How to follow up Hypertrophic cardiomyopathy ? : Too much reliance on LVOT gradient is problematic

Hypertrophic cardiomyopathy (HCM)  is the most common primary disorder of cardiac muscle. The incidence is about 1 in 500, which would mean 1.5 crore HCM patients will be living on our planet at any moment. The root cause of pathology is located in 20 odd genes that define cardiac muscle protein integrity. (Myosin, Troponin, Titin, etc) This leads to the bizarre architecture of cardiac muscle, prone to progressive fibrosis.(Paradoxically, 90% of HCM have normal or supernormal contractility till very late stages, proving that the much-dreaded term myocardial disarray has little effect on contractility. It is all the more fun, as we strive hard to suppress this excess contractility caused by disarray with beta-blockers. SCD is the scary face of this disease. If th incidence of SCD is less than 1 %  per year, do a little maths to know how many will succumb every year to this disease. However, It is the symptoms like exertional dyspnea (most common,) followed by syncope and rarely angina that bring HCM  patients to the physician. Though the pathology is diffuse and global, I don’t understand why we got stuck with the outflow tract gradients and dynamic obstruction. HCM is an equally a disorder of LV inflow obstruction (rather a restriction). It can be presumed myocardial disarray makes more impact on diastole than systole. The relationship between inflow and outflow gradient is a poorly explored area in HCM. Detailed analysis of E and A velocity profiles along with tiss...
Source: Dr.S.Venkatesan MD - Category: Cardiology Authors: Tags: hypertrophic cardiomyopathy criley concept hocm hcm lobster claw hcm lobster claw is pulsus bisferiens mavacamten explorer trial what is the mechanism of hocm gradient lvot Source Type: blogs