Associations between genotype and arrhythmia manifestations in patients with hypertrophic cardiomyopathy
10.18501/ija.2026.27.e3
- Author:
Hwajung KIM
;
Soyoon PARK
;
Nilar AUNG
;
Soohyun KIM
;
Sung-Hwan KIM
;
Yong-Seog OH
;
Young CHOI
- Publication Type:Research
- From:International Journal of Arrhythmia
2026;27(1):e3-
- CountryRepublic of Korea
- Language:English
-
Abstract:
Background and Objectives:Cardiac arrhythmia is highly prevalent in hypertrophic cardiomyopathy (HCM), but its relation to genotype has not been well established.We investigated the arrhythmia manifestations according to genotype in HCM patients.
Methods:This single-center, retrospective study included 119 patients with HCM who underwent genetic testing using a next-generation sequencing panel. Incidence of cardiac arrhythmias and long-term outcomes were analyzed according to the presence of pathogenic variants (PVs).
Results:PVs related to HCM were identified in 45 (37.8%) patients. Presentation of sustained ventricular tachycardia (VT) prior to HCM diagnosis was more prevalent in the PV-positive group. During a median follow-up of 45.9 months, the incidence of sustained VT was significantly higher in the PV-positive group (11.1% vs. 1.4%, hazard ratio, 8.71; 95% confidence interval, 1.02–74.61). A new implantable cardioverter defibrillator insertion rate was comparable in the 2 groups, but appropriate shocks were delivered in only 4 patients in the PV-positive group (8.9% vs. 0%, P = 0.037). Incidence of bradyarrhythmias requiring pacemaker and new-onset atrial fibrillation (AF) was not significantly different between the 2 groups. Among 56 patients with AF, there was no significant difference in treatment strategies in the 2 groups. However, sinus rhythm was maintained over the last 6 months of follow-up in 4/17 (23.5%) patients in the PV-positive group and 24/39 (61.5%) patients in the PV-negative group (P = 0.031).
Conclusions:Patients with PVs related to HCM exhibited higher incidence of life-threatening VTs. While overall AF incidence was similar between the groups, the presence of PVs was significantly associated with poorer rhythm outcomes of AF.
