Endomyocardial Fibrosis Treatment & Management

Updated: Dec 29, 2020
  • Author: Ali A Sovari, MD, FACP, FACC; Chief Editor: Henry H Ooi, MD, MRCPI  more...
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Treatment

Approach Considerations

Transfer

Transfer suitable patients with severe symptoms for consideration of endomyocardial resection. Although the operative mortality rate is high (approximately 20%), prognosis is favorably altered in those that survive the surgery.

Consider heart transplant in patients who are classified in function class III or IV.

Deterrence/prevention

No specific preventive measures have been proven effective.

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Medical Care

In general, the response to medical therapy is unproven in endomyocardial fibrosis (EMF) due to a lack of well-designed studies.

Because most patients with EMF present long after any possible period of early active myocarditis may have existed, little, if any, role exists for immunosuppressive therapy as is used in some patients with Löeffler disease. If the patient presents with acute myocarditis, prednisone may be considered as medical therapy.

Symptomatic therapy with diuretics has been shown to be useful. Similar to other patients with diastolic dysfunction, these patients may also benefit from angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), and beta-blockers, but only to some degree. There are experimental studies to support an antifibrotic role for ACE inhibitors and ARBs; however, there are no data for this particular fibrotic disease. 

Pineton de Chambrun et al described the case of a 29-year-old woman with idiopathic hypereosinophilic syndrome who developed severe endomyocardial fibrosis with heart failure during pregnancy. [20]  The patient was successfully treated with prednisone and azathioprine, thereby demonstrating a favorable outcome of endomyocardial fibrosis during pregnancy.

For patients with severe symptoms, consider surgical therapy because the prognosis for these patients with continued medical therapy alone is dismal.

There is no reliable evidence on the rate of the embolic events in these patients and whether anticoagulation and antiplatelet therapy are effective in their prevention. It is reasonable to anticoagulate patients who have thrombus on echocardiography and are compliant with medical treatments. Antiplatelet therapy with aspirin or clopidogrel can be considered as an alternative approach. Those patients with atrial fibrillation should be anticoagulated, although the ability to do this safely and effectively is again limited by availability of local services in endemic regions.

Diet and activity

No specific diet is recommended for this condition. No activity restrictions are recommended because activity restrictions have not been proven to alter the prognosis of this condition, and no specific activity has been shown to hasten the onset of fatal arrhythmias or sudden death.

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Surgical Care

Surgical therapy by endocardial decortication seems to be beneficial for many patients with advanced endomyocardial fibrosis (EMF) who are in functional-therapeutic class III or IV. The operative mortality rate is high (15-20%), but successful surgery has a clear benefit on symptoms and seems to favorably affect survival as well.

The most commonly used approach is endocardiectomy, combined with mitral and/or tricuspid repair or replacement (when indicated), using a midline thoracotomy and cardiopulmonary bypass. Depending on the location of the disease (right or left ventricle, apex or inflow tract), a transapical or transventricular approach can be used.

Because a well-defined plane of cleavage usually exists between the endocardium and myocardium, endocardiectomy is most frequently feasible. Because the myocardium is not usually affected, the severe hemodynamic derangement associated with EMF is relieved with successful resection of the endocardium. Common postoperative complications include low cardiac output, heart block, and ventricular arrhythmias.

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