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Hypertrophic Cardiomyopathy (HCM)
Hypertrophic cardiomyopathy (HCM) is a complex cardiac disease marked by a thickening of the heart muscle (myocardium). The thickening makes it difficult for the heart to pump blood, forcing it to work harder to get blood throughout the body. HCM also causes disruptions in heart rhythm, which sometimes can be fatal. Hypertrophic cardiomyopathy is often asymmetrical, meaning that one part of the heart is thicker than other parts. HCM is usually passed down through families (inherited), and is believed to be a result of several defects in genes that control heart muscle growth. HCM doesn’t affect everyone the same way, with different degrees of muscular wall thickening and a variety of symptoms. Patients may experience shortness of breath, exercise intolerance, chest pressure or pain, fainting or no symptoms at all. In rare cases, adolescents and young adults, with no prior symptoms, can die suddenly without knowing they have the disease. Hypertrophic cardiomyopathy is the most common heart condition that causes sudden death in young athletes and people under the age of 30. The Hypertrophic Cardiomyopathy Program at St. Luke’s and Roosevelt Hospitals, run by nationally known HCM expert Dr. Mark Sherrid, is the only program in the tri-state New York area specializing in evaluating and treating HCM. Our physicians, with more than 20 years experience caring for patients with hypertrophic cardiomyopathy, have made important discoveries about the disease’s mechanisms, and offer comprehensive diagnostic evaluations, a wide range of treatments, and screening for family members who might be at risk. To properly diagnosis HCM, St. Luke’s and Roosevelt Hospital cardiologists use echocardiography or sound wave imaging to photograph the heart and determine the thickness of its muscle. Normally, heart walls are 11 mm or less in thickness, but patients with HCM have a characteristic thickening of the heart’s walls that can range from 14 to 60 mm. Patients with obstructive HCM have blockages inside the ventricular septum—the part of the heart that separates the right and left ventricles. The blockage restricts the flow of oxygenated blood from the left ventricle into the aorta and ultimately the body. In patients with unobstructive HCM, the ventricles’ walls thicken, but not in a way to impede blood flow out of the heart. Rather, the chambers can’t sufficiently relax to fill with blood, making the pumping action inefficient. Patients with unobstructive HCM can experience shortness of breath, chest pain and blackouts. However, they usually are at low risk of developing complications or dying, and often require no treatment. After a diagnosis of hypertrophic cardiomyopathy, each patient’s treatment plan is based on severity of symptoms, whether they have obstructive or non-obstructive HCM and any other mitigating circumstances. Most hypertrophic cardiomyopathy patients with symptoms can be treated successfully with medications. Patients with obstructions or symptoms that do not respond to medication generally benefit from surgery. Patients who don’t want surgery or have other illnesses that would complicate a surgical solution may benefit from pacemakers. Patients at risk for sudden death because of the presence of high risk factors are treated with medication or implantable cardioverter defibrillators—devices that shock the heart back to normal rhythm if a life-threatening irregular heartbeat occurs. Defibrillators are appropriate for only a minority of patients with HCM. For a referral to Dr. Mark Sherrid, Director of The Hypertrophic Cardiomyopathy Program at St. Luke’s and Roosevelt Hospitals in Manhattan, New York City, call 1 (877) 996-9334 or (212) 492-5550. |
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