Assessment of Sudden Cardiac Death Risk in Pediatric Primary Electrical Disorders: A Comprehensive Overview.

Autor: Pupaza A; Department of Cardiology, Clinic Emergency Hospital Bucharest, 050098 Bucharest, Romania., Cinteza E; Department of Pediatrics, 'Carol Davila' University of Medicine and Pharmacy, 020021 Bucharest, Romania.; Department of Pediatric Cardiology, 'Marie Curie' Emergency Children's Hospital, 041451 Bucharest, Romania., Vasile CM; Pediatric and Adult Congenital Cardiology Department, M3C National Reference Centre, Bordeaux University Hospital, 33600 Bordeaux, France., Nicolescu A; Department of Pediatric Cardiology, 'Marie Curie' Emergency Children's Hospital, 041451 Bucharest, Romania., Vatasescu R; Department of Cardiology, Clinic Emergency Hospital Bucharest, 050098 Bucharest, Romania.; Cardio-Thoracic Department, 'Carol Davila' University of Medicine and Pharmacy Bucharest, 020021 Bucharest, Romania.
Jazyk: angličtina
Zdroj: Diagnostics (Basel, Switzerland) [Diagnostics (Basel)] 2023 Nov 28; Vol. 13 (23). Date of Electronic Publication: 2023 Nov 28.
DOI: 10.3390/diagnostics13233551
Abstrakt: Sudden cardiac death (SCD) in children is a devastating event, often linked to primary electrical diseases (PED) of the heart. PEDs, often referred to as channelopathies, are a group of genetic disorders that disrupt the normal ion channel function in cardiac cells, leading to arrhythmias and sudden cardiac death. This paper investigates the unique challenges of risk assessment and stratification for channelopathy-related SCD in pediatric patients-Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, idiopathic ventricular fibrillation, long QT syndrome, Anderson-Tawil syndrome, short QT syndrome, and early repolarization syndrome. We explore the intricate interplay of genetic, clinical, and electrophysiological factors that contribute to the complex nature of these conditions. Recognizing the significance of early identification and tailored management, this paper underscores the need for a comprehensive risk stratification approach specifically designed for pediatric populations. By integrating genetic testing, family history, and advanced electrophysiological evaluation, clinicians can enhance their ability to identify children at the highest risk for SCD, ultimately paving the way for more effective preventive strategies and improved outcomes in this vulnerable patient group.
Databáze: MEDLINE
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