Hypertrophic Cardiomyopathy Services

In addition to a personal and family history and conducting a comprehensive physical examination, there are several noninvasive tests that may be performed to confirm your HCM diagnosis:

• Electrocardiogram (ECG): This is a common, painless, and quick test that assesses the electrical activity of the heart.
• Echocardiogram (Echo): This is the standard test for diagnosing HCM. It is an ultrasound test that takes pictures of the thickened heart muscle and valve function in real-time.
• Cardiac MRI (Magnetic Resolution Imaging): This is a safe and common test that uses radio waves and a strong magnetic field (rather than x-rays) to diagnose and assess a variety of medical conditions. We use an MRI as part of our initial assessment to define the anatomy of the heart. It provides remarkably clear visualization of the heart muscle walls so we can precisely measure wall thickness and find the exaction location where the muscle is the thickest.
• Exercise (Stress) Echocardiogram: This test is performed only when a resting echocardiogram shows the heart muscle is thickened but blood still flows normally. Patients walk on a treadmill to determine if blood flow becomes obstructed when the heart rate increases. Our team reviews both sets of heart images side-by-side to compare the way the heart looks at rest versus during exercise.
• Ambulatory ECG Monitoring: The ZIO patch is a small, peel-and-stick, wireless, unobtrusive, and water-resistant device. A patient wears the patch for 2-4 weeks so we can track any irregular or abnormal heart rhythms that would represent possible risk markers for sudden cardiac arrest.
• Cardiopulmonary Exercise Test (CPET): This test is performed on a treadmill to measure exercise limitation and the ability to adapt to the energy demands of exercise.

Since HCM is often genetically transmitted, we recommend screening of family members with imaging (Echo and MRI). We may also use laboratory genetic testing to identify those relatives with a disease-causing mutation who could develop overt HCM. We collect blood samples or swabs from the inside of the cheek with results back in 4-6 weeks. Genetic testing does not determine if family members will have the same treatment or course; we test solely to identify who may be at risk for developing HCM. We will discuss with you the benefits of genetic testing as well as any potential financial, psychological, and insurance limitations. Your HCM cardiologist will provide comprehensive genetic counselling and answer any questions you may have.

Atrial fibrillation (AF) is an irregular, and often rapid heartbeat that produces symptoms in at least 20% of HCM patients. AF in HCM is treatable and risk for stroke is preventable using these options:

• Antiarrhythmic medications (to control heart rhythm)
• Anticoagulant medications (to reduce embolic stroke risk)
• Catheter-ablation (to reduce episodes)

Medications are commonly used in HCM to control symptoms, rhythm disturbances, or prevent stroke:

• Antiarrhythmic drugs (e.g. Amiodarone; Sotalol)
• Anticoagulant drugs
• Beta blockers
• Calcium channel blockers (Verapamil)
• Disopyramide (Norpace)
• Myosin inhibitor drugs (currently under investigation and review by the FDA)

Patients who come to our HCM Center have access to the most advanced hypertrophic cardiomyopathy treatment options available today:

• Septal Myectomy: This is a low risk/high benefit open heart surgery that eliminates obstruction, restores heart pressures to normal, and relieves symptoms of shortness of breath — employed in HCM effectively for 60 years.
• Alcohol Septal Ablation: This is a minimally-invasive catheter-based procedure that uses alcohol to burn the thickened heart muscle, relieve obstruction, and decrease heart failure symptoms (shortness of breath) in selected HCM patients. Alcohol ablation is performed in the catheterization lab and is an effective option for patients who are not candidates for, or do not choose, surgery.
• Implantable Cardioverter Defibrillators (ICDs): An ICD is a device that prevents sudden cardiac death by sensing abnormal heart rhythms, automatically reversing them, and re-establishing a normal heart rhythm (pictured below). At Lahey’s HCM Center there is a 95% accuracy rate in selecting which patients will benefit from an ICD placement.
• Heart Transplant: End-stage heart failure in nonobstructive HCM is rare but is treated by heart transplant to extend life expectancy and greatly improve quality of life. Survival after heart transplant for HCM patients is now >90% at 5 years.