Hypertrophic cardiomyopathy (HCM), the most common inherited cardiovascular disease (1:500), is usually characterized by markedly heterogeneous morphologic, functional, and clinical spectra. Sudden cardiac death (SCD), albeit rare, is undoubtedly the most devastating clinical manifestation, however the development of heart failure (HF) represents the actual major concerns.
In recent years, we highlighted the possibility to achieve a global risk characterization of HCM patients throughout an accurate analysis of their functional capacity by means of cardiopulmonary exercise test (CPET). Anyway the pathophysiology of HCM exercise limitation still remains tremendously complex and, up to now, few studies specifically explored it multidimensionally (i.e. relationship between ECG, gas exchange, autonomic nervous system variables).
Therefore, following our recent research track, we plan to investigate the exercise capacity in a large cohort of stable HCM outpatients on optimized therapy. Specifically, we are going to organize two distinct protocols, the first focusing on the exercise capacity pathophysiology (single-center experimental study) whereas the second one on a possible prognostic role of some CPET variables.