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

Endocardial Fibroelastosis: Treatment & Medication

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

Treatment

Medical Care

  • The treatment of endocardial fibroelastosis (EFE) is essentially the same as that of chronic cardiac failure; 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.
  • 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.

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

  • 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.

Activity

  • Limitations to activity are dictated by the symptomatology.

Medication

If the patient is asymptomatic and his or her heart size is normal, provide early, adequate, and prolonged therapy with digitalis and diuretics for at least 2-3 years, with gradual discontinuation. 

Early and prolonged treatment with digoxin is suggested. Anticoagulants may be required in the presence of thromboembolic complications.

Antibiotics for endocarditis prophylaxis are administered to patients with certain cardiac conditions, such as endocardial fibroelastosis, before procedures that may cause bacteremia are performed. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

Diuretics

These agents are used to eliminate retained fluid and to lower preload.


Furosemide (Lasix)

Inhibits reabsorption of fluid from ascending limb of Henle loop in renal tubule. IV administration has a venodilator action. Lowers preload even before diuresis sets in. DOC in acute heart failure and in exacerbations of chronic heart failure. Used for long-term management of chronic heart failure.

Adult

40 mg PO bid; not to exceed 200 mg/d
Alternatively, 20-50 mg IV q6-8h

Pediatric

1-4 mg/kg PO qd/bid
Alternatively, 1-4 mg/kg IV q8h

Metformin decreases furosemide concentrations; furosemide interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; auditory toxicity appears to increase upon coadministration of aminoglycosides and furosemide; varying degrees of hearing loss may occur; anticoagulant activity of warfarin may be enhanced when administered concurrently; increased plasma lithium levels and toxicity are possible when administered concurrently

Documented hypersensitivity; severe hypovolemia; severe electrolyte imbalance; hepatic failure with impending encephalopathy

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Hypokalemia, hyponatremia, and hypotension are possible; aggravates diabetes mellitus, porphyria, and liver failure; use caution in pregnancy and breastfeeding


Spironolactone (Aldactone)

Potassium-sparing diuretic that acts on distal convoluted tubule of kidney as an aldosterone antagonist. Has synergistic action with furosemide.

Adult

100-200 mg PO qd

Pediatric

0.5-1.5 mg/kg PO bid

Risk of hyperkalemia with ACE inhibitors, cyclosporin, or potassium supplements

Documented hypersensitivity; hyperkalemia, hyponatremia, and severe renal impairment; Addison disease

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Main adverse effects include GI upset, hyponatremia, hyperkalemia, hepatotoxicity, lethargy, confusion, impotence, and gynecomastia; reportedly carcinogenic in rodents

ACE inhibitors

These agents reduce afterload and decrease myocardial remodeling, which worsens chronic heart failure.


Captopril (Capoten)

Accepted as an essential part of any antifailure therapy; promotes symptomatic improvement and enhances survival.

Adult

6.25-25 mg PO tid

Pediatric

0.1-1 mg/kg PO tid; initiate at lower dose; if needed, gradually titrate upward

Anesthetic agents enhance hypotensive effect; NSAIDs enhance renal impairment; cyclosporin enhances risk of hyperkalemia; potassium-sparing diuretics or potassium supplements enhance risk of hyperkalemia

Documented hypersensitivity; renal artery stenosis; LV outflow obstruction

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Pregnancy category C in first trimester; main adverse effects include hypotension, tachycardia, and renal failure; must commence therapy while patient has adequate BP and satisfactory state of hydration; small doses started while in hospital, and BP is monitored; renal function assessed before increasing dose; persistent dry cough reported in 5-20% of children; may require change to another agent in the category or to an angiotensin receptor blocker; other adverse effects include angioedema, skin rash, serum sickness, GI upset, pancreatitis, hepatitis, cholestatic jaundice, blood dyscrasias, bronchospasm, headache, dizziness, and fatigue

Cardiac glycosides

These agents provide symptomatic improvement.


Digoxin (Lanoxin)

Improves myocardial contractility, reduces heart rate, and lowers sympathetic stimulation in chronic heart failure.

Adult

Maintenance dose: 125-250 mcg PO qd

Pediatric

Maintenance dose:
Preterm infant: 5-7.5 mcg/kg/d PO divided bid
Term infant: 6-10 mcg/kg/d PO divided bid
1 month to 2 years: 10-15 mcg/kg/d PO divided bid
2-5 years: 7.5-10 mcg/kg/d PO divided bid
5-10 years: 5-10 mcg/kg/d PO divided bid
>10 years: 2.5-5 mcg/kg PO qd

Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, PO amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil; medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, PO colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid

Documented hypersensitivity; severe hypokalemia; renal failure; WPW syndrome with antegrade conduction of accessory pathway; AV block; idiopathic hypertrophic subaortic stenosis or constrictive pericarditis

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Reduce dose in renal impairment; avoid hypokalemia; avoid IV administration except when absolutely essential; avoid in sick sinus syndrome or thyroid disease; monitor blood level in suspected toxicity and high-risk situations; major adverse effects include arrhythmias and heart block and noncardiac effects (eg, vomiting, nausea, abdominal pain, visual disturbances, headache, and fatigue); paroxysmal atrial tachycardia with block is characteristic arrhythmia

Oral anticoagulants

These agents prevent recurrence of thromboembolic episodes of cardiac origin.


Warfarin (Coumadin)

Prevents thrombus formation within cardiac chambers and venous circulation by antagonizing effects of vitamin K.

Adult

3-9 mg PO qd; adjust to keep INR 2.5-3

Pediatric

Administer as in adults

Drugs that may decrease anticoagulant effects include griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, PO contraceptives, and sucralfate; medications that may increase anticoagulant effects of warfarin include PO antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac

Documented hypersensitivity; severe liver or kidney disease; open wounds or GI ulcers

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Requires loading dose to initiate therapy; if immediate anticoagulation is desired, administer IV heparin; usual adverse effects include hemorrhaging, hypersensitivity, rashes, alopecia, diarrhea, jaundice, hepatic dysfunction, nausea, vomiting, and pancreatitis; warfarin sodium tablets are teratogenic

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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