Histiocytoid cardiomyopathy management at the era of extracorporeal membrane assistance (ECMO): A series of 4 cases

Autor: Nicolas Combes, Olivia Domanski, Alice Maltret, Daphné Vens, Lucile Houyel, Nadir Benbrik, Fanny Bajolle, Damien Bonnet
Rok vydání: 2021
Předmět:
Zdroj: Archives of Cardiovascular Diseases Supplements. 13:311-312
ISSN: 1878-6480
DOI: 10.1016/j.acvdsp.2021.06.070
Popis: Introduction Histiocytoid cardiomyopathy is a rare life-threatening pediatric condition characterized by incessant ventricular tachyarrhythmia, dilated cardiomyopathy but also sudden cardiac death in over 20% of cases [1] . Management is challenging and aggressive therapy as catheter ablation [2] , [3] , surgical resection [4] and even heart transplantation is reported in the literature [5] . Methods Over the last decade, 4 patients were referred to our institution for incessant ventricular arrythmia consistent with histiocytoid cardiomyopathy (HC) diagnosis. We retrospectively reviewed clinical data in order to highlight the management and document natural history. Results Mean patient age at diagnosis was 7 months [1.5–16], 3 were girls. Mean weight at diagnosis was 6.65 kg [4.8–8.2]. Initial clinical presentation was incessant rapid ventricular tachycardia with right bundle branch block pattern ( Fig. 1 ) associated with dilated cardiomyopathy in 3, 1 presented with resuscitated sudden cardiac arrest (SCA). Despite association of antiarrhythmic drugs including amiodarone, hemodynamic deterioration required intubation for mechanical ventilatory support in 3 and extracorporeal membrane assistance (ECMO) in 2 patients. The patient diagnosed after SCA have implanted with a cardioverter defibrillator (ICD) at 4 months old ( Fig. 2 ). The first patient of this case series diagnosed 10 years ago, for who ECMO was not initiated, died of terminal cardiac failure despite rate control. Arrythmia burden decreased for the 3 others patients, allowing to slowly tapped down medication. Conclusion HC is known to be a rare severe condition affecting mostly girls under 2 years old. Initial management can be dreadful. Still, aggressive therapy such as ECMO and ICD can allow to overcome the acute phase. Based on this small case series and report in the literature [6] , secondary natural history seams favorable.
Databáze: OpenAIRE
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