Endocardial Fibroelastosis (EFE) Treatment- Cardiology

Doctors: Cardiologist, Cardiac Surgeon

What is Endocardial Fibroelastosis (EFE)?

Endocardial fibroelastosis (EFE) is a rare heart disease, which affects infants and children. It is characterized by a thickening within the muscular lining of the heart chambers due to a rise in the quantity of supportive connective tissue (inelastic collagen) and elastic fibers.

Treatment:

If infants are diagnosed during the early stage of the disease, they respond more favorably to the treatment compared to those who are not diagnosed until considerable heart damage has already happened.

The treatment options for EFE and chronic heart failure are the same. Treatment options include medicines and surgery.

• Medicines:
  • Early and persistent treatment with digoxin is recommended. Even the symptoms disappear, continuation of this medicine is recommended. However, termination of the medicine might result in acute cardiac failure, even if the heart size has returned to normal.
  • Anticoagulants (to prevent blood clotting) might be necessary in the presence of thromboembolic complications.
  • Diuretics might be used to eliminate fluids from the body.
  • Antiarrhthmics might be prescribed to maintain normal heart rhythm and might be used to correct arrhythmias.
  • Prolonged bed rest might expedite healing of myocardial lesions since the heart is working at a reduced load in resting condition.
• Surgery:
  • Cardiac transplantation might be recommended for patients with end-stage disease.

References:

• https://rarediseases.org/rare-diseases/endocardial-fibroelastosis/
• https://emedicine.medscape.com/article/896375-treatment#d5
• https://emedicine.medscape.com/article/896375-treatment#d6

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