From the Guidelines
Treatment for hypertrophic cardiomyopathy (HCM) should prioritize symptom management and prevention of complications, with first-line medications including beta-blockers or calcium channel blockers to reduce heart rate, improve filling, and decrease outflow obstruction, as recommended by the 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR guideline 1. The goal of treatment is to alleviate symptoms such as exertional dyspnea and chest discomfort, while also preventing complications like sudden cardiac death and systemic thromboembolism.
- Key considerations in managing HCM include:
- Pharmacological therapy targeted at dynamic left ventricular (LV) obstruction for symptom relief, with nonvasodilating beta blockers considered first-line therapy 1
- Calcium channel blockers, such as verapamil or diltiazem, as reasonable alternatives to beta-blocker therapy 1
- Advanced therapies, including disopyramide, mavacamten (a cardiac myosin inhibitor), or septal reduction, for patients who do not respond to initial treatments 1
- Elimination of medications that may promote outflow tract obstruction, such as pure vasodilators and high-dose diuretics 1
- Low-dose diuretics, when added to other first-line medications, for patients with persistent dyspnea or congestive symptoms 1 In addition to pharmacological management, lifestyle modifications are essential, including avoiding dehydration, excessive alcohol, and intense competitive sports.
- Regular cardiology follow-up with imaging is necessary to monitor disease progression and adjust treatment, as the natural history of HCM can be altered by various therapeutic interventions, including implantable cardioverter-defibrillators (ICDs) for secondary or primary prevention of sudden death, surgical septal myectomy, alcohol septal ablation, and heart transplantation for severe unrelenting symptoms 1.
From the FDA Drug Label
Patients with hypertrophic cardiomyopathy (IHSS): In 120 patients with hypertrophic cardiomyopathy (most of them refractory or intolerant to propranolol) who received therapy with verapamil at doses up to 720 mg/day, a variety of serious adverse effects were seen
- Verapamil can be used to treat Hypertrophic Cardiomyopathy (HCM), but with caution due to potential serious adverse effects.
- The use of verapamil in HCM patients requires careful monitoring and dose adjustment to minimize the risk of adverse effects such as pulmonary edema, severe hypotension, and AV block 2.
From the Research
Treatment Options for Hypertrophic Cardiomyopathy (HCM)
The treatment for HCM aims to provide symptomatic relief and prevent sudden death. The following are some of the treatment options:
- Medical treatment with negative inotropic agents, such as beta-blockers and verapamil, is the first therapeutic choice in all symptomatic patients 3.
- Beta-blockers appear to have prognostic merit in children 3.
- Verapamil therapy was not associated with a higher incidence of adverse events than β-blocker therapy in a real-world cohort of low-risk patients with HCM 4.
- Disopyramide can be added to patients who are still symptomatic after β-blockade or verapamil, and pharmacological control of symptoms can be achieved in a significant proportion of patients 5.
- Surgical myectomy and percutaneous septal ablation are standard treatments for patients with drug-resistant disease 5, 6.
Surgical and Interventional Procedures
The following are some of the surgical and interventional procedures used to treat HCM:
- Surgical myectomy is effective in reducing symptoms in children with left ventricular (LV) obstruction who are unresponsive to medical treatment 3.
- Percutaneous septal ablation can be regarded as a feasible alternative to surgical myectomy in some patients 3.
- Septal reduction therapy, such as myectomy or septal ablation, can be used to reduce the thickness of the septum and improve symptoms 7.
Prevention of Sudden Death
The prevention of sudden death is an important aspect of HCM treatment. The following are some of the strategies used to prevent sudden death:
- Regular clinical risk stratification for sudden death is of vital importance for the prevention of sudden death in young patients 3.
- Implantable cardioverter defibrillator (ICD) implantation is recommended for patients who are at high risk of sudden death 3, 6.
- Amiodarone can be used as a bridge in children at high risk of sudden death, until they reach adulthood or until their physical growth permits ICD implantation as long-term therapy 3.