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What Is Hypertrophic Cardiomyopathy (HCM)?

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What Is Hypertrophic Cardiomyopathy (HCM)?

Hypertrophic cardiomyopathy (HCM) is a genetic disease that causes the heart muscle to thicken. HCM, which often develops at a young age, can make it more difficult for the heart to pump blood, potentially leading to symptoms such as chest pain, dizziness, or fainting.e60dc2a1-f33c-4a05-9b50-8e3e8e597629d86366dc-9b28-4e88-995b-0135e70501d3 The symptoms of HCM can be very mild, and many people go undiagnosed because they have no symptoms at all. Nevertheless, the condition requires medical treatment — including drugs, surgery, or implantable medical devices — to help preserve long-term heart health. HCM is a lifelong condition with no cure.e60dc2a1-f33c-4a05-9b50-8e3e8e597629431f5f8e-7e2e-4ec9-b630-018f104c32b5 In rare cases, HCM can cause early death, sometimes by sudden cardiac arrest.e60dc2a1-f33c-4a05-9b50-8e3e8e597629ee778e2d-ede0-4ced-8d42-35129eac6837
Types of Hypertrophic Cardiomyopathy (HCM) Types There are two main types of hypertrophic cardiomyopathy. Both can impact how the heart pumps blood throughout the body. Obstructive Two-thirds of people who have HCM have obstructive HCM. This occurs when the wall (septum) between the heart’s bottom two ventricles thickens and stiffens, blocking or seriously reducing blood flow out of the left ventricle to the body. Nonobstructive: In about one-third of people with the disease, HCM is nonobstructive, meaning the heart muscle becomes thick and stiff but doesn’t block blood flow out of the heart. But the amount of blood the heart can pump into the body may be reduced. Other, rarer forms of the disease also exist, though for most the treatment does not differ. One rare type known as apical hypertrophic cardiomyopathy may increase the risk of cardiovascular diseases, such as heart failure.e60dc2a1-f33c-4a05-9b50-8e3e8e5976293475e5ab-f923-4e2f-8c85-806e43d9af15
Signs and Symptoms of Hypertrophic Cardiomyopathy (HCM) Symptoms Many people living with hypertrophic cardiomyopathy don’t experience any symptoms. Some will never learn that they have the condition, and will never experience any obvious downsides. Others, however, will experience symptoms, including:e60dc2a1-f33c-4a05-9b50-8e3e8e597629af00c01b-b0a9-464f-b108-9f676594ea70 Chest pain Shortness of breath Abnormal heart rhythms ( arrhythmia ) or palpitations Rapid heart beat Fatigue or lack of energy Dizziness or lightheadedness Fainting Heart murmur Swelling in the ankles, feet, legs or neck veins Some may only experience these symptoms when they’re exercising or physically exerting themselves. These symptoms are not unique to HCM, and can be caused by many other cardiovascular conditions, some of which are more dangerous than HCM, such as heart failure and atrial fibrillation . People experiencing any of these symptoms should see a healthcare provider for evaluation. It is also common for people with HCM to be misdiagnosed with non-cardiac ailments such as asthma, anxiety, or depression.e60dc2a1-f33c-4a05-9b50-8e3e8e597629f5b69d31-8397-4fc3-9b50-e4952aa9f11a
Causes and Risk Factors of Hypertrophic Cardiomyopathy (HCM) Causes Hypertrophic cardiomyopathy often runs in families, and a parent with a gene that causes HCM is about 50 percent likely to pass the gene on to a child. Not everyone with the gene will develop HCM, and some people may carry the gene without developing the condition themselves.e60dc2a1-f33c-4a05-9b50-8e3e8e597629af32c3ac-2378-471f-8066-b8a3bef33e5e Not everyone with HCM has a family history of the condition. Nonfamilial hypertrophic cardiomyopathy often develops later in life, and can have milder symptoms. It’s not known why these nonfamilial cases occur.e60dc2a1-f33c-4a05-9b50-8e3e8e597629f0a83fe9-cae6-4e10-943e-13e155bd7cf1
How Is Hypertrophic Cardiomyopathy Diagnosed? Diagnosis If you are experiencing symptoms of heart disease, a provider may start with a physical exam, listening to your heart for any sign of a heart murmur. If the provider hears the whooshing sound that could indicate the heart is having trouble pumping blood normally, they will likely move on to diagnostic tests. Identifying HCM can require a number of tests, but typically begins with an echocardiogram , or “echo.” The noninvasive test uses sound waves to create images of the heart that can show how thick the heart muscle is. Other options to help confirm an HCM diagnosis include: Electrocardiogram (ECG) , a test that checks the heart’s electrical signals and rhythm A Holter monitor, an ECG that is worn for a day or more Magnetic resonance imaging (MRI), another way for doctors to see the heart Stress tests, which assess the heart’s performance during exercise A family history analysis HCM is often subtle and doesn’t have any signature symptoms, so it can easily be missed or misdiagnosed. Sometimes the condition is noticed during a routine checkup.e60dc2a1-f33c-4a05-9b50-8e3e8e597629b3a61df8-390e-475e-9fad-1f781e69bef0 The condition can be diagnosed at any age, but is most commonly detected in middle age.e60dc2a1-f33c-4a05-9b50-8e3e8e5976293f69a87d-6eb2-4061-98e1-d76ba55f33b2
Treatment and Medication Options for Hypertrophic Cardiomyopathy (HCM) Treatment Cardiologists have a number of options to treat HCM, including medications, surgery, and implantable devices. These treatments are designed to reduce the long-term risk of cardiovascular disease and to alleviate symptoms and improve quality of life. What treatment is right for you depends on what type of HCM you have, how bad your symptoms are, and if you have any related conditions, such as hypertension, type 2 diabetes , or arrhythmias.e60dc2a1-f33c-4a05-9b50-8e3e8e597629aed75bbe-cb38-49e7-a885-aea49e7ad5fb Medication Options A number of medications may be used to help reduce the symptoms of HCM and reduce the risk of long-term health problems, including:e60dc2a1-f33c-4a05-9b50-8e3e8e59762995508406-dd82-4bd7-bf48-038420fc6765 Beta-blockers to lower blood pressure and heart rate Calcium channel blockers to help blood vessels relax Diuretics to help the body remove excess water and sodium If you have complications of the disease, such as arrhythmia, you may need medications to treat those conditions specifically. These may include: Antiarrhythmic drugs to treat abnormal heart rhythms, such as amiodarone (Pacerone) or disopyramide (Norpace) Blood thinners , which can prevent blood clots in people with HCM and atrial fibrillation There is currently just one medication approved to specifically treat HCM. Mavacamten (Camzyos) can treat obstructive HCM in people who have mild to moderate symptoms when they exercise. This drug can reduce symptoms and increase exercise capacity, but it is only available through a restricted program because it can create a risk of heart failure if not used properly.e60dc2a1-f33c-4a05-9b50-8e3e8e59762987391f5b-c50f-4cde-95f9-8a3180884638e60dc2a1-f33c-4a05-9b50-8e3e8e597629000144ba-64cc-42ae-a84a-cd1094aa2c0d Surgery Options Some people may need surgery to treat HCM: Septal Myectomy is an open-heart surgery that removes parts of thickened heart tissue between the walls of the heart chambers. This surgery is considered safe and its benefits are long-lasting.e60dc2a1-f33c-4a05-9b50-8e3e8e59762957b9c05b-9b9c-4799-8243-45175199ce02 Septal Ablation is a less-invasive treatment in which a doctor guides a catheter through a blood vessel and into the heart, spraying alcohol onto thickened heart tissue. The alcohol causes some of the muscle to shrink and die, improving blood flow.e60dc2a1-f33c-4a05-9b50-8e3e8e597629bb063f1f-f55a-49ca-a1ca-db5a6e7a79b5 A variety of other surgeries may be appropriate, especially for people who experience other heart conditions in addition to HCM. Implanted Devices You may need an implanted device to help regulate your heart rhythms:e60dc2a1-f33c-4a05-9b50-8e3e8e597629f0b95a7d-a479-4b9b-8b5d-226e5cf67185 An implantable cardioverter-defibrillator (ICD) is a device implanted in the chest or near the armpit that detects potentially fatal arrhythmias. In the event of a disrupted heart rhythm, the device sends an electrical shock to the heart, causing it to beat regularly again. A pacemaker is a battery-operated device implanted near the collarbone that uses electrical signals to regulate your heartbeat.
Prevention of Hypertrophic Cardiomyopathy (HCM) Prevention There is no known way to prevent HCM. Experts recommend children with a family history of HCM to begin screening with an echocardiogram starting around age 12. Screening should be done every one to three years until age 21; after that, screenings can be done every five years.e60dc2a1-f33c-4a05-9b50-8e3e8e597629d33071c9-3ffb-4f7d-b298-f8b68305e587 Visit a cardiologist regularly, even if you don’t have any HCM symptoms. This is the best way to preserve your long-term heart health.
Lifestyle Changes for Hypertrophic Cardiomyopathy Lifestyle Changes People with HCM benefit from the same healthy lifestyle habits as people with other forms of heart disease, such as maintaining a healthy weight, staying active, eating a heart healthy diet , getting good quality sleep , and not smoking.e60dc2a1-f33c-4a05-9b50-8e3e8e597629274a31b4-03db-45c8-ac92-9222bf1ffcb9 People with HCM may be cautioned against certain types of exercise. Though physical activity remains important for good health, your doctor may warn you against athletic exertion that requires straining or short bursts of activity. In very rare cases, HCM can lead to sudden cardiac death during exercise. These deaths often occur in young athletes who had no awareness of their condition.e60dc2a1-f33c-4a05-9b50-8e3e8e5976294dd8818e-5b14-49a8-b779-8498dee8d6c3 Your doctor may also recommend other behaviors to help reduce the strain on your heart, including:e60dc2a1-f33c-4a05-9b50-8e3e8e59762926d40398-b9b2-4bcc-9cee-bfcf6a9a6807 Staying well hydrated Avoiding extreme cold or heat (including saunas and hot tubs) Reducing stress
Hypertrophic Cardiomyopathy (HCM) Prognosis Prognosis and Outlook Hypertrophic cardiomyopathy is a chronic, life-long condition. Many people living with the disease don’t know they have it and live normal lives. HCM does not necessarily have an effect on life expectancy, even when it requires drugs, an implanted device, or surgery. The condition can, however, increase the risk of early death and outcomes such as heart failure and stroke, especially when a person has more than one cardiovascular condition.e60dc2a1-f33c-4a05-9b50-8e3e8e59762992eea9f9-f35d-45a8-af93-b3809456c50e It’s important to monitor your heart health regularly, even if you lack any HCM symptoms.
Complications of Hypertrophic Cardiomyopathy (HCM) Complications Though HCM can be benign on its own, it often occurs with other cardiovascular conditions. People with HCM may be at higher risk for other types of cardiovascular disease, including stroke, heart failure, and atrial fibrillation, which can lead to blood clots.e60dc2a1-f33c-4a05-9b50-8e3e8e597629f3f8e7ce-f926-4842-958f-9305819b72bc About half of people who have HCM also have high blood pressure , or hypertension. This can be particularly challenging because the medications used to treat hypertension may worsen obstructive HCM. It’s important for people who have both high blood pressure and HCM to follow nonmedication lifestyle treatments for hypertension .e60dc2a1-f33c-4a05-9b50-8e3e8e597629af41889c-d2c0-46bb-95a2-aaa9083dabef Women with HCM who become pregnant may experience more serious symptoms during pregnancy, including an enhanced risk of cardiac events such as heart failure and arrhythmia. These issues increase the chance that a delivery by cesarean section will become necessary, but new symptoms and complications usually improve after pregnancy.e60dc2a1-f33c-4a05-9b50-8e3e8e5976298b1443af-286a-4bd0-8380-4cc23cf80e19e60dc2a1-f33c-4a05-9b50-8e3e8e5976296e4708db-1bbe-4fd2-909d-aefb4b187507 A very small fraction of people with HCM, mostly young people, are at risk for sudden death caused by fatal arrhythmias. If detected during a diagnosis or follow-up appointment, arrhythmias can be treated with an implantable cardioverter defibrillator (ICD).e60dc2a1-f33c-4a05-9b50-8e3e8e59762992c88827-9210-4b11-acad-83672e5dbedd
Research and Statistics: How Many People Have Hypertrophic Cardiomyopathy (HCM)? Research and Statistics It’s difficult to know how common HCM is, because a substantial number of people with the condition are never diagnosed. Estimates suggest that about 1 of every 500 people has HCM, for a total of about 700,000 Americans and 15 million people worldwide with the disease.e60dc2a1-f33c-4a05-9b50-8e3e8e597629a114e690-3d9b-4e6a-8e80-e5622e53cf46
Disparities and Inequities in Hypertrophic Cardiomyopathy Disparities Data on racial disparities in HCM are sparse, but some studies have suggested that people from different racial and ethnic backgrounds have different levels of risk due to the condition: Black Americans with HCM may have more severe symptoms and outcomes than white Americans, although it is difficult to know if the difference is due to genetic factors or to inequities in healthcare access and delivery.e60dc2a1-f33c-4a05-9b50-8e3e8e59762966790135-b2fd-46db-adac-6be43181a54f People of Hispanic or Latino descent with HCM may have an enhanced risk of serious heart failure.e60dc2a1-f33c-4a05-9b50-8e3e8e5976298b3760de-5eaa-417c-b9c0-832101bb8f81
Support for Hypertrophic Cardiomyopathy (HCM) Support Hypertrophic Cardiomyopathy Association The Hypertrophic Cardiomyopathy Association has online patient discussion groups that can make it easy for people living with HCM to connect with others who have the disease and learn more about it. The organization also sponsors volunteer opportunities and advocacy events. The Takeaway Hypertrophic cardiomyopathy (HCM) affects about 1 in 500 people, and many living with the disease do not know they have it. The symptoms of HCM (including shortness of breath, chest pain, and dizziness) overlap with many other conditions. Getting screened is important, especially for people with a family history of the condition. HCM is extremely treatable, and though some will require drugs, surgery, or an implantable device, many people with the condition do not experience a significant decline in life expectancy or quality of life.
Resources We Trust Cleveland Clinic: CardiomyopathyBritish Heart Foundation: 10 Signs You Might Have Heart DiseaseStanford University: Athlete’s HeartNational Heart, Lung, and Blood Institute: What to Expect During Heart SurgeryTemple Health: Frequently Asked Questions About Implantable Heart Devices

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