Extracorporeal membrane oxygenation after prosthetic valve replacement in a child with neonatal Marfan syndrome: a case report.

ABSTRACT:

Background

Neonatal Marfan syndrome (nMFS), the most severe form of Marfan syndrome, is a rare condition that presents a clinical and treatment challenge. nMFS has high infant mortality related to progressive valvular dysfunction. Valve replacement in this setting improves long-term prognosis but carries high morbidity and mortality. Thus, sharing clinical experience in treating such patients is valuable.

Case summary

A 2 year old with nMFS underwent tricuspid valve annuloplasty and prosthetic mitral valve replacement. Postoperative management was complicated by pulmonary hypertension, cardiogenic shock, and arrythmias. Venoarterial extracorporeal membrane oxygenation (VA-ECMO) helped overcome these challenges but posed a high risk for prosthetic valve thrombosis (PVT). Desp

SUBMITTER: Wadia T 

PROVIDER: S-EPMC9477202 | biostudies-literature | 2022 Sep

REPOSITORIES: biostudies-literature