Treatment outlook for adults with hypertrophic cardiomyopathy moves from grim to good.

Newly published research led by the Minneapolis Heart Institute Foundation (MHIF) and Tufts Medical Center in Boston shows that implantable defibrillators (ICDs), along with other modern treatments, have reduced mortality rates and are helping patients with hypertrophic cardiomyopathy (HCM) live longer, including normal life expectancy. This research "changes our perceptions of HCM from a grim, unrelenting, and largely untreatable condition to a contemporary disease with effective treatment options and a low rate of death,"
HCM is a genetic heart condition in which the walls of the heart are abnormally thick. Despite the condition, many people with HCM live normal, healthy lives. But others develop cardiac problems that affect their quality of life or, in some cases, lead to early death. In particular, HCM can cause a dangerously rapid heart rhythm that can lead to sudden cardiac death (SCD). With research and technology advances, treatment for adults with HCM has changed dramatically over the past 10-15 years. For example, ICDs, which were originally designed to prevent SCD in people with coronary artery disease, are now commonly prescribed for people with HCM who are at high risk for sudden death. If a dangerously rapid heart rhythm is detected, ICDs work by delivering a shock that restores the heart's normal rhythm.
In the study, while 4% of patients had died from an HCM-related death at follow-up, 6% had survived a life-threatening event, most commonly attributed to an ICD (but also heart transplant, surgical myectomy or out-of-hospital defibrillation). Notably, of 17 HCM sudden deaths, 6 occurred in patients who declined a formal recommendation for ICD therapy and 11 were either initially evaluated in the 1990s prior to general employment of ICDs for HCM, or had no risk factors to justify an ICD decision.