Medical Care

Although no cure exists for endocardial fibroelastosis (EFE), treatment typically centers on the patient's symptoms, which frequently focuses on chronic cardiac failure^[2]; its acute exacerbations are often precipitated by respiratory infections.

Early and prolonged treatment with digoxin is suggested. Continue therapy for several years after the symptoms disappear; cessation of drug administration may result in acute cardiac failure, even when heart size has returned to normal.

Other measures for acute failure and exacerbations of failure may be required, and precipitating factors, such as infection and anemia, require attention. Invasive procedures may be required.

Although beta blockers are used frequently in adults with heart failure, a Cochrane review concluded evidence is insufficient to recommend or discourage their use in children.^[15]

Anticoagulation may be required in the presence of thromboembolic complications.

Case reports in the literature cite resolution of antenatally diagnosed endocardial fibroelastosis associated with positive anti-Ro and anti-La antibodies with corticosteroid therapy.

Outpatient follow-up

Schedule regular follow-up care until symptoms subside and cardiac size and function are normal.

Educate patients about the potential for the reappearance of symptoms if therapy is withdrawn.

Permit activity to the limit of tolerance.

Surgical Care

Both pericardial poudrage and mitral valve (MV) replacement have had disappointing results.

Cardiac transplantation may be recommended for patients with end-stage disease.

Consultations

Consider consultations with the following specialists:

Pediatric cardiologist
Radiologist
Nuclear medicine specialist
Family physician
Occupational therapist
Physiotherapist
Psychologist
School teacher
Specialist nurse
Pharmacist
Dietitian

Diet

Diet is dictated by the underlying heart disease and degree of malnutrition.

The Academy of Nutrition and Dietetics provides recommendations regarding heart failure and evidence-based nutrition.

Activity

Limitations to activity are dictated by the symptomatology.

Medication

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Media Gallery

Endocardial Fibroelastosis. Chest radiograph, anteroposterior view, showing cardiomegaly and pulmonary venous congestion in a 6-month-old infant with endocardial fibroelastosis (EFE).
Endocardial Fibroelastosis. Chest radiograph, left lateral view, showing an enlarged heart in 6-month-old infant.