• Many treatments are available for hypertrophic cardiomyopathy (HCM), and new treatments are on the way.
• The best treatment options for your HCM depend on the features of your disease and any complications you may have.

Hypertrophic cardiomyopathy (HCM) is a disease of muscle tissue in the heart that affects as many as 1 in 500 people in the United States, making it the most common inherited cardiomyopathy.

Although HCM does not cause symptoms for many people, it can lead to dangerous complications for others. Treatment of HCM can include medication, surgery, implanted devices, and lifestyle modifications. Treatment varies based on the symptoms HCM causes and the changes to the heart that it produces.

What Is Hypertrophic Cardiomyopathy?

HCM is an inherited disease caused by mutations in genes related to the proteins that make up heart muscle. HCM causes the walls of the left ventricle to become thickened, called left ventricular hypertrophy. This thickening can prevent the heart from pumping oxygen-rich blood to the body.

Symptoms of HCM can include shortness of breath, dizziness, palpitations, chest pain, fainting, and a fast or irregular heartbeat. Symptoms often only appear when performing exercise or strenuous physical activity. Some people with mild HCM may not experience any symptoms, while people with severe HCM may experience symptoms even while resting.

HCM can cause obstruction of blood flow through the aorta, which is called obstructive HCM or HCM with left ventricular outflow obstruction. HCM can also cause complications, such as arrhythmias and heart failure. Treatment options for HCM vary based on the features and health complications an individual has.

Treatment Options for Hypertrophic Cardiomyopathy

Treatment options for HCM include lifestyle modification, medication, and surgery, as well as the use of noninvasive procedures and implantable devices (such as pacemakers). Treatments are determined based on the features present with HCM, including which specific parts of the heart are involved and whether there are complications, including arrhythmia or heart failure.

If you don’t have symptoms from HCM, treatment may not be needed and may not have any benefit. However close follow-up with a cardiologist is recommended, along with regular echocardiograms to track any potential progression of disease.

Treatment guidelines recommend that people with moderate to severe HCM or HCM with complications seek care from a center that specializes in HCM treatment. HCM can be a complicated disease to treat. It is important to get care from doctors with experience treating it. For some surgeries unique to HCM, it is especially important to have surgeons with a great deal of experience.

Medication

Medication is the first line of treatment for HCM. Medications used to treat HCM include:

• Beta-blockers — These slow the heart’s rhythm and allow it to beat with less force. Examples include metoprolol (sold as Lopressor and Toprol-XL) and propranolol (Inderal and Innopran XL).
• Calcium channel blockers — These drugs prevent irregular heartbeat, relax blood vessels, and keep the heart from contracting too strongly. Diltiazem (sold as Cardizem and Tiazac) and verapamil (including the brands Verelan, Covera-HS, and Calan) are examples.
• Antiarrhythmic drugs — These medications, which include sodium channel blockers and potassium channel blockers, prevent irregular heartbeat. Some medications your doctor may prescribe include amiodarone (sold as Cordarone and Pacerone) or disopyramide (Norpace).
• Anticoagulants — Also called blood thinners, these help prevent blood clots and stroke. Warfarin (sold as Coumadin and Jantoven) is an example of a blood thinner.
• Cardiac myosin inhibitor — This type of treatment helps the heart muscle relax. Mavacamten (sold as Camzyos) is the first of this treatment to be approved by the U.S. Food and Drug Administration (FDA) for HCM. It was approved in 2022.

Surgery

If medication does not sufficiently control your condition, your doctor may recommend surgery to treat HCM. Surgical procedures for HCM can include:

• Septal myectomy — Reduces the thickness of the interventricular septum
• Alcohol septal ablation — Reduces septal wall thickness without invasive surgery
• Transapical myectomy or apical aneurysm repair — Treats problems in the lower tip, or apex, of the left ventricle
• Heart valve surgery — Repairs damage to the mitral valve or replaces the mitral valve
• Cardiac ablation — Stops conduction abnormalities, such as arrhythmias, using a catheter or, less commonly, heart surgery
• Heart transplant — Used in cases of severe HCM with heart failure

Implantable Devices

Implantable devices can be used to help control heart rate, prevent sudden cardiac death, regulate heartbeat, and help synchronize heart contractions. Devices include:

• Implantable cardioverter defibrillator (ICD) — Prevents sudden cardiac death by “shocking” the heart to correct dangerous arrhythmias
• Pacemaker — Used for cardiac resynchronization therapy to help correct abnormal heart contractions and prevent the heart from beating too slowly
• Left ventricular assist device — Used to treat severe heart failure to help the heart pump blood to the body

Lifestyle Changes

Certain lifestyle changes can help improve HCM symptoms. Regular mild to moderate exercise is recommended for most people with HCM. Avoiding competitive sports or intense exercise is also recommended, depending on how severe the HCM is.

Maintaining a healthy body weight can help the heart function better and prevent problems such as sleep apnea and high blood pressure that can make HCM worse.

What Determines the Best Treatment for Hypertrophic Cardiomyopathy?

Current guidelines for treating HCM recommend first-line treatments and advanced treatments based on specific circumstances. Medication is always the first choice, and the medications used depend on the features of HCM that are present. Surgical procedures and implantable devices are used when medication fails to achieve treatment goals.

Communication with your doctor is important when determining which treatment options are best for you. It is important to have two-way communication with your doctor to find treatments that support your goals and your personal circumstances.

Obstructive HCM

Obstructive HCM, or HCM with left ventricular outflow obstruction, is caused by thickening of the interventricular septum, the wall between the left and right ventricles. If the septum becomes too large, it can interfere with normal blood flow out of the heart through the aorta. Most cases of HCM are obstructive HCM.

Treatment for obstructive HCM begins with beta-blockers and calcium channel blockers. People who do not respond to these medications may need different calcium channel blockers or potassium channel blockers in addition to beta-blockers.

Alcohol septal ablation or a septal myectomy procedure may be needed if medication is insufficient to treat obstructive HCM. Additionally, surgery to repair damage to the mitral valve can help improve outcomes.

Nonobstructive HCM

Nonobstructive HCM is treated with beta-blockers and calcium channel blockers first. Diuretics, or water pills, may be used to treat shortness of breath in nonobstructive HCM. Transapical myectomy to enlarge the interior of the left ventricle may be used in some cases if medication alone does not help.

HCM With Atrial Fibrillation

Atrial fibrillation describes disorganized contractions of the atria, the chambers of the heart that lead to the ventricles. Beta-blockers and calcium channel blockers are the first-line treatment, but amiodarone, an antiarrhythmic drug, may also be prescribed.

Anticoagulants are needed to prevent blood clots and stroke caused by atrial fibrillation. Atrioventricular node ablation may be needed if drugs fail. This procedure requires a pacemaker.

HCM With Ventricular Arrhythmia

Ventricular arrhythmias caused by HCM can be life-threatening and usually require an ICD to prevent sudden cardiac death. Medication is used to help control arrhythmias, including beta-blockers and amiodarone or similar antiarrhythmic drugs.

If medication does not work, then ablation may be needed. Additionally, surgery may be needed to treat an apical aneurysm in the left ventricle, essentially a pouch of thinner heart muscle at the tip of the ventricle. A heart transplant may be needed if heart function is severely impaired.

HCM With Heart Failure

Heart failure is one of the more serious and debilitating complications of HCM. Beta-blockers and calcium channel blockers may be used to treat HCM and heart failure, as well as an ICD.

ICDs typically also function as pacemakers, as people with HCM and heart failure may need cardiac resynchronization therapy as well. Severe heart failure may require a left ventricular assist device or heart transplant.

New Treatments Are on the Horizon

Existing treatments for HCM are constantly being studied and refined, but new treatments are rare. A new drug called mavacamten is the first drug of its kind, a cardiac myosin inhibitor designed specifically to treat HCM.

Clinical trials have shown that mavacamten is effective in many people for treating symptoms of HCM. Mavacamten has been shown to improve left ventricular obstruction, exercise tolerance, and heart function.

Studies on mavacamten have shown significant improvement in quality of life, including both physical and social function. Currently, the drug is awaiting approval from the U.S. Food and Drug Administration.

Summary

HCM can be a very complicated disease to treat, requiring care from cardiologists and other physicians who specialize in treating HCM. However, there are many effective treatments available for HCM that can improve day-to-day life and allow people with HCM to live a normal life span.

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