ABSTRACT
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac condition, most often caused by a sarcomeric mutation, which results in myocardial hypercontractility, reduced compliance, myofibrillar disarray, hypertrophy of the left ventricle (LV), and fibrosis. HCM presents with many different phenotypes but can be largely sub-grouped into those with obstructive and non-obstructive phenotypes. Imaging and hemodynamic assessment tools are at the center of HCM evaluation and have both diagnostic and prognostic implications. Traditional treatment approaches are effective in reducing mortality but do not address the molecular underpinnings of HCM and still result in significant morbidity. Novel pharmacotherapies such as mavacamten and CK-274 are promising and will take HCM treatment armamentarium to the next level. In this chapter, we provide an overview of the clinical evaluation of HCM, traditional treatment approaches, novel therapeutics, electrophysiological considerations, and implications of HCM on lifestyle and patients’ livelihood.
