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      News & Perspective Drugs & Diseases CME & Education Academy Video Decision Point
      Drugs & Diseases > Cardiology

      Hypertrophic Cardiomyopathy Medication

      Updated: Apr 29, 2022
      • Author: Sandy N Shah, DO, MBA, FACC, FACP, FACOI; Chief Editor: Gyanendra K Sharma, MD, FACC, FASE  more...
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      Medication

      Medication Summary

      The purpose of pharmacologic therapy is to reduce the pressure gradient across the LV outflow tract by reducing the inotropic state of the left ventricle, improving compliance of the left ventricle, and reducing diastolic dysfunction. Mavacamten, a first-in-class allosteric inhibitor of cardiac myosin, has gained FDA approval for adults with symptomatic New York Heart Association class II-III obstructive hypertrophic cardiomyopathy (HCM) to improve exercise capacity and symptoms. Amiodarone has been shown to reduce the incidence of arrhythmogenic sudden cardiac death. [40, 41]

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      Beta-Adrenergic Blocking Agents

      Class Summary

      These reduce the inotropic state of the left ventricle. They decrease diastolic dysfunction and increase LV compliance, thereby reducing the pressure gradient across LV outflow tract. Beta-adrenergic blocking agents decrease heart rate, thus lowering myocardial oxygen consumption and reducing the potential for myocardial ischemia.

      Metoprolol (Lopressor, Toprol XL)

      This is a first-line therapy in the treatment of obstructive and nonobstructive hypertrophic cardiomyopathy (HCM). Rarely, patients may require up to 200 mg orally twice daily to achieve the desired effect. The dose is titrated to a heart rate of between 50 and 60 bpm.

      Atenolol (Tenormin)

      Atenolol selectively blocks beta1 receptors, with little or no effect on beta2 types.

      Sotalol (Betapace, Sorine)

      This is a class III antiarrhythmic agent that blocks K+ channels, prolongs action potential duration, and lengthens the QT interval. Sotalol is a noncardiac selective beta-adrenergic blocker that may be helpful in the conversion of atrial fibrillation and flutter to normal rhythm. It may also suppress the recurrence of atrial fibrillation and flutter.

      Propranolol (Inderal LA, InnoPran XL)

      Propranolol is a class II antiarrhythmic nonselective beta-adrenergic receptor blocker with membrane-stabilizing activity that decreases the automaticity of contractions. The

      dose is titrated to a heart rate of between 50 and 60 bpm.

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      Antiarrhythmics

      Class Summary

      These agents alter the electrophysiologic mechanisms responsible for arrhythmia.

      Disopyramide (Norpace, Norpace CR)

      Disopyramide decreases the inotropic state of the left ventricle. It decreases ventricular and supraventricular arrhythmias. The drug decreases diastolic dysfunction and increases LV compliance, thereby reducing the pressure gradient across the LV outflow tract. Disopyramide raises the threshold for atrial and ventricular ectopy.

      Amiodarone (Cordarone, Pacerone)

      Amiodarone is the only agent proven to reduce the incidence and risk of cardiac sudden death, with or without obstruction to LV outflow. It is very effective at converting atrial fibrillation and flutter to sinus rhythm and at suppressing the recurrence of these arrhythmias.

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      Calcium Channel Blockers

      Class Summary

      An alternative to beta blockers, calcium channel blockers decrease the inotropic state of the left ventricle, decrease the gradient across the LV outflow tract, decrease diastolic dysfunction, and increase diastolic filling of the left ventricle by improving LV diastolic relaxation. These agents may have a better effect on exercise performance.

      Verapamil (Calan, Isoptin, Verelan)

      During depolarization, verapamil inhibits calcium ions from entering slow channels or voltage-sensitive areas of the vascular smooth muscle and myocardium. The drug provides an alternative to beta-blocker therapy. It is useful in patients with moderate to severe chronic obstructive pulmonary disease (COPD).

      The use of short-acting calcium channel blockers is being discouraged because of numerous reports of adverse cardiac and hemodynamic events associated with their use, particularly in patients with known coronary artery disease.

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      Anticoagulants, Cardiovascular

      Class Summary

      Anticoagulants are used to prevent thromboembolic complications.

      Warfarin (Coumadin, Jantoven)

      Warfarin is a competitive, direct thrombin inhibitor. Thrombin enables fibrinogen conversion to fibrin during the coagulation cascade, thereby preventing thrombus development. It inhibits both free and clot-bound thrombin and thrombin-induced platelet aggregation. Warfarin is indicated for the prevention of stroke and thromboembolism associated with nonvalvular atrial fibrillation.

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      Cardiac Myosin Inhibitors

      Class Summary

      Excess myosin actin cross-bridge formation and dysregulation of the super-relaxed state are mechanistic hallmarks of HCM. 

      Mavacamten (Camzyos)

      Indicated for symptomatic New York Heart Association class II-III obstructive hypertrophic cardiomyopathy (HCM) to improve exercise capacity and symptoms in adults. Mavacamten is a selective allosteric inhibitor of cardiac myosin. Modulates number of myosin heads that can enter “on actin” (power-generating) states, thus reduces probability of force-producing (systolic) and residual (diastolic) cross-bridge formation. Mavacamten shifts overall myosin population towards an energy-sparing, recruitable, super-relaxed state. In patients with HCM, myosin inhibition with mavacamten reduces dynamic left ventricular outflow tract (LVOT) obstruction and improves cardiac filling pressures. 

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