Decompensated heart failure in an adolescent with Naxos syndrome managed with central ECMO and HeartMate III LVAD: A case report

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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Publication Details

Journal
The International Journal of Artificial Organs
Published
2026-04-01
DOI
https://doi.org/10.1177/03913988261435548
Primary Topic
Cardiovascular Effects of Exercise
Type
article
Field-Weighted Citation Impact
0.00
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article

Decompensated heart failure in an adolescent with Naxos syndrome managed with central ECMO and HeartMate III LVAD: A case report

Sınan Sabit Kocabeyoğlu, Ümit Kervan, Mustafa Akdi
The International Journal of Artificial Organs
Cardiovascular Effects of Exercise
article

Decompensated heart failure in an adolescent with Naxos syndrome managed with central ECMO and HeartMate III LVAD: A case report

Sınan Sabit Kocabeyoğlu, Ümit Kervan, Mustafa Akdi
article en

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.

The International Journal of Artificial OrgansVol. 49(4)
Sağlık Bilimleri Üniversitesi (TR), Yüksek İhtisas Üniversitesi (TR)
Peace, Justice and strong institutions
Openalex Percentile: Top 28%
Cardiovascular Effects of Exercise
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