Hypertrophic Cardiomyopathy (HCM) is the most common inherited heart disease; it causes thickening of the heart muscle without a clinical cause to explain the extent of thickening observed.
HCM causes symptoms of dyspnea or shortness of breath, chest pain, exercise intolerance, syncope or fainting, and uncommonly, sudden cardiac death (SCD). It affects individuals of all ages but most commonly presents after age 30. Many patients with HCM have a relatively benign course and can have normal life expectancy, and symptoms can be managed with first-line pharmacologic agents like beta blockers or verapamil. However, a quarter of patients will experience in their course either severe disabling symptoms or SCD.
HCM differs in the pharmacotherapy offered for symptoms from other cardiac diseases- potent negative inotropic drugs are the mainstay of treatment for obstruction. Surgical septal myectomy is a highly effective procedure for relief of symptoms when pharmacotherapy is ineffective. The surgical procedure removes a small part of the heart’s thickened muscle wall to restore proper blood flow within the heart. Relief of obstruction through either advanced pharmacotherapy or myectomy has been associated with very low mortality in the ensuing years.
Overall, SCD occurs in 1 HCM patient per 100 patient/years. For the individual patient we cannot reliably predict who will suffer SCD. However in populations the presence of risk factors may allow prediction of higher risk, on the order of 2-3 percent/year. This level of risk assumes clinical importance because of its cumulative effect in young patients who may otherwise anticipate normal life expectancies of 30-50 more years. These patients comprise up to a quarter of the HCM patients who present for clinical consultation; it is these patients who are candidates for implanted cardioverter-defibrillator therapy to prevent SCD. Atrial fibrillation occurs in 25 percent of HCM patients and often exacerbates symptoms.
Treatment for severely affected patients with HCM has advanced measurably in recent years. Twenty years ago such HCM patients had a poor prognosis and little could be offered to improve symptoms. Now we have a variety of highly effective treatments to both improve functional status and outcomes for almost all patients. In this period of time there has been a corresponding transit from outpatient care to inpatient advanced care.
Under my leadership and partnership with Daniel Swistel, MD, in Cardiac Surgery the HCM Program at Mount Sinai Roosevelt and Mount Sinai St. Luke’s hospitals has grown to follow 1,180 patients since its inception in 1985. We have developed expertise in the advanced therapy of symptoms with the combination of disopyramide and beta blocker therapy; patients who fail this combination or young patients who have high resting outflow gradients and long mitral valve leaflets are often good candidates for a surgical septal myectomy procedure.
We have developed novel surgical treatments that comprehensively address all components of HCM pathophysiology (both the thickened septum and the elongated mitral valve) that have allowed patients to return to full functional capacity and NYHA 1 status, meaning they experience no more daily symptoms or limitations on their physical activity.
The HCM Programs of Mount Sinai St. Luke’s and Mount Sinai Roosevelt hospitals are offering a Symposium titled “Hypertrophic Cardiomyopathy Treatment: Medical, Surgical, Sudden Death Prevention and Newer Modalities,” at the New York Yale Club on April 18 and 19.
For more information visit: www.chpnet.org/CME or email: aroman@chpnet.org.
View event’s brochure: https://cmetracker.net/STLUKESCME/Files/Brochures/62923.pdf
Mark V. Sherrid, MD, FACC, FASE is Director of the Echocardiography Laboratory and Program Director of the Hypertrophic Cardiomyopathy Program at Mount Sinai Roosevelt and Mount Sinai St. Luke’s. He also serves as Professor of Medicine (Cardiology) at Icahn School of Medicine at Mount Sinai.