دورية أكاديمية
Clinical Characteristics and Follow-Up of Pediatric-Onset Arrhythmogenic Right Ventricular Cardiomyopathy
| العنوان: | Clinical Characteristics and Follow-Up of Pediatric-Onset Arrhythmogenic Right Ventricular Cardiomyopathy |
|---|---|
| المؤلفون: | Roudijk, RW, Verheul, L, Bosman, LP, Bourfiss, M, Breur, JMPJ, Slieker, MG, Blank, AC, Dooijes, D, van der Heijden, JF, van den Heuvel, F, Clur, SA, Udink ten Cate, FEA, van den Berg, MP, Wilde, AAM, Asselbergs, FW, Peter van Tintelen, J, te Riele, ASJM |
| المصدر: | JACC: Clinical Electrophysiology , 8 (3) pp. 306-318. (2022) |
| بيانات النشر: | ELSEVIER |
| سنة النشر: | 2022 |
| المجموعة: | University College London: UCL Discovery |
| مصطلحات موضوعية: | Science & Technology, Life Sciences & Biomedicine, Cardiac & Cardiovascular Systems, Cardiovascular System & Cardiology, arrhythmogenic right ventricular cardiomyopathy, cascade screening, genetics, heart failure, pediatric-onset, sudden cardiac death, ventricular tachycardia, TASK-FORCE CRITERIA, CHILDREN, DIAGNOSIS, RISK, ADOLESCENTS, AGE |
| الوصف: | 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 V1-V3 (P < 0.01), premature ventricular complexes/runs (P ≤ 0.01), and decrease in biventricular ejection fraction (P ≤ 0.01) were associated with VT occurrence. 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 ... |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | text |
| اللغة: | English |
| العلاقة: | https://discovery.ucl.ac.uk/id/eprint/10160735/1/Manuscript_PediatricARVC_V8_20210811_ACC.pdfTest; https://discovery.ucl.ac.uk/id/eprint/10160735Test/ |
| الإتاحة: | https://discovery.ucl.ac.uk/id/eprint/10160735/1/Manuscript_PediatricARVC_V8_20210811_ACC.pdfTest https://discovery.ucl.ac.uk/id/eprint/10160735Test/ |
| حقوق: | open |
| رقم الانضمام: | edsbas.44C92067 |
| قاعدة البيانات: | BASE |
| الوصف غير متاح. |