Abstract

Hypertrophic cardiomyopathy (HCM) is a complex cardiac disease, relatively common among genetic disorders. The prevalence is about 1:500 in the general population. Obstructive type occurs in about 25% of the cases. The clinical course is heterogeneous due to the large variety of genetic-based phenotypes with different prognostic impact. Primary HCM is one of the most important causes of sudden death in young people and may be a medical problem in athletes with physiologic left ventricular hypertrophy. In the last decade, however, several studies reported normal longevity. Echocardiography has emerged as the most important noninvasive method to make diagnosis and provide classifications of the disease. In this commentary, some out of the most recent and sophisticated applications of this method in HCM are reported. The most common pathophysiologic aspects and a proposal of classification of the obstruction-causing mechanisms, like systolic anterior motion of the mitral valve, papillary hypertrophy and dislocation, chordal slack, mid-cavity obliteration, are described. Some recent studies on coronary blood flow velocity and coronary reserve, performed by sophisticated Doppler echocardiography, have demonstrated important pathophysiological insights on microcirculatory impairment in patients with HCM. At present, advanced echocardiography surely improves the clinical management of these patients, and contributes to optimize the therapeutic strategies. However, the most appropriate framework of tests to be performed in the majority of them still remains a challenging clinical matter.

Keywords: Left Ventricular Mass, Systolic Flow Velocity, Coronary Flow Velocity Reserve, Alcohol septal ablation, Disopyramide