Restrictive cardiomyopathy associated with the eosinophilia-myalgia syndrome.

OBJECTIVE

We report the first case of restrictive cardiomyopathy occurring in a patient with the eosinophilia-myalgia syndrome.

DESIGN

In this article, we discuss the various clinical manifestations of the eosinophilia-myalgia syndrome.

MATERIAL AND METHODS

In a 46-year-old woman with the eosinophilia-myalgia syndrome, orthopnea, chronic persistent edema, and severe dyspnea on exertion developed 2 years after she had discontinued use of L-tryptophan. Doppler echocardiography showed ventricular filling confined to early diastole and no atrial filling during ventricular systole--the Doppler hallmarks of restrictive disease. Right-sided cardiac catheterization revealed that the pulmonary wedge pressure equaled the pulmonary artery diastolic pressure and the mean right atrial pressure. A myocardial biopsy specimen showed dense endocardial fibrosis. Special immunofluorescent stains for eosinophilic granule major basic protein showed substantial deposition along the endocardial myocardial interface, an indication that eosinophils were present some time in the past.

RESULTS

A follow-up telephone call 14 months after the patient's initial assessment at the Mayo Clinic revealed that she had class III symptoms of congestive heart failure. She was receiving high doses of three diuretics daily, and her condition had improved considerably since her first examination at our institution.

CONCLUSION

Restrictive cardiomyopathy may occur in the setting of the eosinophilia-myalgia syndrome and should be considered in patients with this disease in whom exertional dyspnea and peripheral edema occur.