What Causes Hypertrophic Cardiomyopathy?

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease, affecting at least one out of 200 people. HCM affects all age groups, from newborns to the elderly. It is a genetic condition that causes heart muscle tissue to become abnormally thick.

• When the heart muscle grows thick, its chambers get narrow and thus, accommodate less blood.
• The thickening can make it harder for blood to leave the heart, forcing the heart to work harder to pump the blood. It also can make it harder for the heart to relax and fill with blood.
• Usually, in hypertrophic cardiomyopathy, the muscle of the heart’s left ventricle is thicker than normal, or the wall between the two ventricles (septum) becomes enlarged and blocks the blood flow from the left ventricle.
• Hypertrophic cardiomyopathy may cause an arrhythmia, a disturbance in the heart’s rate or rhythm. In addition, people with hypertrophic cardiomyopathy are susceptible to endocarditis, an infection of the lining of the heart.

Many people do not experience any symptoms until the condition is advanced. People may not have any symptoms prior to sudden death. All the below symptoms may become more prominent after eating, especially after a large meal

• Shortness of breath
• Chest pain
• Dizziness
• Lightheadedness or fainting
• Palpitations (awareness of one’s heartbeat)
• Lack of energy
• Swelling of legs and feet (the sign of failing heart)
• Syncope (fainting)
• Abnormal heart rhythm (atrial fibrillation)

Hypertrophic cardiomyopathy is usually treated with:

Medications:

• Beta-blockers lower blood pressure and heart rate, reducing the heart workload. These are metoprolol (Lopressor, Toprol-XL), propranolol (Inderal, Innopran XL), or atenolol (Tenormin).
• Calcium channel blockers, verapamil (Verelan, Calan SR,) or diltiazem (Cardizem, Tiazac), which dilate (open) the blood vessels by increasing blood flow to the heart.
• Antiarrhythmic drugs, which help to correct an abnormal heart rate or rhythm. These include amiodarone (Pacerone) or disopyramide (Norpace).
• Diuretics, which reduce fluid retention, is a common symptom of heart failure.
• Anticoagulants, which inhibit blood clot formation by preventing the clumping of platelets in the blood, e.g., warfarin (Coumadin, Jantoven), dabigatran (Pradaxa), rivaroxaban (Xarelto), or apixaban (Eliquis).

Procedures:

• Alcohol septal ablation: Also known as septal alcohol ablation. It is a minimally invasive procedure that shrinks abnormal tissue and improves blood flow throughout the body.
• Implantable cardioverter-defibrillator (ICD): It is a device implanted below the collarbone that monitors the heart’s rhythm. When it detects an abnormal rhythm, it delivers an electrical impulse or shock to the heart to correct it.
• Septal myectomy: It is a surgical procedure to remove overgrown heart muscle and improve blood flow through the heart.

Lifestyle modifications:

• Do not smoke.
• Eat heart-healthy foods, such as fruits, vegetables, whole grains, fish, lean meats, and low-fat or non-fat dairy foods. Limit sodium, sugars, and alcohol.
• Drink plenty of fluids (unless the doctor has told to limit your fluid intake).
• Be active with minimal exercises
• Stay at a healthy weight. Lose weight if you need to.
• Manage other health conditions, such as high blood pressure and high cholesterol.
• Get checkups as often as per the doctor’s recommendation.

Ongoing research offers a more optimistic outlook; it’s now increasingly being viewed as a treatable disease that can allow patients to enjoy a normal life expectancy and good quality of life. However, people with hypertrophic cardiomyopathy are at higher risk of sudden death, especially at a young age. If you have a first-degree relative, a parent, sibling, or child, with hypertrophic cardiomyopathy, genetic testing or regular ECGs may be required. Hypertrophic obstructive cardiomyopathy occurs in men and women with equal distribution across all races. Females tend to be more symptomatic causing more disability and present at a younger age some people with hypertrophic cardiomyopathy may not have symptoms and may have a normal lifespan. Others may get worse slowly or quickly. Hypertrophic cardiomyopathy is a well-known cause of sudden death in athletes. Almost half of the deaths due to this condition happen during or just after some type of physical activity. The condition is progressive and eventually, all patients will develop symptoms.