Vnitr Lek 2025, 71(2):93-102 | DOI: 10.36290/vnl.2025.017

New trends in the diagnosis and treatment of hypertrophic cardiomyopathy

Mária Bakošová^{1, 2}, Hana Poloczková^{1, 2}, Tomáš Honek^{1, 2}, Anna Chaloupka^{1, 2}, Jan Krejčí^{1, 2}

¹ Fakultní nemocnice u sv. Anny v Brně, I. interní kardioangiologická klinika, Brno

² Lékařská fakulta Masarykovy univerzity, Brno

Hypertrophic cardiomyopathy (HCMP) is an inherited heart disease characterized by thickening of the left ventricular musculature that cannot be explained by hemodynamic load. The prevalence of HCMP is estimated to be 1:500, the disease has a good prognosis in general, but can lead to serious complications from dyspnea, arrhythmias, syncope to heart failure and sudden cardiac death. Today's progress in imaging techniques allows the identification of individual phenocopies of HCMP to target therapy properly. This paper mainly focuses on sarcomeric HCMP, where a recent breakthrough in treatment is the inclusion of the cardiac myosin inhibitor mavacamten in the therapeutic algorithm. Marginal attention is given to the diagnosis and therapy of other phenocopies of HCMP. Management of these diseases requires a multidisciplinary approach in specialized centers to target therapy precisely.

Keywords: hypertrophic cardiomyopathy, hypertrophy, LVOT obstruction, mavacamten, sudden cardiac death.

Accepted: March 18, 2025; Published: March 27, 2025  Show citation