Hypertrophic Cardiomyopathy

Among different heart diseases, hypertrophic cardiomyopathy (HCM) is a complex, genetic disorder. It is also referred to as idiopathic hypertrophic subaortic stenosis (IHSS), hypertrophic obstructive cardiomyopathy (HOCM) or asymmetric septal hypertrophy (ASH). Cardiomyopathy is a heart muscle disorder. There are four major types of cardiomyopathy such as dilated, restrictive, arrhythmogenic right ventricular and hypertrophic cardiomyopathy. In hypertrophic cardiomyopathy, there is abnormal thickening of heart muscle (myocardium); that means, the muscle becomes hypertrophied, making it difficult for the heart to pump blood. Hypertrophic cardiomyopathy also causes stiffness in the left ventricular as well as abnormal changes in the muscle cells and mitral valve. The electrical system of the heart is also damaged by this disease. This can increase the risk of abnormal heartbeats (arrhythmias) and sudden death, in rare cases. HCM causes symptoms like chest pain, shortness of breath, dizziness etc. Read more about the symptoms of HCM.

What are the signs and symptoms of hypertrophic cardiomyopathy?

People with mild muscle thickening may not experience any symptoms. In some cases, HCM progresses and affects the heart function. The severity of symptoms may vary from individual to individual. Some of the possible signs and symptoms of HCM are shortness of breath with exertion, chest pain (angina) that occurs with physical activity or exercise, fatigue, syncope or fainting caused by irregular heart rhythms, dizziness, lightheadedness and palpitations due to arrhythmias. It can also lead to high blood pressure (hypertension), heart attack and/or sudden cardiac death. HCM is one of the leading causes of death in young athletes.

What are the causes of hypertrophic cardiomyopathy?

Hypertrophic cardiomyopathy is a rare disease that affects 1 in 500 people. Since it is an inherited disorder, it is primarily caused by genetic mutations. Abnormality in the gene stimulates proteins, called sarcometric contractile proteins, leading to abnormal growth of the heart muscle fibers. A person with a defective gene or family history of HCM is at a higher risk of developing this disease. However, a few people with defective HCM gene don't develop this disease. In some cases, HCM may result from aging or high blood pressure.

How is hypertension cardiomyopathy diagnosed?

The diagnosis of HCM is done on the basis of physical examinations and various investigations. The doctor first reviews the family and medical history of the patient. In the physical examination, the doctor checks for abnormal heart sounds or murmur using a stethoscope. Different tests are performed to diagnose the abnormal thickening of the heart muscles, mitral valve regurgitation or problems with blood flow. Some of the common tests used for the diagnosis of HCM are 24-hour Holter monitor, ECG, Chest X-ray, Cardiac catheterization, Cardiac MRI scan, Echocardiography with Doppler ultrasound and Transesophageal echocardiogram (TEE). Genetic tests are performed to detect abnormal gene(s).

How is hypertension cardiomyopathy treated?

There is no cure for HCM. The treatment is aimed at relieving symptoms and preventing further complications. Some people require hospitalization, until the condition is stabilized. Various treatment options include medications, specialized pacemakers and at times even surgery. Drugs like calcium channel blockers, beta blockers or anti-arrhythmic medications are prescribed, which relax the heart muscles and improve the functioning of the heart.

Pacemaker is inserted under the skin, which sends electrical signals to the heart in order to monitor and regulate the heartbeats. Implantable cardioverter defibrillator (ICD) is implanted in the chest to monitor the heartbeats. It also helps restore a normal heart rhythm by delivering small electrical shocks.

Septal myectomy is an open-heart surgery, in which an abnormally grown, thickened heart muscle wall is removed. This improves blood flow and minimizes mitral regurgitation. Septal ablation is the latest treatment option, in which alcohol is injected through a catheter to destroy a small part of thickened muscle.

Hypertension cardiomyopathy is a life-threatening heart disease; if diagnosed earlier, you can immediately seek appropriate medical treatment in order to prevent its dangerous complications. Also, try to avoid strenuous exercises and overexertion to maintain heart health.