Romanian hypertrophic cardiomyopathy registry: Profile of patients included between 2014–2017

Background Hypertrophic cardiomyopathy (HCM) represents a disease with increased left ventricular (LV) wall thickness that is not solely explained by abnormal loading conditions. Purpose The aim of the present study was to obtain an overview of the patients with HCM admitted to cardiology centers and enrolled in the Romanian Hypertrophic Cardiomiopathy Registry (Ro-HCM). Methods Between Dec 2014–Jan 2017, 196 patients (pts) diagnosed with HCM in 7 tertiary cardiology centers were enrolled in the Ro-HCM, using a detailed form including clinical, laboratory, imaging and invasive data. Results Mean age at enrolment was 56 ± 15y, with a male preponderance (60.2%). Almost half pts (46.6%) presented obstructive HCM, while 47.9% had non-obstructive HCM. Maximal wall thickness was 20.3 ± 4.6 mm, while LV ejection fraction was 60 ± 8%. Eighty-three pts underwent coronary angiography. Atherosclerotic coronary artery disease was present in 18% and myocardial bridging in 9.6%. Cardiac magnetic resonance was performed in 12 pts. Symptoms of heart failure (dyspnea 67%, fatigue 54%) were the most prevalent; the majority of pts being in NYHA class II. Mean BNP value was 384 ± 463 pg/mL. The most common arrhythmias were atrial fibrillation (26%) and non-sustained ventricular tachycardia (17%). Mean sudden cardiac death risk score (SCD-RS) was 3.0 ± 2.3%. ICDs were implanted in 11 pts (5.5%). Patients were mainly treated with beta-blockers (72.4%), diuretics (26.5%) and oral anticoagulants (27.6%). Invasive treatment of LVOT obstruction was performed in a small number of pts (23 pts underwent myectomy, while 13 pts underwent alcohol septal ablation). Conclusion This study shows that the management of HCM requires access to a large range of diagnostic and treatment modalities, emphasizing the role of expert centers. The Ro-HCM describes the profile of patients with HCM treated in Romanian tertiary centres, the way the guidelines are applied and the weak points in management.