14-year-old girl with Naxos syndrome, refractory cardiac decompensation, and malignant ventricular arrhythmia (n=1)
Central veno-arterial extracorporeal membrane oxygenation (VA-ECMO) followed by HeartMate III left ventricular assist device (LVAD)
Central VA-ECMO can serve as an effective bridge to durable LVAD support in pediatric patients with complex inherited arrhythmogenic cardiomyopathies like Naxos syndrome.
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.
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Mustafa Akdi
Ayşenur Yılmaz
Sinan Sabit Kocabeyoğlu
The International Journal of Artificial Organs
Sağlık Bilimleri Üniversitesi
Yüksek İhtisas Üniversitesi
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Akdi et al. (Sat,) studied this question.
www.synapsesocial.com/papers/69dc89473afacbeac03eb0d0 — DOI: https://doi.org/10.1177/03913988261435548