Refined Echocardiographic Assessment and Contemporary Medical Treatment of Obstructive Hypertrophic Cardiomyopathy

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.

Left Atrial Morphology, Size and Function in Patients With Transthyretin Cardiac Amyloidosis and Primary Hypertrophic Cardiomyopathy – Comparative Strain Imaging Study –

“…17,18 subsequent functional impairment, in which the fibers' disarray, microvasculature impairment, abnormal collagen deposits, ischemic spots and chronic LV pressure overload are, in turn, important prognosticators. 11, 12 In both conditions, there would be a clinical advantage in recognition of functional impairment of the LA, probably earlier than LV dysfunction, and recent studies indicate strain imaging as the most valuable noninvasive technique to disclose subclinical atrial dysfunction. 8, 13 LA morphofunctional changes are emerging as prognosticators in various cardiac diseases, but only scanty literature is available on comparative strain studies between TTR-CA and HCM patients, being all cardiac chambers target organs for either amyloid deposits or interstitial fibrosis.…”

Hypertrophic Cardiomyopathy, a Modern View of the Etiology and Treatment of This Pathology

“…ст. в покое требует назначения медикаментозной терапии, направленной на снижение степени таковой [10]. Фармакологическую коррекцию обструкции выносящего тракта ЛЖ следует также проводить больным с латентной и среднежелудочковой обструкцией [7,12,18].…”