Endocardial Fibroelastosis Follow-up

Updated: Feb 21, 2014
  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: Stuart Berger, MD  more...
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Follow-up

Further Outpatient Care

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Educate patients about the potential for the reappearance of symptoms if therapy is withdrawn.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

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Further Inpatient Care

Further inpatient care in patients with endocardial fibroelastosis (EFE) may be required for exacerbations of heart failure and for invasive procedures.

Although beta blockers are used frequently in adults with heart failure, a recent study concluded evidence is insufficient to recommend or discourage their use in children. [11]

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Complications

Complications include the following:

  • Resistant cardiac failure

  • Recurrent chest infections

  • Severe failure to thrive

  • Cardiac cirrhosis

  • Cerebral, coronary, and pulmonary thromboembolism

  • Persistent collapse of left lower lobe or entire left lung

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Prognosis

Primary endocardial fibroelastosis prognosis is relatively poor, although the condition is not universally fatal.

The prognosis is worse if onset of heart failure occurs earlier.

Congestive heart failure (CHF) may be present. Note the following:

  • Acute CHF becomes progressive CHF and terminates in death within weeks, usually within the first 6 months of life.

  • In patients who survive from a few months to several years, a more chronic course is common. Such patients respond to medications for CHF. A variable cyclical clinical course ensues, with CHF recurrence related to respiratory or other intercurrent infections or to progression of disease. Remissions can occur through intensification of medical therapy.

  • Progressive CHF causes deteriorating conditions that lead to death in one third of patients. One third of the patients survive and may experience persistent symptoms or show residual ECG abnormalities or evidence of cardiomegaly. Some believe that approximately one third of patients completely recover, although others are skeptical.

  • Early diagnosis and prompt persistent administration of digitalis may result in clinical improvement and reversion of the ECG and cardiac enlargement (CE) to normal.

  • Poor prognostic signs of CHF include (1) presentation in the newborn period and (2) recurrent episodes of CHF despite adequate therapy, especially if episodes recur more than 6 months after initial onset of symptoms.

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