Hypertrophic cardiomyopathy is a genetic disease where thickening of heart muscle often results in obstruction of part of the left ventricle known as the left ventricular outflow tract. The condition often doesn’t cause any symptoms. But when it does, symptoms may include chest discomfort, difficulty breathing, exhaustion and fainting spells.
Having hypertrophic cardiomyopathy, also called HCM, doesn’t have to prevent you from living a full and healthy life.
“The important thing is not to scare people too much,” says Said Alsidawi, M.D., director of the Hypertrophic Cardiomyopathy Clinic for Mayo Clinic in Arizona. “The majority of people who have hypertrophic cardiomyopathy will have a normal life expectancy if they are treated at a comprehensive hypertrophic cardiomyopathy center.”
Being diagnosed with hypertrophic cardiomyopathy likely will mean a treatment plan that can include medications and surgery.
Vague symptoms of hypertrophic cardiomyopathy
Many people with hypertrophic cardiomyopathy don’t realize they have it. That’s because they have few, if any, symptoms. But in a small number of people with HCM, the thickened heart muscle can cause serious symptoms. These include shortness of breath and chest pain. Some people with the condition have changes in the heart’s electrical system. These changes can result in life-threatening irregular heartbeats or sudden death.
“When there are symptoms, they’re usually quite vague. So they’re like any other heart disease. Usually they present with chest pain, shortness of breath, fatigue, lightheadedness,” Dr. Alsidawi says. “It’s usually nonspecific things. We always tell people, if you’re having any of these symptoms, make sure you check with a healthcare professional. Especially when these symptoms happen with exercise.”
Treatments for hypertrophic cardiomyopathy aim to ease symptoms and to prevent sudden cardiac death, although it’s uncommon.
Medications come first, including a recently approved drug
Medications often are the first treatment option considered for people with hypertrophic cardiomyopathy. The process usually starts with a look at medications you’re already taking. Sometimes, drugs for other conditions can make hypertrophic cardiomyopathy worse, and your healthcare team likely will consider stopping or adjusting these in some way. Once your care team has addressed any medicines that could be making things worse, they can consider medications that help treat hypertrophic cardiomyopathy.
Medications for hypertrophic cardiomyopathy may be used to reduce how strongly the heart muscle squeezes. This can help to slow your heart rate, allowing your heart to pump blood better and easier. There are a variety of medications that your care team may prescribe:
- Beta blockers such as metoprolol (Lopressor, Toprol-XL), propranolol (Inderal LA, Innopran XL) or atenolol (Tenormin). These are the first treatments used in most people.
- Calcium channel blockers such as verapamil (Verelan) or diltiazem (Cardizem, Tiazac, others). These generally are used for people who don’t get adequate symptom relief from beta blockers.
- Cardiac myosin inhibitors such as mavacamten (Camzyos). This drug is a relatively recent addition to the list of options, gaining U.S. Food and Drug Administration approval in 2022. It’s a medicine that reduces strain on the heart. It can be used to treat obstructive hypertrophic cardiomyopathy in adults with symptoms. Mavacamten may be recommended if you can’t take beta blockers or calcium channel blockers, or your symptoms don’t improve with either of those treatments. “It’s extremely effective,” Dr. Alsidawi says of mavacamten, while noting that, due in part to its newness, it’s only prescribed at certain medical centers, such as Mayo Clinic.
In addition to these main treatment options, heart rhythm medications such as amiodarone (Pacerone) or disopyramide (Norpace) are used to treat atrial or ventricular arrhythmias. People who have atrial fibrillation or the atypical type of hypertrophic cardiomyopathy can develop blood clots that can result in serious issues such as stroke. Warfarin (Jantoven), dabigatran (Pradaxa), rivaroxaban (Xarelto) or apixaban (Eliquis) are drugs that may be used to help prevent development of harmful blood clots.
Surgical approaches to hypertrophic cardiomyopathy
There are a variety of surgical procedures to treat hypertrophic cardiomyopathy or its symptoms. Typically, surgery is reserved for people whose symptoms don’t improve with medications or when medications cause severe side effects. Surgical options include:
- Septal myectomy. This procedure involves removing part of the thickened wall between the heart chambers. This wall is known as the septum. Septal myectomy can help improve blood flow out of the heart and may reduce the backward flow of blood through the mitral valve. The mitral valve divides the left atrium from the left ventricle. The surgery can be performed using different approaches depending on the location of the thickened heart muscle. In one procedure, known as an apical myectomy, surgeons remove thickened heart muscle from near the tip of the heart. The mitral valve also may be repaired, if damaged. These procedures are best performed at highly specialized centers, such as Mayo Clinic, that perform high volumes of the procedure and typically have better outcomes.
- Septal ablation. This procedure uses alcohol to shrink the thickened heart muscle. A long, thin tube known as a catheter is placed into an artery that supplies blood to the affected area. Alcohol flows through the tube and into the thickened tissues. There may be complications, such as changes in the heart’s electrical signaling system. Sometimes, a pacemaker may need to be placed in the chest to help control the heartbeat.
- Implanting a cardioverter-defibrillator, also called ICD. This device is placed under the skin, near the collarbone, with wires that touch the heart. The device continually checks your heart rhythm and, if it detects an irregular heartbeat originating from the lower chamber of the heart, sends out shocks to reset the heart’s rhythm. Using an ICD has been found to help prevent sudden cardiac death, which occurs in a small number of people with hypertrophic cardiomyopathy.
- Implanting a cardiac resynchronization therapy device. While not a common treatment for hypertrophic cardiomyopathy, this implanted device may be used to help optimize electrical signals in the chambers of the heart so that the heart pumps in a way that’s more efficient.
- Implanting a ventricular assist device, also called VAD. This device, while rarely used to treat hypertrophic cardiomyopathy, may be used to help blood flow through the heart. This is usually done in end-stage disease when all other options fail.
- Heart transplant. This surgery replaces a diseased heart with a donor’s healthy heart. It can be a treatment option for end-stage heart failure when medicines and other treatments no longer work.
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