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March 8, 2000

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Rare But Deadly Heart Disease Is Curable, Study Shows

"This illness has a very rapid progression...patients can die suddenly." Joshua Hare, M.D.

Fatigue, fever and muscle aches classic symptoms of flu are for a small number of patients symptoms of a very rare but deadly form of heart disease.

The condition, fulminant myocarditis, is underdiagnosed. The good news, according to a study led by Johns Hopkins researchers, is that patients who are properly diagnosed and treated aggressively can fully recover from the condition within two weeks without much damage to the heart or chance of relapse.

Results are reported in the March 9 issue of the New England Journal of Medicine.

"This illness has a very rapid progression and the consequences are very dramatic -- patients can die suddenly," says Joshua M. Hare, M.D., associate director of the heart failure and cardiac transplant program at Hopkins and an author of the study. "We want to make physicians aware that although this condition is uncommon, it should be considered in patients with flu-like symptoms and fluid in the lungs. An echocardiogram suggests the disease, and a heart tissue biopsy confirms the diagnosis."

Fulminant myocarditis is a severe inflammation of the muscular wall of the heart, usually caused by a viral or other infection, or an immune disorder. It comes on suddenly, leaving the heart virtually powerless to contract and circulate blood. The condition is so rare that it affects less than one in a million patients, yet in those people it often proves fatal.

Effective treatments include medications to stimulate the heart muscle, thereby increasing blood flow, or an implantable mechanical pump to temporarily assist the heart with circulation, Hare says. Pumps can usually be removed safely after seven to 10 days.

"Physicians should use any means necessary to maintain these patients' heart function, as virtually all will recover within two weeks and then lead a normal life," says Kenneth L. Baughman, M.D., senior author of the study and director of the Division of Cardiology at Hopkins.

Patients with fulminant myocarditis appear much more seriously ill than those with acute myocarditis, a less severe form of the disease that yields shortness of breath and fatigue. Yet "surprisingly, they fare better in the long run, have longer life expectancy and are less likely to need a heart transplant," Hare says.

Researchers compared the outcomes of 15 patients treated at Johns Hopkins for fulminant myocarditis and 132 patients seen for acute myocarditis between 1984 and 1997. The patient population for the fulminant group was 73 percent caucasian and 73 percent male, and had a mean age of 35. The acute group was 74 percent caucasian and 64 percent male, and had a mean age of 43.

None of the fulminant myocarditis patients needed a heart transplant, compared with seven of the nonfulminant group who did. Ninety-three percent of those with the fulminant form were still alive 11 years following diagnosis. By comparison, only 45 percent of those with the less severe form were living within the same time interval.

The study was supported in part by the National Institutes of Health. Other authors were Robert E. McCarthy III, M.D.; Ralph H. Hruban, M.D.; Grover M. Hutchins, M.D.; and Edward K. Kasper, M.D., of Hopkins; and John P. Boehmer, M.D., of Hershey Medical Center, Hershey, Pa.

Related Web Sites:

Johns Hopkins Cardiomyopathy and Heart Transplant Service:

American Heart Association:

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