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Written by Michelle Clapham, RN, BSN, MSN, APRN (retired)
What is Hypertrophic Cardiomyopathy?
Hypertrophic cardiomyopathy (HCM) is a heart condition where the heart muscle becomes thickened and stiff. These changes interfere with the normal pumping and emptying of the left ventricle.
Hypertrophic cardiomyopathy is a genetic condition. Approximately 1 in every 500 persons are diagnosed with HCM. There are two types of HCM: obstructive and non-obstructive.
Approximately two-thirds of persons diagnosed with HCM have the obstructive form of HCM while the remaining one-third will be diagnosed with the non-obstructive form of HCM.
In obstructive HCM, the wall (septum) between the two bottom chambers of the heart (right and left ventricle) is thickened. This thickening reduces the blood flow from the heart through the aortic valve to the aorta. In non-obstructive HCM, the heart muscle (myocardium) is thickened but does not impede blood flow from the heart.
What are the symptoms of HCM?
Symptoms of HCM can include:
Dizziness
Lightheadedness
Irregular heart rhythms such as atrial fibrillation
Shortness of breath, especially with activity
Passing out
Swelling, usually in the lower legs and feet
If left untreated, HCM may lead to heart failure which is a condition where the pumping function of the heart is severely impaired. The development of atrial fibrillation increases the risk of the development of blood clots which could lead to strokes or heart attacks.
How is HCM diagnosed?
The diagnosis of HCM is most commonly made after a thorough history and physical examination, review of family history as well as diagnostic testing such as a heart ultrasound (echocardiogram), ECG (electrocardiogram), portable heart monitor (Holter monitor), cardiac stress testing, cardiac MRI, or a heart catheterization. In certain instances, genetic testing may be recommended by your health care provider.
What treatments are available for HCM?
Treatment of HCM includes medications such as beta blockers and calcium channel blockers. If swelling is present in the lower legs and/or feet, a diuretic (water pill) may also be prescribed by your health care provider. If there is a co-diagnosis of heart failure, atrial fibrillation or other heart rhythm disturbances, your health care provider will prescribe medications to address the heart failure.
A newer medication, Mavacamtem (trade name CamzyosTM) has been approved by the FDA for use in the treatment of HCM. Mavacamtem reduces the thickness of the heart muscle and is prescribed in people who have HCM with mild to moderate symptoms of shortness of breath with activity. Because of how Mavacamtem changes the heart muscle, its use requires very close follow-up with your cardiologist.
Lifestyle changes are an important component of the treatment of HCM. Regular physical activity, such as walking, as well as maintaining a healthy weight, eating a healthy diet, reducing stress, getting adequate sleep, and avoiding nicotine/smoking/vaping contribute to a healthier life.
In certain cases, a person with HCM may need additional interventions such as a myectomy. A myectomy is a type of open-heart surgery where a portion of the thickened heart muscle (myocardium) is removed which eliminates the obstruction and improves the function of the heart. In people with serious heart rhythm disturbances, consideration may be given to implanting automatic cardiac devices (defibrillator, pacemaker) in an effort to improve the functioning of the heart.
In conclusion, HCM is a genetic condition that affects the heart muscle’s ability to pump blood to the body. Treatment includes medications, lifestyle changes and, in certain cases, heart surgery or the implantation of automatic cardiac devices help to improve the functioning of the heart. Most individuals diagnosed with HCM lead normal lives with little or no limitations to their day-to-day activities. It is important to maintain regular follow up with your health care provider to monitor your HCM and your overall health.
Sources:
Camzyos: Uses, Dosage, Side Effects & Warnings (drugs.com) Assessed and Endorsed by the MedReport Medical Review Board