HCM Therapy

Treatment cannot begin until there is a correct diagnosis. Diagnosis is usually made with echocardiography, an ultrasound examination of the heart. Echocardiography is a non-invasive, non-Xray, imaging technique that uses sound waves to picture the heart. MRI (magnetic resonance imaging) of the heart is often ordered for clarification of ambiguities and to assess the degree of fibrosis in the heart. 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.

Since sudden death is a potentially catastrophic complication in patients with HCM, we attempt to assess individual risk. Though we can estimate the risk for populations of patients with HCM, in individual patients it is impossible to accurately predict risk for sudden death. Another way of saying this is that “we don’t have a crystal ball” to predict the future. However, for patients who are deemed to be at high risk because of the presence of risk factors we recommend implantation of an implantable defibrillator, either an intravenous or subcutaneous device depending on the circumstances. The implanted automatic defibrillator, is a device that shocks the heart back to normal rhythm if a life-threatening irregular heart rhythm occurs. The decision to implant a ICD depends on judgments concerning the potential benefits and risks of the ICD and its leads.

Patients without symptoms who are determined to be at low risk of developing complications or death often require no treatment. Most patients with symptoms can be treated successfully with medications:

Treatment of symptoms in obstructive HCM: Symptomatic patients with obstruction usually are first begun on beta-blocker medication. If this does not help, other medications, disopyramide or verapamil often are successful at relieving obstruction and symptoms.

Medical treatment of hypertrophic cardiomyopathy

Refractory symptoms: Patients with outflow tract obstruction and symptoms that do not respond to medication generally benefit from surgery called extended myectomy.

Surgery has been performed for more than 40 years and is generally considered the gold-standard for relief of obstruction in medically refractory patients. Non-surgical interventions to relieve obstruction are available as well.

Alcohol ablation of the septum is a non-surgical procedure to improve outflow tract obstruction. It is a percutaneous catheter-based method to decrease septal thickness by therapeutic myocardial infarction. It is generally reserved for patients who cannot have surgical myectomy because of their age or other diseases, or who strongly prefer a non-surgical approach. Older patients or those with significant medical conditions who are not candidates for surgery or ablation may benefit from a dual chamber pacemaker implantation.

Treatment of non-obstructive HCM: Symptoms of shortness of breath, chest pain and blackouts can also occur in patients with no obstruction. These symptoms can generally be treated with medication as well. For patients with non-obstructive hypertrophic cardiomyopathy, verapamil is often the best choice because of its efficacy in relieving myocardial ischemia. The usual surgical procedure for HCM (myectomy that relieves obstruction) is not of help for these patients, who are then, generally treated medically. Alcohol ablation has no role in the treatment of non-obstructive HCM. Very uncommonly, patients with progressive HCM require heart transplantation.

Those patients at risk for sudden death because of the presence of risk factors may be treated with medication or an implanted automatic defibrillator, a device that shocks the heart back to normal rhythm if a life-threatening irregular heart rhythm occurs.The decision to implant a ICD depends on judgments concerning the potential benefits and risks of the ICD and its leads.

Treatment is often a multidisciplinary approach. We coordinate the efforts an echocardiographer, electrophysiologist, pacemaker specialist, cardiac surgeon or interventional cardiologist for rapid and thorough evaluation and treatment.

To see New York University Medical Center HCM Program research publications, click here.