Hypertrophic Cardiomyopathy Management

First-Line Medical Management

The American College of Cardiology recommends beta-blockers as the first-line treatment for symptomatic patients with both obstructive and non-obstructive hypertrophic cardiomyopathy, with a goal of achieving a resting heart rate of less than 60-65 bpm 1, 2
Beta-blockers improve symptoms by slowing heart rate, improving diastolic function, reducing left ventricular filling pressures, and reducing myocardial oxygen demand in patients with hypertrophic cardiomyopathy 3
Beta-blockers should be used with caution in patients with sinus bradycardia or severe conduction disease, and are particularly important as primary medical therapy in neonates and children with HCM 1, 3

For patients who do not respond to beta-blockers, have side effects, or contraindications, non-dihydropyridine calcium channel blockers (verapamil or diltiazem) are recommended, with verapamil effective at reducing chest pain, improving exercise capacity, and improving stress myocardial perfusion defects in patients with hypertrophic cardiomyopathy 1, 2, 3
Verapamil should be started at low doses and titrated up as needed, with caution in patients with high outflow gradients, advanced heart failure, or sinus bradycardia 1, 2, 4

For patients with obstructive HCM who remain symptomatic despite beta-blockers or calcium channel blockers, disopyramide combined with a beta-blocker or verapamil may be beneficial, and loop or thiazide diuretics may be used cautiously to improve dyspnea and volume overload when present 1, 2, 3
Aldosterone antagonists may also be considered in some patients with refractory symptoms, with diuretics used intermittently or at chronic low doses to prevent symptomatic hypotension and hypovolemia 3

In patients with HCM who develop systolic dysfunction with LVEF <50%, guideline-directed therapy for heart failure with reduced ejection fraction is recommended, according to the American Heart Association 4
In patients with nonobstructive HCM who have a pathogenic cardiac sarcomere genetic variant and mild phenotype, valsartan may be beneficial to slow adverse cardiac remodeling, as suggested by the American College of Cardiology 3
Avoid using beta-blockers with either verapamil or diltiazem due to potential for high-grade atrioventricular block, as warned by the American College of Cardiology 1

Dihydropyridine calcium channel blockers (e.g., nifedipine) are potentially harmful in patients with resting or provocable LVOT obstruction, and vasodilators (ACE inhibitors, ARBs) should be used cautiously or avoided in obstructive HCM, as they may worsen symptoms, according to the American College of Cardiology 1, 2
Digitalis is potentially harmful in treating dyspnea in HCM patients without atrial fibrillation, as stated by the American Heart Association 1

For severely symptomatic patients with obstructive HCM despite optimal medical therapy, septal reduction therapy should be considered, including surgical myectomy and alcohol septal ablation, as recommended by the American College of Cardiology 1, 2
Septal reduction therapy should only be performed by experienced operators in comprehensive HCM clinical programs, and for patients with extensive apical hypertrophy extending to the midventricle who have refractory symptoms, transapical myectomy may be considered, according to the American College of Cardiology 1, 3