Stage-specific therapy for hypertrophic cardiomyopathy

Abstract

Hypertrophic cardiomyopathy (HCM) is the most common inherited myocardial disease and is defined by otherwise unexplained left ventricular hypertrophy. The main complications include heart failure and arrhythmias such as atrial fibrillation and ventricular arrhythmias. Current treatment rests on septal reduction therapies, prevention of sudden cardiac death through implantable cardioverter defibrillator, and use of drugs such as beta-blockers, calcium antagonists, or amiodarone. In the last years, new pharmacological agents specifically targeting the pathophysiology of the disease have been developed with encouraging results in terms of functional capacity and symptoms improvement from clinical trials. In this review, we summarize the possible treatment approaches for each phase of the natural history of the disease: pre-phenotype expression, classic phenotype, adverse remodelling, and overt dysfunction.

Keywords: Heart failure; Hypertrophic cardiomyopathy; Therapy.

Figure 1

Stages of hypertrophic cardiomyopathy. LVEF, left ventricular ejection fraction.

Figure 2

Stage-specific therapy in hypertrophic cardiomyopathy. ASA, alcohol septal ablation; AF, atrial fibrillation; ARNI, angiotensin receptor-neprilysin inhibitor; SGLT2i, sodium-glucose transporter 2 inhibitors; CRT, cardiac resynchronization therapy; LVAD, left ventricular assist device.