Inherited Arrhythmias in the Pediatric Population: An Updated Overview

Autor: Marco Valerio Mariani, Nicola Pierucci, Francesca Fanisio, Domenico Laviola, Giacomo Silvetti, Agostino Piro, Vincenzo Mirco La Fazia, Cristina Chimenti, Marco Rebecchi, Fabrizio Drago, Fabio Miraldi, Andrea Natale, Carmine Dario Vizza, Carlo Lavalle
Jazyk: angličtina
Rok vydání: 2024
Předmět:
Zdroj: Medicina, Vol 60, Iss 1, p 94 (2024)
Druh dokumentu: article
ISSN: 1648-9144
1010-660X
DOI: 10.3390/medicina60010094
Popis: Pediatric cardiomyopathies (CMs) and electrical diseases constitute a heterogeneous spectrum of disorders distinguished by structural and electrical abnormalities in the heart muscle, attributed to a genetic variant. They rank among the main causes of morbidity and mortality in the pediatric population, with an annual incidence of 1.1–1.5 per 100,000 in children under the age of 18. The most common conditions are dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM). Despite great enthusiasm for research in this field, studies in this population are still limited, and the management and treatment often follow adult recommendations, which have significantly more data on treatment benefits. Although adult and pediatric cardiac diseases share similar morphological and clinical manifestations, their outcomes significantly differ. This review summarizes the latest evidence on genetics, clinical characteristics, management, and updated outcomes of primary pediatric CMs and electrical diseases, including DCM, HCM, arrhythmogenic right ventricular cardiomyopathy (ARVC), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), long QT syndrome (LQTS), and short QT syndrome (SQTS).
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