Abstract
The most common genetic cardiomyopathy, HCM has a prevalence about 0.2%. It is transmitted autosomal dominant inheritance pattern. The natural history is benign but adverse outcomes can be happened in some patients including sudden cardiac death, symptoms secondary to dynamic left ventricular outflow tract (LVOT) obstruction, diastolic filling abnormalities, atrial fibrillation, and LV systolic dysfunction. Imaging modalities can be used to evaluate cardiac structure and function, the presence and severity of dynamic obstruction, mitral valve abnormalities, mitral regurgitation, as well as myocardial ischemia and fibrosis. Echocardiography is the initial imaging modality for evaluation of cardiac structure. CMR is recommended when echocardiographic images is not adequate in selected patients with high index of suspicion for HCM. In case of contraindication to CMR, patients with ICDs or pacemakers, Cardiac CT is recommended. Imaging can be used to guide treatment, screening and preclinical diagnosis in patient HCM.
Highlights
Hypertrophic cardiomyopathy (HCM) is the genetic cardiomyopathy that can be divided into two categories, hypertrophic obstructive cardiomyopathy (HOCM) and hypertrophic non-obstructive cardiomyopathy (HNCM)
A careful examination to see the presence of other structural heart diseases by imaging is crucial to do a systematic evaluation of the structure and function of the heart in terms of proper patient selection for further therapy
The pathogenesis of HCM is due to the presence of mutated genes responsible for producing sarcomere complex proteins including heavy-chain beta myosin protein, troponin, and myosin-binding protein C, which will result in impaired contraction of heart muscle.[5]
Summary
Hypertrophic cardiomyopathy (HCM) is the genetic cardiomyopathy that can be divided into two categories, hypertrophic obstructive cardiomyopathy (HOCM) and hypertrophic non-obstructive cardiomyopathy (HNCM). Prevalence of HCM is 1:500 in the general population. It is an autosomal dominant disease caused by over 1,400 mutations in at least 11 genes encoding proteins of the cardiac sarcomere.[1] the majority of patients with HCM is asymptomatic, subset remains at risk for having sudden death. The rate of mortality of HCM in the general population ranges
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