Systolic Heart Failure in a Patient With Hypertrophic Obstructive Cardiomyopathy: A Potentially Life-Threatening Complication

This disease is incurable; treatment is focused on improving patients’ signs and symptoms and decreasing complications.

Hypertrophic obstructive cardiomyopathy (HOCM) is a genetically transmitted disease that affects approximately 0.2% to 4% of the US population.¹ It is the result of a mutation in multiple genes that encode protein in the cardiac sarcomere, the primary contractile unit of the cardiac muscle.² HOCM is typically inherited in an autosomal manner, with variable penetrance and variable expressivity.³ Approximately half of all cases of HOCM have an autosomal dominant pattern; the rest are the result of sporadic gene mutations.^3,4 Recently, HOCM was linked to the cardiac myosin heavy-chain genes on chromosome 14 in some families, suggesting genetic heterogeneity.⁵ The age at which clinical manifestations become apparent varies. The existence of different disease genes or mutations in a gene may account for the differences in genotypic and phenotypic...