Patients with hypertrophic cardiomyopathy live into their 90s

Hypertrophic cardiomyopathy (HCM) is consistent with survival to normal life expectancy, including particularly advanced age into the tenth decade of life, with demise ultimately largely unrelated to this disease, according to a study being presented Nov. 13 at the American Heart Association (AHA) scientific sessions in Orlando, Fla.

HCM is the most common cause of sudden death in the young, but survival to a particularly advanced age is less well understood.

"In the past, this disease has been associated with a grim prognosis, due to the deadly nature in young people, but we have learned through this analysis that those assumptions were inaccurate," said the study's lead author Barry J. Maron, MD, director of the Hypertrophic Cardiomyopathy Center at the Minneapolis Heart Institute Foundation. "We are continuing to learn about this unique disease state."

In the study, Maron and colleagues assessed the prevalence, clinical features and demographics of HCM patients surviving to the age of 90 years or older through an interrogation of the Minneapolis Heart Institute Foundation's HCM Center database.

Of the 1,297 HCM patients, 26 had achieved the age of at least 90 years; 69 percent were women. The age at which HCM was diagnosed ranged from 61 to 92 years, with disease recognition under fortuitous circumstances by detection of a heart murmur or during family screening (six patients), or after onset of new symptoms (20 patients).

At the most recent evaluation (or death) patients were 90.0 to 96.7 years of age, with six presently alive at 90 to 96 years of age. Maron noted that HCM did not appear to be the primary cause of demise in any patient.

HCM-related complications occurred in18 patients, including heart failure symptoms, atrial fibrillation and non-fatal embolic stroke. Although no patient died suddenly, 13 still carried conventional HCM markers of risk.

Interestingly, a greater proportion of these HCM patients reached the age 90 years of older (2 percent) than expected in the general population (0.8 percent).

"We showed that hypertrophic cardiomyopathy - the most common cause of death among young people - is associated not only with normal life, but also extended longevity," Maron said. "These findings underscore a principle of the disease that has been falsely assumed; namely, that this disease will lead to an early demise in all patients."

Finally, these data can reassure mainly patients who are diagnosed with HCM that their lives will not necessarily be cut short, Maron concluded.
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