Authors
Mathilde R Rivaud, John A Jansen, Pieter G Postema, Eline A Nannenberg, Yuka Mizusawa, Roel van der Nagel, Rianne Wolswinkel, Ingeborg van der Made, Gerard A Marchal, Sridharan Rajamani, Luiz Belardinelli, J Peter van Tintelen, Michael WT Tanck, Allard C van der Wal, Jacques MT de Bakker, Harold V van Rijen, Esther E Creemers, Arthur AM Wilde, Maarten P van den Berg, Toon AB van Veen, Connie R Bezzina, Carol Ann Remme
Publication date
2018/8/14
Journal
European heart journal
Volume
39
Issue
31
Pages
2898-2907
Publisher
Oxford University Press
Description
Aims
Management of patients with inherited cardiac ion channelopathy is hindered by variability in disease severity and sudden cardiac death (SCD) risk. Here, we investigated the modulatory role of hypertrophy on arrhythmia and SCD risk in sodium channelopathy.
Methods and results
Follow-up data was collected from 164 individuals positive for the SCN5A-1795insD founder mutation and 247 mutation-negative relatives. A total of 38 (obligate) mutation-positive patients died suddenly or suffered life-threatening ventricular arrhythmia. Of these, 18 were aged >40 years, a high proportion of which had a clinical diagnosis of hypertension and/or cardiac hypertrophy. While pacemaker implantation was highly protective in preventing bradycardia-related SCD in young mutation-positive patients, seven of them aged >40 experienced life-threatening arrhythmic events despite …
Scholar articles
MR Rivaud, JA Jansen, PG Postema, EA Nannenberg… - European heart journal, 2018