دورية أكاديمية

Individualized Family Screening for Arrhythmogenic Right Ventricular Cardiomyopathy

التفاصيل البيبلوغرافية
العنوان: Individualized Family Screening for Arrhythmogenic Right Ventricular Cardiomyopathy
المؤلفون: Muller, SA, Gasperetti, A, Bosman, LP, Schmidt, AF, Baas, AF, Amin, AS, Houweling, AC, Wilde, AAM, Compagnucci, P, Targetti, M, Casella, M, Calò, L, Tondo, C, van der Harst, P, Asselbergs, FW, van Tintelen, JP, Oerlemans, MIFJ, te Riele, ASJM
المصدر: Journal of the American College of Cardiology , 82 (3) pp. 214-225. (2023)
بيانات النشر: Elsevier BV
سنة النشر: 2023
المجموعة: University College London: UCL Discovery
مصطلحات موضوعية: ARVC, family screening, predictors, screening interval, ventricular arrhythmia
الوصف: BACKGROUND: Clinical guidelines recommend regular screening for arrhythmogenic right ventricular cardiomyopathy (ARVC) to monitor at-risk relatives, resulting in a significant burden on clinical resources. Prioritizing relatives on their probability of developing definite ARVC may provide more efficient patient care. OBJECTIVES: The aim of this study was to determine the predictors and probability of ARVC development over time among at-risk relatives. METHODS: A total of 136 relatives (46% men, median age 25.5 years [IQR: 15.8-44.4 years]) from the Netherlands Arrhythmogenic Cardiomyopathy Registry without definite ARVC by 2010 task force criteria were included. Phenotype was ascertained using electrocardiography, Holter monitoring, and cardiac imaging. Subjects were divided into groups with “possible ARVC” (only genetic or familial predisposition) and “borderline ARVC” (1 minor task force criterion plus genetic or familial predisposition). Cox regression was performed to determine predictors and multistate modeling to assess the probability of ARVC development. Results were replicated in an unrelated Italian cohort (57% men, median age 37.0 years [IQR: 25.4-50.4 years]). RESULTS: At baseline, 93 subjects (68%) had possible ARVC, and 43 (32%) had borderline ARVC. Follow-up was available for 123 relatives (90%). After 8.1 years (IQR: 4.2-11.4 years), 41 (33%) had developed definite ARVC. Independent of baseline phenotype, symptomatic subjects (P = 0.014) and those 20 to 30 years of age (P = 0.002) had a higher hazard of developing definite ARVC. Furthermore, patients with borderline ARVC had a higher probability of developing definite ARVC compared with those with possible ARVC (1-year probability 13% vs 0.6%, 3-year probability 35% vs 5%; P < 0.01). External replication showed comparable results (P > 0.05). CONCLUSIONS: Symptomatic relatives, those 20 to 30 years of age, and those with borderline ARVC have a higher probability of developing definite ARVC. These patients may benefit from more frequent ...
نوع الوثيقة: article in journal/newspaper
اللغة: English
العلاقة: https://discovery.ucl.ac.uk/id/eprint/10175076Test/
الإتاحة: https://discovery.ucl.ac.uk/id/eprint/10175076Test/
رقم الانضمام: edsbas.B65D7BDE
قاعدة البيانات: BASE