Current management of hypertrophic cardiomyopathy

Abstract

Hypertrophic cardiomyopathy is a common yet under-recognized genetic structural heart condition characterized by left ventricular hypertrophy. Patients may present with obstructive disease characterized by an elevated left ventricular outflow tract gradient or non-obstructive disease. Long established medical and surgical treatment options for patients with obstructive hypertrophic cardiomyopathy and refractory symptoms can be effective in eliminating outflow tract gradients and improving symptoms. Cardiac myosin inhibitors have emerged as a new class of evidence based medical therapy for patients with obstructive hypertrophic cardiomyopathy and an alternative to septal reduction therapies. However, effective treatments for patients with non-obstructive hypertrophic cardiomyopathy remain limited, with several clinical trials ongoing. Variants in cardiac sarcomeric genes are the primary genetic cause of hypertrophic cardiomyopathy and are being investigated as targets for gene based therapies. Stratification of the risk of sudden death is an important component of caring for patients with hypertrophic cardiomyopathy. Recommendations for implantable cardioverter-defibrillator implantation are based on well validated risk factors in combination with shared decision making. Atrial fibrillation is common in patients with hypertrophic cardiomyopathy, and anticoagulation is strongly recommended for stroke prevention. Rhythm control is essential for patients with symptomatic atrial fibrillation. Historically, vigorous exercise has been restricted; however, newer data suggest that the arrhythmic risk is less than previously thought and emphasize an individualized approach. Advanced heart failure is an uncommon but important cause of morbidity and mortality. Early identification is key to improving outcomes with advanced therapies including cardiac transplantation. The management of hypertrophic cardiomyopathy is rapidly evolving toward a more personalized approach, based on genotype and phenotype, to alter disease progression and improve patients' outcomes.