Association between common cardiovascular risk factors and clinical phenotype in patients with hypertrophic cardiomyopathy from the European Society of Cardiology (ESC) EurObservational Research Programme (EORP) Cardiomyopathy/Myocarditis registry.

Luís Rocha Lopes ,Jelena Čelutkienė ,Jens Mogensen ,José Manuel García‐Pinilla ,Cécile Laroche ,Eloisa Arbustini ,Eduardo Villacorta ,Aldo P Maggioni ,Luigi Tavazzi ,Albert Hagege ,Aleš Linhart ,Juan R Gimeno ,Hubert Seggewiß ,Dulce Brito ,Tomás Ripoll‐Vera ,Juan Pablo Kaski ,Alida L.p Caforio ,Nabeel Sheikh ,Philippe Charron ,Maria Angela Losi ,Perry Elliott

doi:10.1093/ehjqcco/qcac006

Abstract

The interaction between common cardiovascular risk factors (CVRF) and hypertrophic cardiomyopathy (HCM) is poorly studied. We sought to explore the relation between CVRF and the clinical characteristics of patients with HCM enrolled in the EURObservational Research Programme (EORP) Cardiomyopathy registry. 1739 patients with HCM were studied. The relation between hypertension (HT), diabetes (DM), body mass index (BMI), and clinical traits was analysed. Analyses were stratified according to the presence or absence of a pathogenic variant in a sarcomere gene. The prevalence of HT, DM, and obesity (Ob) was 37, 10, and 21%, respectively. HT, DM, and Ob were associated with older age (P<0.001), less family history of HCM (HT and DM P<0.001), higher New York Heart Association (NYHA) class (P<0.001), atrial fibrillation (HT and DM P<0.001; Ob p = 0.03) and LV (left ventricular) diastolic dysfunction (HT and Ob P<0.001; DM P = 0.003). Stroke was more frequent in HT (P<0.001) and mutation-positive patients with DM (P = 0.02). HT and Ob were associated with higher provocable LV outflow tract gradients (HT P<0.001, Ob P = 0.036). LV hypertrophy was more severe in Ob (P = 0.018). HT and Ob were independently associated with NYHA class (OR 1.419, P = 0.017 and OR 1.584, P = 0.004, respectively). Other associations, including a higher proportion of females in HT and of systolic dysfunction in HT and Ob, were observed only in mutation-positive patients. Common CVRF are associated with a more severe HCM phenotype, suggesting a proactive management of CVRF should be promoted. An interaction between genotype and CVRF was observed for some traits.

Highlights

Hypertrophic cardiomyopathy (HCM) is a common genetic disease associated with heart failure, atrial fibrillation (AF) and sudden cardiac death (SCD).(1) In many patients, theT disease is caused by mutations in genes encoding cardiac sarcomere proteins typified by RIP incomplete penetrance and variable clinical expression, even within families carrying the SC same causal variant. (1) Comorbidities such as hypertension provide a possible explanation U for some of this variability, but there are few data on the influence of common cardiovascular N risk factors (CVRF) on the HCM phenotype and almost no studies comparing the effect of A various CVRFs. (2),(3),(4),(5)
HT, DM U and Ob were associated with older age (p
M This study shows that patients with HCM enrolled into the EURObservational Research Programme (EORP) registry have a high prevalence of cardiovascular risk factors, comparable with data in the general European

Summary

Introduction

Hypertrophic cardiomyopathy (HCM) is a common genetic disease associated with heart failure, atrial fibrillation (AF) and sudden cardiac death (SCD).(1) In many patients, theT disease is caused by mutations in genes encoding cardiac sarcomere proteins typified by RIP incomplete penetrance and variable clinical expression, even within families carrying the SC same causal variant. (1) Comorbidities such as hypertension provide a possible explanation U for some of this variability, but there are few data on the influence of common cardiovascular N risk factors (CVRF) on the HCM phenotype and almost no studies comparing the effect of A various CVRFs. (2),(3),(4),(5). Hypertrophic cardiomyopathy (HCM) is a common genetic disease associated with heart failure, atrial fibrillation (AF) and sudden cardiac death (SCD).(1) In many patients, the. (1) Comorbidities such as hypertension provide a possible explanation U for some of this variability, but there are few data on the influence of common cardiovascular N risk factors (CVRF) on the HCM phenotype and almost no studies comparing the effect of A various CVRFs. ED is designed to collect prospective clinical data on patients with cardiomyopathies, with the IT aim of providing insight into disease characteristics and contemporary management of ED patients with heart muscle diseases in Europe(6),(7). Our goal was to report the N prevalence of common cardiovascular risk factors in patients with HCM and to determine U their association with the clinical phenotype.

Methods

Results

Conclusion