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Fluid dynamic aspects of ejection in hypertrophic cardiomyopathy.

H ypertrophic cardiomyopathy (HCM) is a fascinating and polymorphic cardiac disease. Its impact on cardiac function retains intense clinical interest as it is one of the most common inherited cardiac diseases, affecting about 1 in 500 people. According to the prevalent view, HCM is a predominantly obstructive disease compromising systolic left ventricular (LV) ejection. Then again, impeded left ventricular orthograde emptying is not an invariable finding. Diastolic dysfunction associated with the abnormal hypertrophy patterns and their sequelae may also be responsible for impaired LV pumping performance. The diastolic peculiarities of LV dynamics in HCM were the focus of a companion survey. Additionally, the myocyte disorganization typifying HCM probably creates systolic contraction inefficiencies, and may be implicated in marked mechanical functional limitation in some HCM patients. As ejection flow characteristics became clinically measurable diagnostic features—with conventional and multisensor left heart catheterization, echocardiography, Doppler velocimetry, and MRI—details have been sought with a view to localizing LV outflow obstruction and characterizing the role of the systolic anterior motion (SAM) of the mitral valve. Mitral leaflet coaptation is disrupted by SAM, resulting in significant mitral regurgitation and impairing forward cardiac output; there can be an apparent “normalization” of the Doppler mitral inflow pattern, associated with the orthograde surge of the regurgitant volume ensuing in early filling.