Best Hypertrophic Cardiomyopathy Treatment in Delhi, India

Hypertrophic cardiomyopathy (HCM) is a condition in which the heart muscle, especially the walls of the left ventricle, thickens abnormally. This thickening can obstruct blood flow out of the heart, making it harder for the heart to pump blood effectively. HCM is often a genetic condition, passed down through families, and its severity can range from mild to life-threatening. While some individuals with HCM may not exhibit any symptoms, others might experience chest pain, shortness of breath, dizziness, or fainting, particularly during physical activity. The condition can also cause the heart's electrical system to become irregular, leading to arrhythmias, which can increase the risk of sudden cardiac arrest. HCM is the most common cause of sudden death in young athletes.

Hypertrophic cardiomyopathy can be classified into two main types based on whether the thickened heart muscle obstructs blood flow: 

Obstructive Hypertrophic Cardiomyopathy (HOCM) 

In this type, the thickened septum (the wall between the heart’s left and right chambers) bulges into the left ventricle, partially blocking or obstructing the flow of blood out of the heart. This can cause a significant increase in pressure within the heart and lead to symptoms like shortness of breath, giddiness, and chest pain. HOCM is the most common form of HCM and is often the type associated with more noticeable symptoms. 

Non-Obstructive Hypertrophic Cardiomyopathy (NO-HCM) 

In non-obstructive HCM, the heart muscle is thickened, but it does not significantly block the flow of blood out of the heart. While the heart's pumping function may still be affected, the absence of obstruction typically results in fewer or less severe symptoms. However, patients with NO-HCM can still be at risk for complications such as arrhythmias leading to Sudden cardiac Death and heart failure. 

These types help determine the approach to treatment and management, as obstructive and nonobstructive forms of HCM may require different strategies to effectively control symptoms and reduce the risk of complications.

Hypertrophic cardiomyopathy is primarily caused by genetic mutations that affect the proteins in the heart muscle. These mutations lead to abnormal thickening of the heart muscle, particularly in the walls of the left ventricle. The condition is often inherited in an autosomal dominant pattern, meaning that if one parent has the gene mutation, there is a certain percentage of transmission to offspring.

While genetics play a significant role, there are other factors that can contribute to the development or worsening of hypertrophic cardiomyopathy. These include: 

• Family History: Individuals with a family history of hypertrophic cardiomyopathy are at a higher risk of developing the condition. 
• Age: Although hypertrophic cardiomyopathy can develop at any age, symptoms often appear during adolescence or early adulthood. 
• High Blood Pressure: Chronic high blood pressure can exacerbate the thickening of the heart muscle, especially in those with a genetic predisposition to hypertrophic cardiomyopathy. 
• Athletic Activity: Intense athletic activity may trigger or worsen symptoms in individuals predisposed to hypertrophic cardiomyopathy, though the relationship between exercise and the condition is complex. 
• Other Health Conditions: Certain conditions, such as diabetes or thyroid disease, can add upon the morbidity , though they are not direct causes of hypertrophic cardiomyopathy.

The symptoms of hypertrophic cardiomyopathy can vary widely from person to person, and some individuals may not experience any symptoms at all. However, when symptoms do occur, they are often related to the heart's reduced ability to pump blood effectively due to the thickened heart muscle. 

Common symptoms include 

Shortness of Breath (Dyspnoea): Difficulty breathing, especially during physical activity, is one of the most common symptoms. This occurs because the thickened heart muscle makes it harder for the heart to pump blood efficiently, leading to a build-up of fluid in the lungs. 

• Chest Pain (Angina): Chest pain or discomfort may occur, particularly during exertion, due to the heart's increased demand for oxygen that it struggles to meet. 
• Fainting (Syncope): Some people with hypertrophic cardiomyopathy may experience episodes of fainting, especially during or after exercise. This can be due to irregular heart rhythms or a sudden drop in blood pressure. 
• Palpitations: Individuals may feel their heart beating rapidly, irregularly, or forcefully, which can be caused by abnormal heart rhythms (arrhythmias) common in hypertrophic cardiomyopathy. 
• Fatigue: Persistent tiredness and fatigue are common, as the heart's reduced efficiency in pumping blood can lead to a decreased supply of oxygen to the body's tissues. 
• Swelling in the Legs, Ankles, and Feet (Oedema): Fluid retention may occur in the lower extremities due to the heart's inability to pump blood effectively, leading to swelling. 
• Dizziness or Lightheadedness: Dizziness can occur due to reduced blood flow to the brain, often linked to arrhythmias ,blood flow obstruction or in some condition get precipitated by dehydration. 
• Sudden Cardiac Arrest: In rare and severe cases, hypertrophic cardiomyopathy can lead to sudden cardiac arrest, particularly in young athletes during intense physical activity. 

The severity and presence of symptoms can vary, and some individuals may only become aware of their condition through routine screening or genetic testing. It's important to seek medical evaluation if any of these symptoms are experienced, especially if there is a family history of hypertrophic cardiomyopathy.

Physical Examination 

During a physical exam, the provider will listen to the heart with a stethoscope to detect abnormal heart sounds, such as murmurs that indicate turbulent blood flow. They will also check for signs of heart failure, such as swelling in the legs or fluid retention. 

Electrocardiogram (ECG or EKG) 

An electrocardiogram records the electrical activity of the heart through electrodes placed on the skin. It can reveal abnormalities in heart rhythm, evidence of thickened heart muscle, and other electrical conduction issues, which are common in hypertrophic cardiomyopathy. 

Echocardiogram 

This ultrasound test uses sound waves to create images of the heart. It provides detailed information about the heart's structure and function, allowing the physician to visualise the thickening of the heart muscle, assess the size of the heart chambers, and evaluate blood flow. It is the most commonly used test for diagnosing hypertrophic cardiomyopathy. 

Cardiac MRI 

A cardiac MRI uses powerful magnets and radio waves to create detailed images of the heart's anatomy and function. This test can provide information about the extent and location of muscle thickening, assess the heart’s blood supply, and identify any scarring or fibrosis in the heart tissue, offering a more comprehensive view than an echocardiogram. 

Genetic Testing 

If hypertrophic cardiomyopathy is suspected or if there is a strong family history of the condition, genetic testing may be recommended. This test looks for specific mutations in genes associated with hypertrophic cardiomyopathy, which can help confirm the diagnosis and guide family screening and management. 

Exercise Testing 

An exercise stress test involves monitoring the heart's performance during physical activity, typically on a treadmill or stationary bike. It can help identify how the heart responds to increased workload and may reveal symptoms or arrhythmias that occur during exercise. 

Holter Monitor 

A Holter monitor is a portable ECG device worn continuously for 24 to 48 hours. It records the heart's rhythm throughout the day and night, helping to identify any irregular heartbeats or episodes of arrhythmia that may not occur during a standard ECG. 

Consultation with one of the top hypertrophic cardiomyopathy doctors in India ensures one receives valuable insights and expertise in test result interpretation, which in turn helps ensure a thorough comprehension of the condition.

The treatment for hypertrophic cardiomyopathy focuses on managing symptoms, preventing complications, and improving the quality of life. The approach may vary depending on the severity of the condition and the presence of symptoms. Common treatment options include: 

Medications 

• Beta-Blockers: These medications help slow down the heart rate and decrease the force of contraction, making it easier for the heart to pump blood effectively. They are often prescribed to relieve symptoms like chest pain and shortness of breath, and can also help reduce the risk of arrhythmias. 
• Calcium Channel Blockers: Drugs relax the heart muscle and blood vessels, improving blood flow and reducing the heart's workload. They can be particularly helpful for patients who cannot tolerate beta-blockers. 
• Anticoagulants: In cases where patients experience atrial fibrillation (an irregular heartbeat), anticoagulants may be prescribed to reduce the risk of blood clots and stroke. 

Newer therapies 

• Mavacampten- cardiac myosin inhibitor:  Recently approved by FDA , for patients with severe symptoms. Oral pills that need to be taken as per physician. 
• Aficamten: An oral selective cardiac myosin inhibitor, still in early phases. 
• Gene Therapy: certain gene editing may be able to partially restore faulty protein expressions and improve cardiac function. 

Surgical Procedure 

• Surgical Myectomy: This open-heart surgery involves removing a portion of the thickened heart muscle, particularly in the septum, to relieve the obstruction to blood flow. Myectomy can significantly improve symptoms and enhance the patient’s quality of life. It is typically considered for patients with severe symptoms who do not respond to medication. 
• Alcohol Septal Ablation: This less invasive procedure involves injecting alcohol into the small arteries supplying the thickened part of the heart muscle. The alcohol causes localised damage to the muscle, leading to its reduction over time. This option is often considered for patients who are not suitable candidates for surgery and fulfils the necessary criteria for ASA. 

Implantable Devices 

• Pacemaker: In cases where patients experience bradycardia (slow heart rate) or other rhythm disturbances, a pacemaker may be implanted to regulate the heart's electrical signals. This device can help maintain a steady heartbeat and prevent symptoms related to slow heart rates. 
• Implantable Cardioverter-Defibrillator (ICD): For patients at high risk of sudden cardiac arrest due to potentially life-threatening arrhythmias, an ICD may be recommended. The device continuously monitors the heart's rhythm and can deliver shocks to restore normal rhythm if dangerous arrhythmias occur.

Complications of hypertrophic cardiomyopathy can arise due to the structural and functional changes in the heart. These complications may vary in severity and can significantly impact a patient's health. 

Common complications include: 

• Arrhythmias: Hypertrophic cardiomyopathy can lead to various abnormal heart rhythms, including atrial fibrillation, ventricular tachycardia, and ventricular fibrillation. These arrhythmias can cause palpitations, dizziness, and an increased risk of stroke or sudden cardiac arrest. 
• Heart Failure: The thickening of the heart muscle can impede its ability to pump blood effectively, leading to heart failure. Symptoms of heart failure may include shortness of breath, fatigue, swelling in the legs, and fluid retention. 
• Sudden Cardiac Arrest: In some cases, especially in younger individuals and athletes, hypertrophic cardiomyopathy can increase the risk of sudden cardiac arrest. This can occur due to dangerous arrhythmias that disrupt the heart's normal rhythm. 
• Mitral Valve Problems: The thickened heart muscle can affect the function of the mitral valve apparatus , leading to mitral valve regurgitation. This occurs when the valve does not close properly, causing blood to leak backward into the heart. This condition can worsen heart failure symptoms and may require surgical intervention. 
• Endocarditis: Individuals with hypertrophic cardiomyopathy may be at increased risk of endocarditis, an infection of the heart's inner lining. This risk is particularly relevant if there are structural abnormalities in the heart. 
• Stroke: Atrial fibrillation, which can occur in individuals with hypertrophic cardiomyopathy, increases the risk of blood clots forming in the heart. If a clot travels to the brain, it can lead to a stroke. 
• Decreased Exercise Tolerance: Many individuals with hypertrophic cardiomyopathy may experience reduced exercise tolerance due to symptoms like shortness of breath and fatigue, impacting their overall quality of life.

Preventing hypertrophic cardiomyopathy primarily involves managing risk factors and recognising the condition in those at risk. While HCM is often genetic and cannot be entirely prevented, certain measures can help reduce complications and promote heart health. Here are some preventive strategies: 

Get Regular Screening Done 

Individuals with a family history of HCM or unexplained fainting episodes should undergo regular screening, including echocardiograms and electrocardiograms. Early detection can help manage symptoms and reduce the risk of complications. 

Manage Co-morbid Conditions 

Proper management of conditions like high blood pressure, diabetes, and high cholesterol can help protect heart health. Regular check-ups and adherence to treatment plans are essential for controlling these conditions. 

Avoid Intense Physical Activity 

Athletes and individuals with known HCM or a family history of the condition should avoid competitive sports and intense physical exertion, which can increase the risk of serious complications, including arrhythmias and sudden cardiac arrest. 

Maintaining a Healthy Lifestyle 

Adopting a heart-healthy lifestyle can benefit overall cardiovascular health. This includes: 

• Eating a balanced diet rich in fruits, vegetables, whole grains, and lean proteins. 
• Engaging in regular, moderate exercise as recommended by healthcare providers. 
• Avoiding smoking and excessive alcohol consumption. 
• Managing stress through relaxation techniques, mindfulness, or counselling.

1. How does hypertrophic cardiomyopathy affect athletes? 

Hypertrophic cardiomyopathy can pose significant risks for athletes, as intense physical activity may trigger dangerous arrhythmias or sudden cardiac arrest. Many athletes with this condition may be advised to avoid competitive sports or high-intensity exercise. It's essential for athletes to undergo thorough evaluations and discussions with healthcare professionals about their specific risks before participating in physical activities. 

2. What should I do if I experience symptoms of hypertrophic cardiomyopathy? 

If you experience symptoms such as shortness of breath, chest pain, palpitations, or fainting, it’s crucial to seek medical attention promptly. Contact your healthcare provider or visit the nearest emergency hospital for evaluation. Early detection and management can help address potential complications associated with hypertrophic cardiomyopathy. 

3. What are the long-term outcomes for individuals diagnosed with hypertrophic cardiomyopathy? 

The long-term outcomes for individuals with hypertrophic cardiomyopathy vary depending on the severity of the condition, the presence of symptoms, and the effectiveness of management strategies. Many people lead normal lives with appropriate treatment and monitoring. However, some individuals may experience complications such as arrhythmias or heart failure, which require ongoing management. Regular follow-up with healthcare providers is essential for monitoring heart health and adjusting treatment as necessary. 

4. Can hypertrophic cardiomyopathy lead to other heart conditions? 

Yes, hypertrophic cardiomyopathy can lead to several other heart conditions, including arrhythmias, mitral valve regurgitation, and heart failure. The thickening of the heart muscle can disrupt the heart's electrical system, increasing the risk of abnormal heart rhythms. Additionally, the strain on the heart can contribute to heart failure over time if not properly managed. 

5. Is it safe to participate in physical activities if diagnosed with hypertrophic cardiomyopathy? 

The safety of participating in physical activities depends on the individual’s specific condition and the severity of symptoms. Many healthcare providers recommend that individuals with hypertrophic cardiomyopathy avoid competitive sports and intense exercise due to the risk of arrhythmias or sudden cardiac events. It's essential to consult with a healthcare professional to determine a safe level of physical activity based on personal health status. 

6. What should I know about travelling with hypertrophic cardiomyopathy? 

When travelling with hypertrophic cardiomyopathy, it’s important to take certain precautions. Carry any necessary medications and ensure you have access to medical care at your destination. Inform travel companions about your condition and emergency procedures. It may also be wise to consult with your healthcare provider before travelling, especially if you are considering long trips or activities that may strain your heart. 

7. Can hypertrophic cardiomyopathy be misdiagnosed, and what should I do if I feel my diagnosis is incorrect? 

Yes, hypertrophic cardiomyopathy can sometimes be misdiagnosed due to overlapping symptoms with other conditions. If you feel your diagnosis is incorrect, it's important to seek a second opinion from a healthcare professional, preferably a cardiologist with experience in heart muscle disorders. Request further testing, such as an echocardiogram or cardiac MRI, to obtain a clearer understanding of your heart's condition. Your health and peace of mind are crucial, so don’t hesitate to advocate for yourself.

Reviewed By Dr Sumit Kumar - Associate Consultant – Intervention Cardiology on 01 Nov 2024.

• Rise in cases of asymptomatic heart attacks amongst middle aged people
• ART in women with cardiac condition
• Is Heart Disease More Prevalent in Young People?