eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Endocardial Fibroelastosis: Follow-up

Author: Poothirikovil Venugopalan, MBBS, MD, FRCP (Glasg), FRCPCH, Consulting Staff, Department of Child Health, University Hospital of Hartlepool, UK
Contributor Information and Disclosures

Updated: May 14, 2009

Follow-up

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.8

Further Outpatient Care

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

Deterrence/Prevention

  • Withdrawal of therapy may lead to reappearance of symptoms.

Complications

  • 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

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.
    • 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.

Patient Education

  • Withdrawal of therapy may lead to reappearance of symptoms.
  • Permit activity to the limit of tolerance.

Miscellaneous

Medicolegal Pitfalls

  • Failure to identify the disease or to rule out specifically treatable diseases in the differential diagnosis (eg, carnitine deficiency)
  • Failure to recognize an associated congenital heart disease
  • Failure to counsel the parents and caregivers on prognosis
  • Premature discontinuation of medication

Special Concerns

  • Make all possible efforts to detect any treatable cause, such as congenital heart disease or systemic carnitine deficiency.
  • Risk of endocardial fibroelastosis (EFE) in subsequent pregnancies is 3-5% and warrants fetal echocardiography for early diagnosis.
 


More on Endocardial Fibroelastosis

Overview: Endocardial Fibroelastosis
Differential Diagnoses & Workup: Endocardial Fibroelastosis
Treatment & Medication: Endocardial Fibroelastosis
Follow-up: Endocardial Fibroelastosis
Multimedia: Endocardial Fibroelastosis
References
Further Reading
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Further Reading

The American Dietetic Association has released and revised a heart failure evidence-based nutrition practice guideline.

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Keywords

endocardial fibroelastosis, EFE, elastic tissue hyperplasia, endocardial dysplasia, endocardial sclerosis, fetal endocarditis, fetal endomyocardial fibrosis, subendocardial sclerosis, endocardial fibroelastosis, thickening of the ventricular endocardium, unexplained heart failure, congenital heart diseases, aortic stenosis, atresia, primary EFE, secondary EFE, primary endocardial fibroelastosis, secondary endocardial fibroelastosis, acute congestive cardiac failure, congestive cardiac failure, CCF, cardiogenic shock, sudden death in infancy, nonimmune hydrops fetalis, hypoplastic left heart syndrome, coarctation of the aorta, ventricular septal defect, carnitine deficiency, treatment, diagnosis

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Contributor Information and Disclosures

Author

Poothirikovil Venugopalan, MBBS, MD, FRCP (Glasg), FRCPCH, Consulting Staff, Department of Child Health, University Hospital of Hartlepool, UK
Poothirikovil Venugopalan, MBBS, MD, FRCP (Glasg), FRCPCH is a member of the following medical societies: British Cardiac Society and Royal College of Physicians and Surgeons of Glasgow
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital
Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, Cardiac Electrophysiology Society, Heart Rhythm Society, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Julian M Stewart, MD, PhD, Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College
Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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