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Hypertrophic Cardiomyopathy Treatment in Delhi, India
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Hypertrophic cardiomyopathy (HCM) is characterised by the thickening of the heart muscle, commonly at the septum between the ventricles and below the aortic valve.
The thickening can make it harder for the blood to exit the heart, forcing the heart to work harder to pump blood. It may make it difficult for the heart to relax and fill with blood. This may lead to stiffening of the heart walls and abnormal aortic and mitral heart valve function, both of which may impede normal blood flow out of the heart.
Thickening of the heart muscle (myocardium) usually occurs at the septum. The septum is a wall of muscles separating the left and right sides of the heart. When the septum thickens between the heart's lower chambers or ventricles, problems arise. The thickened septum causes the vessels to narrow, blocking or reducing the blood flow from the left ventricle to the aorta. This is called outflow tract obstruction. The ventricles need to pump harder to overcome the narrowing or blockage of the vessels. This type of hypertrophic cardiomyopathy is called hypertrophic obstructive cardiomyopathy (HOCM).
HCM also causes thickening in other parts of the heart muscle, such as the bottom of the heart, called the apex, the right ventricle, or the entire left ventricle.
Hypertrophic Cardiomyopathy Symptoms
Some people with hypertrophic cardiomyopathy may be asymptomatic. At the same time, others may not exhibit symptoms in the early stages but may eventually develop them. Signs and symptoms of HCM include:
- Chest pain, especially with physical exertion
- Shortness of breath, especially with physical exertion
- Fatigue
- A sensation of rapid, fluttering or pounding heartbeats (palpitations)
- Dizziness
- Fainting
- Heart murmur
- Swelling in the ankles, feet, legs, abdomen, and veins of the neck
- Arrhythmias (abnormal heart rhythms)
- Light-headedness
Hypertrophic Cardiomyopathy Causes
- Genetic influence
HCM is typically an inherited condition. Defective genes can cause the heart muscle to thicken. Inheriting the gene doesn't necessarily mean that the disease will manifest. HCM follows a dominant pattern of inheritance. However, symptoms don't always develop in people with a defective gene.
- Other Causes
Other possible causes of HCM include:
- Ageing
- High blood pressure
Hypertrophic Cardiomyopathy Diagnosis
No blood tests are required to diagnose HCM. Genetic testing is not available widely but is slowly gaining popularity. 2-D echocardiography is a commonly advised diagnostic test for HCM. Other tests that may be recommended are:
- Chest radiography
- Cardiac magnetic resonance imaging: It is useful when echocardiography is questionable, particularly with apical hypertrophy
- Radionuclide imaging
- Cardiac catheterisation determines the degree of cardiac haemodynamic, outflow obstruction, anatomy and diastolic characteristics of the left ventricle and the heart.
Hypertrophic Cardiomyopathy Treatment
Currently, there are no disease-specific medications for treating hypertrophic cardiomyopathy. For asymptomatic patients, making lifestyle changes and taking medications for conditions that may contribute to cardiovascular disease are recommended. The aim is symptom management using medications and procedures for those with symptoms.
Medications
Medications called calcium channel blockers, beta-blockers, and diuretics provide limited relief of symptoms.
Procedures
Different surgical and nonsurgical procedures that can be employed to treat HCM are:
- Septal myectomy - Septal myectomy is an open-heart surgery attempted in patients with obstructive hypertrophic cardiomyopathy and severe symptoms. This surgery is usually performed in younger patients and people who do not respond to medications. The surgeon incises a part of the thickened septum bulging into the left ventricle, which improves blood flow to the heart and body.
- Alcohol septal ablation (nonsurgical procedure) - Ethanol is injected through a tube into the artery that supplies blood to the part of the heart muscle thickened by HCM. The alcohol kills the cells, and the thickened tissue shrinks to normal size. The complications of heart surgery increase with age; hence, an ablation may be preferred to myectomy in older patients with pre-existing medical conditions.
- Surgically implanted devices - Surgeons may implant numerous types of devices to help the heart work better, including:
- Implantable cardioverter-defibrillators (ICDs)- This helps maintain a normal heartbeat by producing an electric shock to the heart when it detects an irregular heartbeat. This lowers the risk of sudden cardiac death.
- Pacemakers- These are small devices that use electrical pulses to prompt the heart to beat normally.
- Heart Transplant- A heart transplant may be considered in HCM patients with advanced, end-stage disease. In this procedure, the diseased heart can be transplanted with a healthy donor heart.
Hypertrophic Cardiomyopathy Complications
Most hypertrophic cardiomyopathy (HCM) patients do not present significant health problems. But some complications of hypertrophic cardiomyopathy can be:
- Atrial fibrillation: The thickened muscles of the heart and the abnormal structure of heart cells can lead to changes in the electrical system of the heart. This can result in rapid or irregular heartbeats. Atrial fibrillation carries a risk of developing blood clots, leading to strokes.
- Left ventricle outflow obstruction: In most patients, the thickened heart muscle blocks the blood flow that exits the heart, causing shortness of breath on exertion, pain in the chest, dizziness and fainting.
- Mitral valve problems: If the damaged heart muscle blocks the blood flow that exits the heart, the valve between the left atrium and left ventricle, called the mitral valve, might not close properly. As a result, blood can leak backwards into the left atrium, leading to mitral valve regurgitation, possibly worsening symptoms.
- Dilated cardiomyopathy: The thickened heart muscle may become weak and ineffective in a few people with hypertrophic cardiomyopathy. The ventricle enlarges or dilates and pumps with lesser force. This is a rare complication.
- Heart failure: The heart muscle can become too stiff with time to effectively fill the heart with blood, resulting in inadequate blood pumping to meet the body's requirements.
- Sudden cardiac death: This is a rare event. It can happen in younger individuals or athletes.
Hypertrophic Cardiomyopathy Risk Factors
Most people with hypertrophic cardiomyopathy are at low risk for sudden cardiac death. People with hypertrophic cardiomyopathy who are at a higher risk for sudden death are:
- Patients with a positive family history of sudden cardiac death
- Young patients who have experienced numerous episodes of syncope (or fainting)
- Patients who experience abnormal blood pressure in response to exercise or heavy workouts.
- Patients with a history of arrhythmia (with a fast heart rate)
- Patients with severe symptoms and poor heart function
Prognosis
Some people with hypertrophic cardiomyopathy may not present with clinical symptoms and may have a normal lifespan. Others may show signs that worsen slowly or rapidly. Patients with hypertrophic cardiomyopathy are at risk for sudden death, even at a young age. Some patients develop dilated cardiomyopathy (Burn Out Phase) with time.
The prognosis of different types of cardiomyopathies is different. When it occurs in older people, the outlook is better due to the increased thickness of the heart muscle. Athletes are more prone to sudden death with hypertrophic cardiomyopathy.
Reviewed & Updated On
Reviewed by Dr. Arun Kumar Gupta, Associate Director, Cardiac Sciences, Cardiology, Cardiac Electrophysiology-Pacemaker, Interventional Cardiology on 29-May-2023.
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