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

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

Naxos syndrome central veno-arterial ECMO HeartMate III left ventricular assist device case report

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References

Protonotarios

Tsatsopoulou

Naxos disease and Carvajal syndrome: cardiocutaneous disorders that highlight the pathogenesis and broaden the spectrum of arrhythmogenic right ventricular cardiomyopathy. Cardiovasc Pathol 2004; 13(4): 185–194.

Protonotarios

Tsatsopoulou

Anastasakis

, et al. Genotype–phenotype assessment in autosomal recessive arrhythmogenic right ventricular cardiomyopathy (Naxos disease) caused by a deletion in plakoglobin. J Am Coll Cardiol 2001; 38(5): 1477–1484.

Protonotarios

Tsatsopoulou

Naxos disease: cardiomyocutaneous syndrome due to cell adhesion defect. Orphanet J Rare Dis 2006; 1: 4.

McKenna

Thiene

Nava

, et al. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J 1994; 71(3): 215–218.

Richards

Aziz

Bale

, et al. Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology. Genet Med 2015; 17(5): 405–424.

Gatzoulis

Protonotarios

Tsatsopoulou

, et al. Implantable defibrillator therapy in Naxos disease. Pacing Clin Electrophysiol 2000; 23(7): 1176–1178.

Corrado

Basso

Leoni

, et al. Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 2003; 108(25): 3084–3091.

Wichter

Breithardt

Borggrefe

, et al. Efficacy of antiarrhythmic drugs in patients with arrhythmogenic right ventricular disease. Circulation 1992; 86(1): 29–37.

McDonagh

Metra

Adamo

, et al. 2021 ESC guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J 2021; 42(36): 3599–3726.

10.

Kocabeyoglu

SS.

Is levosimendan a good weapon in preserving right ventricle function after left ventricular assist device implantation or not?

Eur J Cardiothorac Surg 2023; 63(5): ezad166.

11.

Brown

Moynihan

Deatrick

, et al. Extracorporeal Life Support Organization (ELSO): guidelines for pediatric cardiac failure. ASAIO J 2021; 67(5): 463–475.

12.

Marquez

Vargas-Gutierrez

Todd

, et al. A single-center experience and outcomes with central and peripheral cannulations for paediatric extracorporeal cardiopulmonary resuscitation. Resuscitation 2025; 214: 110678.

13.

O’Connor

Lorts

Davies

, et al. Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: a multicenter registry analysis. J Heart Lung Transplant 2020; 39(6): 573–579.

14.

O’Connor

Shezad

Ahmed

, et al. Expanding use of the HeartMate 3 ventricular assist device in pediatric and adult patients within the advanced cardiac therapies improving outcomes network (ACTION). J Heart Lung Transplant 2023; 42(11): 1546–1556.

15.

Rodenas-Alesina

Brahmbhatt

Rao

, et al. Prediction, prevention, and management of right ventricular failure after left ventricular assist device implantation: a comprehensive review. Front Cardiovasc Med 2022; 9: 1040251.