Abstract 11709: The Impact of Hypertension on Disease Expression in Hypertrophic Cardiomyopathy

Abstract

Introduction: Pressure overload from hypertension (HTN) is an important cause of left ventricular hypertrophy (LVH) in the general population, however its impact on disease expression in hypertrophic cardiomyopathy (HCM), and differences based on the presence/absence of a sarcomeric gene variant are not well characterized. Hypothesis: HTN is associated with more prominent manifestations of HCM. Methods: Genotyped HCM patients in the Sarcomeric Human Cardiomyopathy Registry were designated SARC+ ((likely) pathogenic sarcomeric variant present) or SARC- and categorized as having HTN (≥140/90 mmHg) or normal blood pressure (nonHTN; <140/90 mmHg). Heart failure (HF; LVEF<35%, NYHA III-IV, transplant/LVAD), ventricular arrhythmias (VA; sudden death, resuscitated arrest, appropriate ICD discharge), atrial fibrillation (AF), and an overall composite (HF, VA, AF, death) were assessed by Kaplan-Meier analysis and logistic regression. Results: Of 1706 patients (62% male), 445 (26%) had HTN. Median follow up was 4.2 years. SARC+ nonHTN patients were youngest and SARC- HTN patients oldest (p<0.001; Table). Obstructive physiology was most common in SARC- HTN. SARC+ nonHTN patients had the greatest wall thickness, although absolute differences were minimal (Table). HTN was associated with HF and the overall composite outcome, but not VA or AF (Figure). On multivariable analysis, HTN predicted the HF composite (OR 1.59 [95% CI 1.07-2.26], p=0.02), especially in SARC+ patients (OR 2.34 [95% 1.33-4.11], p=0.003). Conclusions: HTN is associated with worse HF outcomes in HCM, especially among SARC+ patients, but has minimal impact on the degree of cardiac remodeling.