Hypertrophic cardiomyopathy
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Hypertrophic cardiomyopathy (HCM) is a complex form of cardiac disease that damages the muscle of the heart. It may result in hypertrophy or thickening of the heart muscle, stiffness in the left ventricle, mitral valve changes, and cellular modifications. The hypertrophy makes the heart work harder to pump blood.
Most people with HCM can lead regular lives, but it can be serious for some. The thickened heart muscle can cause shortness of breath, chest pain, or alterations in the heart’s electrical system, leading to life-threatening irregular heart rhythms or sudden cardiac death. Although in general, most people with hypertrophic cardiomyopathy have a low risk for sudden cardiac death, it is important to determine if one is among those who have a higher risk.
Symptoms
People suffering from hypertrophic cardiomyopathy may experience a range of symptoms or none. Hypertrophic cardiomyopathy is characterized by the following symptoms:
- Chest pain, most common after exercise or physical activity, although it can also happen during rest or after meals.
- Fainting or syncope, immediately following exertion or activity. Irregular cardiac rhythms or irregular blood vessel responses during exercise might cause fainting, or no cause can be detected.
- Cardiac murmur, which is identified while listening to the heart.
- Palpitations, racing, or fluttering sensation in the chest. Palpitations can be caused by abnormal heartbeats (arrhythmias) such as atrial fibrillation or ventricular tachycardia, both of which generate a fast heartbeat.
- Breathing difficulties and weariness, especially with exertion. The cause is increased pressure in the left atrium and lungs.
- Swelling in the lower body or in the veins of the neck.
Those who have a family history of HCM, or any signs or symptoms of hypertrophic cardiomyopathy should visit a healthcare provider for correct diagnosis and treatment. It may be an urgent medical emergency if one is experiencing fast or irregular heartbeat, chest pain, and trouble breathing. Go to the emergency room immediately.
Causes
Hypertrophic cardiomyopathy can be caused by several factors such as genetics, high blood pressure, and aging. In some cases, the cause is unknown.
Hypertrophic cardiomyopathy can be inherited from the parents and passed down to their children. Gene mutations that cause the heart muscle to thicken usually cause hypertrophic cardiomyopathy. This indicates that something is wrong with a gene that codes for cardiac muscle properties. The form of hypertrophic cardiomyopathy that occurs when a gene abnormality is present varies widely within the family. Some individuals with the hypertrophic cardiomyopathy gene may never develop the condition.
Hypertrophic cardiomyopathy is most common in the septum. The septum is the muscular wall that separates the heart’s left and right sides. When the septum between the heart’s ventricles thickens, problems arise.
Hypertrophic obstructive cardiomyopathy (HOCM) develops when the outflow tract blockage is caused by a narrowing of the septum, which can block or restrict blood flow from the left ventricle to the aorta. To counteract the narrowing or obstruction, the ventricles must pump more forcefully. This makes it difficult for the heart to relax and limits the amount of blood that the ventricle can store and send to the body with each beating.
Hypertrophic cardiomyopathy may cause cardiac muscle cells rearrangement or myofiber disarray. As a result, some people may have arrhythmias.
Risk factors
Hypertrophic cardiomyopathy commonly manifests itself during adolescence, however it can appear at any age. People who have a family history of this disease are at a higher risk. A child has a 50% chance of carrying the genetic mutation if either parent has hypertrophic cardiomyopathy. Early screening for this disease is recommended.
Diagnosis
The diagnosis of hypertrophic cardiomyopathy often involves asking questions about the symptoms, and one’s personal and family’s medical history. A physical exam will be conducted to check the heart and lungs. A cardiac murmur may be heard in people with hypertrophic obstructive cardiomyopathy (HOCM).
- Tests: Several tests may be required to confirm the diagnosis and to rule out other diseases that mimic the symptoms of HCM, such as:
- Echocardiogram: This test indicates how efficiently the heart’s chambers and valves pump blood. It uses sound waves to determine whether the heart muscle is unusually thick. Echocardiography is frequently performed to diagnose hypertrophic cardiomyopathy.
- Heart MRI: This test can show many elements of the heart, including chambers, valves, and muscles and how well they perform, as well as how the blood flows. This scans the heart using radio waves and magnets that generate 2D and 3D images which allow the doctor to make a diagnosis.
- Electrocardiogram (ECG or EKG): An ECG can detect abnormal heartbeats as well as signs of cardiac thickening. It monitors, tracks, and records the electrical activity of the heart using temporary electrodes on the chest and limbs.
- Holter monitor: This is a portable ECG device that can be worn for a day or longer to record the heart’s activity. This can be carried in a pocket or worn on a belt or shoulder strap during regular daily activities.
- Stress test: This test is done while being active. Exercise testing can show how the heart reacts to physical exertion. Normally, the ECG is performed while riding a stationary bicycle or walking on a treadmill, to monitor the activity of the heart.
- Cardiac catheterization: During this procedure, a catheter or a small tube is inserted into a blood artery in the arm or groin and then into the coronary arteries. This invasive imaging technique allows the doctor to assess the function of the heart.
Treatment
The treatment of hypertrophic cardiomyopathy is determined by several factors such as the patient’s symptoms, age, level of activity, degree of arrhythmia, how well the heart is working, and if there is an outflow tract. The goal of the treatment is to lessen or eliminate symptoms and the risk of consequences like heart failure and sudden cardiac death.
- Medications: Medication is frequently prescribed to alleviate the symptoms and avoid potential issues. Medications can assist in lessening the strength with which the heart muscle squeezes and slow the heart rate, allowing the heart to pump blood more efficiently.
These medications include:
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- Beta blockers: such as metoprolol, and calcium channel blockers such as verapamil. These drugs relax the heart muscle, allowing it to fill more efficiently and pump more effectively.
- Heart rhythm medications: such as amiodarone or disopyramide. These drugs may help manage the heart rate or reduce the frequency of arrhythmias.
- Blood thinners: such as warfarin, dabigatran, rivaroxaban or apixaban may be prescribed. These medications may help minimize the risk of sudden cardiac death due to blood clots related to atrial fibrillation or apical hypertrophic cardiomyopathy.
- Surgeries or other procedures: Procedures for treatment of hypertrophic cardiomyopathy include:
- Septal myectomy: Depending on the location of the thickened heart muscle, the procedure can be performed in a variety of ways. One kind, known as apical myectomy, involves the removal of thickened cardiac muscle around the tip of the heart. The mitral valve is sometimes repaired at the same time.
Septal myectomy involves removing a little portion of the thickened septal wall to widen the path the blood takes (outflow tract) from the left ventricle to the aorta. It improves blood flow out of the heart and decreases backward flow through the mitral valve. This is often suggested if medication fails to alleviate symptoms.
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- Septal ablation: This surgery is designated for patients who are unable to undergo a septal myectomy. Complications could include a disturbance of the heart’s electrical system or heart block, which necessitates the insertion of a pacemaker.
During the procedure, a balloon catheter is placed and inflated into the artery. The contrast agent is then injected to find the thicker septal wall that narrows the passageway from the left ventricle to the aorta. When the doctor finds the bulge, he or she will inject a small amount of pure alcohol through the catheter. The alcohol destroys the cells on touch, causing your septum to shrink back to normal size over time. This widens the blood flow route.
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- Implantable cardioverter-defibrillator (ICD): People who are at risk of life-threatening arrhythmias or abrupt cardiac death can benefit from ICDs. The ICD is a tiny device that is inserted just beneath the skin and is linked to wire leads that are threaded through a vein to the heart. If a potentially fatal arrhythmia occurs, the ICD delivers precisely calibrated electrical shocks to restore cardiac rhythm.
Most cases of women with hypertrophic cardiomyopathy can support their pregnancy and give birth vaginally. However, these pregnancies often require specialized treatment and careful monitoring. Women who are considering becoming pregnant may consult a doctor who specializes in caring for women with high-risk pregnancies for proper care. In some cases, hypertrophic cardiomyopathy medications may be prescribed during pregnancy.
