Low Risk of Hypertrophic Cardiomyopathy With Contemporary Management Strategies Implemented in Non-Referral Regional...

Area of Science:

• Cardiology
• Genetics
• Internal Medicine

Background:

• Major advances in hypertrophic cardiomyopathy (HCM) diagnosis and treatment have primarily occurred in specialized tertiary referral centers.
• It remains unclear if these therapeutic benefits extend to patients managed in regional or community-based settings.

Purpose of the Study:

• To assess the management and clinical outcomes of hypertrophic cardiomyopathy (HCM) patients in a non-referral, regional center.
• To compare outcomes with those of patients treated at a tertiary referral center.

Main Methods:

• A retrospective analysis of 214 hypertrophic cardiomyopathy (HCM) patients managed at a regional center over a 6.0 ± 3.2-year follow-up period.
• Comparison of HCM-related mortality, implantable cardioverter-defibrillator (ICD) therapy rates, and surgical/ablation interventions with a tertiary center cohort.

Main Results:

• The regional HCM cohort experienced a low HCM-related mortality rate (0.1% per year).
• Fifteen patients (7%) received appropriate ICD therapy for life-threatening ventricular tachyarrhythmias.
• Heart failure due to left ventricular outflow obstruction was successfully treated in 23 patients (11%) via septal myectomy or alcohol septal ablation.
• Outcomes, including mortality and ICD therapy, were comparable to a tertiary referral center population, with a more frequent benign clinical course in the regional cohort (62% vs 46%).

Conclusions:

• Effective contemporary hypertrophic cardiomyopathy (HCM) management strategies and outcomes achieved in referral centers can be successfully replicated in regional and non-referral settings.
• Hypertrophic cardiomyopathy (HCM) is a highly treatable disease compatible with normal longevity when managed in diverse clinical settings beyond specialized tertiary centers.