Clinical Characteristics and Follow-Up of Pediatric-Onset Arrhythmogenic Right Ventricular Cardiomyopathy.
| Autor: | Roudijk RW; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands; Netherlands Heart Institute, Utrecht, the Netherlands., Verheul L; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands., Bosman LP; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands; Netherlands Heart Institute, Utrecht, the Netherlands., Bourfiss M; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands., Breur JMPJ; Department of Pediatric Cardiology, Wilhelmina Children's Hospital, Utrecht, the Netherlands., Slieker MG; Department of Pediatric Cardiology, Wilhelmina Children's Hospital, Utrecht, the Netherlands., Blank AC; Department of Pediatric Cardiology, Wilhelmina Children's Hospital, Utrecht, the Netherlands., Dooijes D; Department of Genetics, University Medical Center Utrecht, University of Utrecht, Utrecht, the Netherlands., van der Heijden JF; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands., van den Heuvel F; Department of Pediatric Cardiology, University Medical Center Groningen, Groningen, the Netherlands., Clur SA; Department of Pediatric Cardiology, Emma Kinderziekenhuis, Amsterdam University Medical Center, Amsterdam, the Netherlands., Udink Ten Cate FEA; Academic Center for Congenital Heart Disease, Department of Pediatric Cardiology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, the Netherlands., van den Berg MP; Department of Cardiology, University Medical Center Groningen, Groningen, the Netherlands., Wilde AAM; Heart Centre, Department of Cardiology, Amsterdam University Medical Center, Amsterdam University, Amsterdam, the Netherlands., Asselbergs FW; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands; Institute of Cardiovascular Science, Faculty of Population Health Sciences, University College London, London, United Kingdom; Health Data Research UK and Institute of Health Informatics, University College London, London, United Kingdom., Peter van Tintelen J; Netherlands Heart Institute, Utrecht, the Netherlands; Department of Genetics, University Medical Center Utrecht, University of Utrecht, Utrecht, the Netherlands., Te Riele ASJM; Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands; Netherlands Heart Institute, Utrecht, the Netherlands. Electronic address: ariele3@umcutrecht.nl. |
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| Jazyk: | angličtina |
| Zdroj: | JACC. Clinical electrophysiology [JACC Clin Electrophysiol] 2022 Mar; Vol. 8 (3), pp. 306-318. Date of Electronic Publication: 2021 Dec 22. |
| DOI: | 10.1016/j.jacep.2021.09.001 |
| Abstrakt: | Objectives: The goal of this study was to describe characteristics, cascade screening results, and predictors of adverse outcome in pediatric-onset arrhythmogenic right ventricular cardiomyopathy (ARVC). Background: Although ARVC is increasingly recognized in children, pediatric ARVC cohorts remain underrepresented in the literature. Methods: This study included 12 probands with pediatric-onset ARVC (aged <18 years at diagnosis) and 68 pediatric relatives (aged <18 years at first evaluation) referred for cascade screening. ARVC diagnosis was based on 2010 Task Force Criteria. Clinical presentation, diagnostic testing, and outcomes (sustained ventricular tachycardia [VT]; heart failure) were ascertained. Predictors of adverse outcome were determined by using univariable logistic regression. Results: Pediatric-onset ARVC was diagnosed in 12 probands and 12 (18%) relatives at a median age of 16.6 years (interquartile range: 13.8-17.4 years), whereas 12 (18%) relatives reached ARVC diagnosis as adults (median age, 22.0 years; interquartile range: 20.0-26.7 years). Sudden cardiac death/arrest was the first disease manifestation in 3 (25%) probands and 3 (4%) relatives. In patients without ARVC diagnosis at presentation (n = 61), electrocardiogram and Holter monitoring abnormalities occurred before development of imaging Task Force Criteria (7.3 ± 5.0 years vs 8.4 ± 5.0 years). Clinical course was characterized by sustained VT (91%) and heart failure (36%) in probands, which were rare in relatives (2% and 0%, respectively). Male sex (P < 0.01), T-wave inversion V Conclusions: Pediatric ARVC carries high arrhythmic risk, especially in probands. Disease progression is particularly observed on electrocardiogram or Holter monitoring. Arrhythmic events are associated with male sex, T-wave inversions, premature ventricular complexes/runs, and reduced biventricular ejection fraction. Competing Interests: Funding Support and Author Disclosures This work was supported by the Dutch Heart Foundation (grant 2015T058 to Dr te Riele; CVON2015-12 eDETECT; 2012-10 PREDICT1; 2018-30 PREDICT2; CVON PREDICT Young Talent Program to Dr te Riele); and the UMC Utrecht Fellowship Clinical Research Talent to Dr te Riele. Further support to Dr Asselbergs by University College London Hospitals National Institute for Health Research Biomedical Research Centre was appreciated. The authors have reported that they have no relationships relevant to the contents of this paper to disclose. (Copyright © 2022. Published by Elsevier Inc.) |
| Databáze: | MEDLINE |
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