학술논문
Catecholaminergic Polymorphic Ventricular Tachycardia in Children
Document Type
article
Author
Roston, Thomas M; Vinocur, Jeffrey M; Maginot, Kathleen R; Mohammed, Saira; Salerno, Jack C; Etheridge, Susan P; Cohen, Mitchell; Hamilton, Robert M; Pflaumer, Andreas; Kanter, Ronald J; Potts, James E; LaPage, Martin J; Collins, Kathryn K; Gebauer, Roman A; Temple, Joel D; Batra, Anjan S; Erickson, Christopher; Miszczak-Knecht, Maria; Kubuš, Peter; Bar-Cohen, Yaniv; Kantoch, Michal; Thomas, Vincent C; Hessling, Gabriele; Anderson, Chris; Young, Ming-Lon; Cabrera Ortega, Michel; Lau, Yung R; Johnsrude, Christopher L; Fournier, Anne; Kannankeril, Prince J; Sanatani, Shubhayan
Source
Circulation Arrhythmia and Electrophysiology. 8(3)
Subject
Language
Abstract
BackgroundCatecholaminergic polymorphic ventricular tachycardia is an uncommon, potentially lethal, ion channelopathy. Standard therapies have high failure rates and little is known about treatment in children. Newer options such as flecainide and left cardiac sympathetic denervation are not well validated. We sought to define treatment outcomes in children with catecholaminergic polymorphic ventricular tachycardia.Methods and resultsThis is a Pediatric and Congenital Electrophysiology Society multicenter, retrospective cohort study of catecholaminergic polymorphic ventricular tachycardia patients diagnosed before 19 years of age. The cohort included 226 patients, including 170 probands and 56 relatives. Symptomatic presentation was reported in 176 (78%). Symptom onset occurred at 10.8 (interquartile range, 6.8-13.2) years with a delay to diagnosis of 0.5 (0-2.6) years. Syncope (P