Atrial Fibrillation Substrate and Catheter Ablation Outcomes in MYBPC3- and MYH7-Mediated Hypertrophic Cardiomyopathy

The effects of disease-causing MYBPC3 or MYH7 genetic variants on atrial myopathy, atrial fibrillation (AF) clinical course, and catheter ablation efficacy remain unclear. The aim of this study was to characterize the atrial substrate of patients with MYBPC3- or MYH7-mediated hypertrophic cardiomyopathy (HCM) and its impact on catheter ablation outcomes. A retrospective single-center study of patients with HCM who underwent genetic testing and catheter ablation for AF was performed. Patients with MYBPC3- or MYH7-mediated HCM formed the gene-positive cohort; those without disease-causative genetic variants formed the control cohort. High-density electroanatomical mapping was performed using a 3-dimensional mapping system, followed by radiofrequency ablation. Twelve patients were included in the gene-positive cohort (mean age 55.6 ± 9.9 years, 83% men, 50% MYBPC3, 50% MYH7, mean ejection fraction 59.3% ± 13.7%, mean left atrial [LA] volume index 51.7 ± 13.1 mL/m2, mean LA pressure 20.2 ± 5.4 mm Hg) and 15 patients in the control arm (mean age 61.5 ± 12.6 years, 60% men, mean ejection fraction 64.9% ± 5.1%, mean LA volume index 54.1 ± 12.8 mL/m2, mean LA pressure 19.6 ± 5.41 mm Hg). Electroanatomical mapping demonstrated normal voltage in 87.7% ± 5.03% of the LA in the gene-positive cohort and 94.3% ± 3.58% of the LA in the control cohort (P < 0.001). Of the abnormal regions, intermediate scar (0.1-0.5 mV) accounted for 6.33% ± 1.97% in the gene-positive cohort and 3.07% ± 2.46% in the control cohort (P < 0.01). Dense scar (<0.1 mV) accounted for 5.93% ± 3.20% in the gene-positive cohort and 2.61% ± 2.19% in the control cohort (P < 0.01). Freedom from AF at 12 months was similar between the gene-positive (75%) and control (73%) cohorts (P = 0.92), though a greater number of procedures were required in the gene-positive cohort. Patients with MYBPC3- or MYH7-mediated HCM undergoing AF ablation have appreciably more low-amplitude LA signals, suggestive of fibrosis. However, catheter ablation remains an effective rhythm-control strategy.