Expect Longevity With Heart Muscle Disease

Hypertrophic cardiomyopathy patients can live normal life spans

(RxWiki News) Living with hypertrophic cardiomyopathy (HCM), a genetic heart condition in which the heart becomes thick, doesn't have to be a death sentence. New guidelines suggest patients can live normal life spans.

Joint recommendations from the American College of Cardiology Foundation and the American Heart Association suggest that HCM, the most common inherited heart disease, is a manageable condition.

"Utilize a cardiologist who specializes in HCM if possible."

Dr. Bernard J. Gersh, co-chairman of the guideline writing committee and professor of medicine at the Mayo Clinic College of Medicine, said that the HCM is widely misperceived as a fatal condition, but noted it is not a diagnosis of sudden cardiac death.

Effective treatments are available to manage the condition, though it remains the most common cause of sudden death in young or competitive athletes as a result of erratic heart beats or obstructed blow flow that can prompt sudden cardiac death, he said.

The writing committee made several recommendations, including:

  • Seek treatment at a clinical center that focuses on the disease where many medical and surgical treatments are available.
  • Consider drug therapy with beta-blockers, verapamil or disopyramide to control symptoms such as shortness of breath.
  • HCM patients who develop advanced heart failure and don't respond to medication should consider transaortic surgical septal myectomy to remove excessive muscle from the septum and improve the symptoms.
  • If not a surgical candidate, catheter-based alcohol septal ablation in which alcohol is introduced to the heart to cause a heart attack to reduce septum muscle mass may be an alternative treatment option.
  • All HCM patients should receive a comprehensive evaluation of their risk of sudden cardiac arrest, including a personal and family history, and noninvasive assessment with an echocardiogram.
  • Patients should be receive genetic counseling and testing, if appropriate.
  • Patients may participate in low-intensity competitive sports, such as golf and recreational sports, but HCM patients should be disqualified from participation in most competitive sports, whether or not they have obstruction.
  • Use of implantable defibrillators in high risk patients with a family history of the condition, or who experiences fainting, can help prevent sudden death.

 

The new guidelines were published in Circulation: Journal of the American Heart Association and Journal of the American College of Cardiology.

Review Date: 
November 10, 2011