Endocardial Fibroelastosis
Classification and external resources
ICD-9	425.3
OMIM	226000 305300

Endocardial Fibroelastosis (EFE) is a rare heart disorder usually associated with children two years old and younger.^[1]

It should not be confused with endomyocardial fibrosis.

Contents 1 Presentation 2 Causes 3 History 4 Treatment 5 See also 6 References

[edit] Presentation

It is characterized by a thickening within the muscular lining of the heart chambers (the endocardium) due to an increase in the amount of supporting connective tissue and elastic fibers.

It is an uncommon cause of unexplained heart failure in infants and children.

It is one component of HEC syndrome.^{[citation needed]}

[edit] Causes

EFE has two distinct genetic forms, each having a different mode of inheritance. An x-linked recessive form,^[2] and an autosomal recessive form^[3] have both been observed.

There is also some evidence that viral infection may play a role as a cause or trigger.^[4]

[edit] History

The term was introduced by Weinberg and Himmelfarb in 1943.^[5]

[edit] Treatment

One form of treatment is heart transplant.^[6]

[edit] See also

• Papillary fibroelastoma

[edit] References

v • d • e Cardiovascular disease: heart disease · Circulatory system pathology (I00-I52, 390-429) Ischaemic CD/CHD CAD · Coronary thrombosis · Coronary vasospasm · Coronary artery aneurysm · Coronary artery dissection · Myocardial Bridge Active ischemia Angina pectoris (Prinzmetal's angina, Stable angina) · Acute coronary (Unstable angina, Myocardial infarction / heart attack) Sequelae hours (Myocardial stunning, Hibernating myocardium) · days (Myocardial rupture) · weeks (Aneurysm of heart/Ventricular aneurysm, Dressler's syndrome) Layers Pericardium Pericarditis (Acute, Chronic/Constrictive) · Pericardial effusion (Hemopericardium, Cardiac tamponade) Myocardium Myocarditis (Chagas disease) Cardiomyopathy: Dilated (Alcoholic) · Hypertrophic · Restrictive (Loeffler endocarditis, Cardiac amyloidosis, Endocardial fibroelastosis) Arrhythmogenic right ventricular dysplasia Endocardium/ valves Endocarditis Infective endocarditis (Subacute bacterial endocarditis) · noninfective endocarditis (Nonbacterial thrombotic endocarditis, Libman-Sacks endocarditis) Valves mitral (regurgitation, prolapse, stenosis) · aortic (stenosis, insufficiency) · tricuspid (stenosis, insufficiency) · pulmonary (stenosis, insufficiency) Conduction/ arrhythmia Bradycardia Sinus bradycardia · Sick sinus syndrome Heart block: Sinoatrial · AV (1°, 2°, 3°) · Intraventricular (Bundle branch/Right/Left, Left anterior fascicular/Left posterior fascicular, Bifascicular/Trifascicular) · Adams-Stokes syndrome Tachycardia (paroxysmal and sinus) Supraventricular Atrial (Multifocal) · Junctional (AV nodal reentrant, Junctional ectopic) Ventricular Torsades de pointes · Catecholaminergic polymorphic · Accelerated idioventricular rhythm Premature contraction Atrial · Ventricular Pre-excitation syndrome Wolff-Parkinson-White · Lown-Ganong-Levine Flutter/fibrillation Atrial flutter · Ventricular flutter · Atrial fibrillation (Familial) · Ventricular fibrillation Pacemaker Wandering pacemaker · Ectopic pacemaker/Ectopic beat · Parasystole · Multifocal atrial tachycardia · Pacemaker syndrome Long QT syndrome Romano-Ward syndrome · Andersen-Tawil syndrome · Jervell and Lange-Nielsen syndrome Cardiac arrest Sudden cardiac death · Asystole · Pulseless electrical activity · Sinoatrial arrest Other/ungrouped hexaxial reference system (Right axis deviation, Left axis deviation) · QT (Short QT syndrome) · T (T wave alternans) · ST (Osborn wave, ST elevation, ST depression) Cardiomegaly Ventricular hypertrophy (Left, Right/Cor pulmonale) · Atrial enlargement (Left, Right) Other Cardiac fibrosis · Heart failure (Cardiac asthma) · Rheumatic fever heart navs: anat/physio/dev, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

v • d • e Congenital heart defects (Q20-Q24, 745-746) Cardiac shunt/ heart septal defect Aortopulmonary septal defect R→L: Double outlet right ventricle (Taussig-Bing syndrome) · Transposition of the great vessels (dextro, levo) · Persistent truncus arteriosus Aortopulmonary window Atrial septal defect L→R: Sinus venosus atrial septal defect · Lutembacher's syndrome Ventricular septal defect L→R and R→L: Eisenmenger's syndrome R→L, with other conditions: Tetralogy of Fallot Atrioventricular septal defect L→R: Ostium primum Valvular heart disease/ heart chambers Right pulmonary valves (stenosis, insufficiency) · tricuspid valves (stenosis, atresia, Ebstein's anomaly) · Hypoplastic right heart syndrome Left aortic valves (stenosis, insufficiency, bicuspid) · mitral valves (stenosis, regurgitation) · Hypoplastic left heart syndrome Other Dextrocardia · Levocardia · Cor triatriatum · Crisscross heart · Brugada syndrome · Coronary artery anomaly · Ventricular inversion heart navs: anat/physio/dev, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

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