Sandbox/HCM
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mugilan Poongkunran M.B.B.S [2]
Overview
Hypertrophic cardiomyopathy (HCM), is a disease state characterized by unexplained LV hypertrophy associated with nondilated ventricular chambers in the absence any underlying disease, or with any degree of hypertrophy in genotypically positive individuals (genotype positive/phenotype negative or subclinical HCM). HCM is caused by autosomal dominant mutations in sarcomere genes which encode components of the contractile apparatus of the heart. The clinical manifestations of HCM individuals depends upon the site and extent of cardiac hypertrophy, having asymptomatic presentation to severe symptoms due to LV outflow obstruction, heart failure, myocardial ischemia, arrhythmia, mitral regurgitation or sudden cardiac death. The diagnosis of HCM is made with cardiac imaging (echocardiography or cardiac MRI), showing maximal LV wall thickness ≥ 15 mm in adults or thickness ≥ 2 SD above the mean for age, sex, body size in children and with genetic testing. The management of HCM involves risk stratification to ascertain which patients are at risk for sudden cardiac death, treatment of comorbidities, ICDs for secondary or primary prevention, pharmacological therapy to control heart failure, surgical options for progressive and drug-refractory heart failure to LV outflow obstruction (LVOT) and heart transplantation for systolic dysfunction with severe unrelenting symptoms.
Causes
FIRE: Focused Initial Rapid Evaluation
A Focused Initial Rapid Evaluation (FIRE) should be performed to identify patients in need of immediate intervention.
Boxes in red color signify that an urgent management is needed.
Complete Diagnostic Approach to Hypertrophic Obstructive Cardiomyopathy
A complete diagnostic approach should be carried out after a focused initial rapid evaluation is conducted and following initiation of any urgent intervention.
Treatment
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