General Outlook for Patients with Hypertrophic Cardiomyopathy

The severity of symptoms and risk of complications varies greatly between HCM patients, and it should be emphasized that many people never experience serious problems related to their disease. Indeed, HCM is compatible with normal longevity. When considering the overall population of adult patients with HCM, this disease may not add significantly to an individual patient's risk (including the known risks of living, such as cancer, coronary heart disease, or accidents), and may not limit considerably the quality or duration of life. The most accurate mortality rate for the overall disease is about one percent per year, which means that each year one of 100 patients may die for a reason directly related to their disease.

On the other hand, some patients do experience significant symptoms and/or disability, or may be at risk for premature death. There are three circumstances in which patients with HCM die prematurely:

1) suddenly and unexpectedly often in the younger patients
2) related to severe progressive heart failure, usually in mid-life
3) due to stroke, usually in older patients with atrial fibrillation.

Disabling symptoms such as shortness of breath and/or chest pain with exertion may remain stable throughout much of adult life, or they may deteriorate and require an intervention. Each patient with HCM, however, must be assessed individually to determine the subgroup of patients to which they most likely belong.

It is an important general principle regarding HCM, therefore, that all patients are not the same in terms of prognosis, clinical presentation, or determination of appropriate treatment. There are several different clinical profiles that patients may present, and it is important not to "lump" these together under the homogeneous label of HCM. Many patients have virtually no risk associated with their disease and some deserve high-risk status. However, the overall disease is not, per se, a high-risk condition, and HCM should not be regarded as a uniformly unfavorable disease.

Profiles in prognosis. Many patients with HCM can be placed in one of these categories for the purposes of judging prognosis, as well as eventually formulating treatment strategies. This diagram emphasizes the necessity of viewing HCM in terms of such subgroups so that treatment can be tailored to particular clinical profiles, with the proper selection of patients.

 
Adapted from Hypertrophic Cardiomyopathy: For Patients, Their Families, and Interested Physicians, by Barry J. Maron, MD, et al., Copyright 2001, with permission from Futura Media Services, Inc., Armonk, New York