kenneth G. Zahka

Research output: Contribution to journal › Article › peer-review

Abstract

We present a unique case of progressive neoaortic root and neoascending aortic dilatation, with poly-valvar regurgitation in a patient with Marfan syndrome and hypoplastic left heart syndrome palliated to Fontan procedure. This progressive dilation and valvar dysfunction necessitated a modified Bentall procedure and tricuspid valve repair. Our case highlights the impact of both a primary and secondary aortopathy complicating the Fontan procedure and the need for mechanical aortic valve replacement.

Original language	English (US)
Journal	World Journal for Pediatric and Congenital Heart Surgery
DOIs	https://doi.org/10.1177/21501351241313314
State	Accepted/In press - 2025
Externally published	Yes

ASJC Scopus subject areas

Surgery
Pediatrics, Perinatology, and Child Health
Cardiology and Cardiovascular Medicine

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10.1177/21501351241313314

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AU - Hansen, Brittany

AU - Rodriguez, Rene E.

AU - Zahka, Kenneth G.

PY - 2025

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Neoaortic Dilation and Polyvalvar Dysfunction: The Coexistence of Marfan Syndrome with Hypoplastic Left Heart Syndrome

Abstract

ASJC Scopus subject areas

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