Abstract
Background:
Advanced heart failure in inherited arrhythmogenic cardiomyopathies poses significant therapeutic challenges, particularly in pediatric patients. Naxos syndrome represents a rare form of such cardiomyopathy, in which mechanical circulatory support may be required.
Case description:
We present a case of a 14-year-old girl with Naxos syndrome who developed refractory cardiac decompensation complicated by malignant ventricular arrhythmia, necessitating central veno-arterial extracorporeal membrane oxygenation (VA-ECMO) followed by implantation of a HeartMate III left ventricular assist device (LVAD).
Conclusion:
This report highlights the multidisciplinary management, surgical decision-making, and perioperative challenges encountered during treatment, emphasizing the role of central ECMO as an effective bridge to durable LVAD support and heart transplantation in young patients with complex cardiomyopathies.
Keywords
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