Hypertrophic cardiomyopathy (HCM) is a complex cardiac disease marked by a thickening of the heart muscle that occurs in the absence of significant high blood pressure or heart valve disease. The disease doesn’t affect everyone the same way, with different degrees of muscular wall thickening and a variety of symptoms. Patients may have 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 that they have the disease. HCM is the most common cause of sudden cardiac death in young athletes and in people under 30 years of age.

Our HCM program is the only such program specializing in the evaluation and treatment of this condition in the tri-state New York metropolitan area. Our physicians have treated patients with this condition for 20 years, and have made important discoveries about the mechanism of the disease and its successful treatment. We offer a comprehensive diagnostic evaluation, a range of treatments, and screening for relatives of affected patients.

Proper therapy depends on accurate diagnosis of HCM. We make the initial diagnosis with echocardiography, a noninvasive imaging technique that uses sound waves to picture the heart and test the heart wall thickness. Normally, heart walls are 11 mm or less in thickness, but patients with HCM have a characteristic thickening of the heart walls that can range from 14 to 60 mm.

After diagnosis, each patient’s treatment plan is based on the severity of symptoms, diagnosis of obstructive or non-obstructive HCM and results of further evaluation. Among the many variations that can occur in HCM, some patients are found to have obstruction while others are non-obstructed. Patients in both groups have heart thickening and both can have troubling symptoms. Patients with obstruction have a blockage inside the heart that results in the heart overworking as it pumps blood to the body. Obstructive HCM used to be called idiopathic hypertrophic subaortic stenosis (IHSS). In patients without obstruction the thickening of the heart itself results in the symptoms. Since sudden death is a potentially catastrophic complication in patients with symptoms, we often perform further tests to assess risk.

Patients without symptoms are at low risk of developing complications or death and often require no treatment. Most patients with symptoms can be treated successfully with medications. Patients with outflow tract obstruction and symptoms that do not respond to medication generally benefit from surgery. A patient who does not want surgery or who has other illnesses complicating his or her situation may benefit from a pacemaker.

Those patients at risk for sudden death because of the presence of risk factors may be treated with medication or an implantable cardioverter defibrillator, a device that shocks the heart back to normal rhythm if a life-threatening irregular heart rhythm occurs. Defibrillators are appropriate for only a minority of patients with HCM.