Molecular underpinnings of myocardial stiffness in patients with hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiovascular disorder with a phenotypic expression in 0.2% of the community [ [1] Maron B.J. Clinical course and management of hypertrophic cardiomyopathy. N. Engl. J. Med. 2018; 379: 655-668 Crossref PubMed Scopus (237) Google Scholar ]. Since its first comprehensive description almost 60 years ago [ [2] Braunwald E. Lambrew C.T. Rockoff S.D. Ross Jr., J. Morrow A.G. Idiopathic hypertrophic subaortic stenosis. I. A description of the disease based upon an analysis of 64 patients. Circulation. 1964; 30: 3-119 PubMed Google Scholar ] HCM has emerged as a highly heterogeneous disease with diverse genetic background, clinical presentation and natural history. While many patients with HCM remain free of symptoms and adverse events, a significant proportion of affected individuals evolve along discrete disease pathways – most commonly leading to heart failure (HF). [ [3] Rowin E.J. Maron M.S. Chan R.H. Hausvater A. Wang W. Rastegar H. et al. Interaction of adverse disease related pathways in hypertrophic cardiomyopathy. Am. J. Cardiol. 2017; 120: 2256-2264 Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar ].