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PubMed2000

[Contracted endocardial fibroelastosis in children: report of a case].

Endocardial fibroelastosis is un uncommon disease and it has a very bad prognosis since fatal evolution is usual before 2 years old. We report the case of a 20 years old woman who is affected with the contracted form of this disease associated with atrial septal defect (ASD) and mitral regurgitation. This disease was discovered by endocardial biopsy when she was 4 years old and underwent surgical resection of endocardial fibrosis, a patch on the ASD and mitral valve replacement. She was rehospitalised 15 years later with heart failure although continuous digitoxin therapy.

Adult↗
PubMed1984

[Dilated cardiomyopathy in childhood: intravital differentiation of endocardial fibroelastosis using transvascular endomyocardial biopsy].

Possibly due to the lack of clinical reports concerned with dilated cardiomyopathy in childhood, pediatric cardiologists may inadvertently designate the angiographic finding of a dilated left ventricle as endocardial fibroelastosis. We report historical and noninvasively-obtained data from twelve children, aged seven months to 17 years, with poorly-functioning dilated left ventricles, in whom no differentiation between the two diseases was enabled. Only by means of transvascular endomyocardial biopsies, performed during cardiac catheterization with a 6-F guide-catheter and a Machida bioptome for light and electron microscopal examination, was differentiation achieved among six patients with endocardial fibroelastosis and six with the nonspecific histological findings of muscular hypertrophy, interstitial fibrosis and marked degenerative changes. After exclusion of noxious and inflammatory etiologies, even in childhood, the diagnosis of primary dilated cardiomyopathy must be regarded as established. Since experience previously reported with endocardial fibroelastosis has not been based on histologic documentation of the diagnosis, questions with respect to natural history and prognosis remain unanswered.

Adolescent↗
PubMed1994

Endocardial fibroelastosis in common domestic cats in the UK.

Endocardial fibroelastosis (EFE) is a pathological disorder characterized by a diffuse and elastic thickening of the endocardium. It is often thought to be congenital as most victims are infants who die of congestive heart failure before reaching one year of age. A naturally occurring animal model of EFE was proposed more than a decade ago on the basis of one pair of adult pure-bred Burmese cats which produced offspring consistently affected with EFE. In the present study a case of EFE is described which occurred in a closed colony of experimental SPF cats. A 5-week-old kitten which was found dead showed hydrothorax, hydropericardium and oedematous lungs at necropsy. Histological examination of the heart revealed left atrio-ventricular dilation with severe, diffuse endocardial thickening which did not extend into the myocardium. A retrospective study was carried out on stored tissues of two further kittens and one adult male cat from the colony which had died during a period of 2 years. The hearts of all the animals showed endocardial thickening in the left atria and ventricle but not in the right chambers. Over a period of 12 years of existence of this colony considerable inbreeding had occurred and it is concluded that an inherited factor was responsible for the disorder.

Animals↗
PubMed1998

Can echocardiography predict the presence or absence of endocardial fibroelastosis in infants <1 year of age with left ventricular outflow obstruction?

This study assesses the use of echocardiography in detecting endocardial fibroelastosis in 32 infants with obstructive left-sided lesions. The data demonstrate that neither endocardial echo-brightness nor geometric variables of the left ventricle allow for accurate prediction of the presence of endocardial fibroelastosis.

Echocardiography, Doppler↗
PubMed2001

[Symptoms of primary endocardial fibroelastosis in a young adult].

HISTORY AND ADMISSION FINDINGS: A 21-year-old woman with known endocardial fibroelastosis diagnosed when aged 3 months was admitted because of progressive dyspnoea. The physical examination revealed symptoms of heart failure, with pulmonary rales, mild hepatomegaly, and tachyarrhythmia. INVESTIGATIONS: The electrocardiogram showed atrial fibrillation, complete right bundle branch block and right ventricular hypertrophy. Echocardiography indicated hypertrophy and dilatation of the right ventricle (61 mm) with tricuspid regurgitation and hypoplasia of the left ventricle. Heart catheterization confirmed pulmonary hypertension (60/46 mmHg) as well as dilatation and hypokinesia of the right ventricle. Right ventricular biopsy showed severe myocardial hypertrophy resulting from secondary pulmonary hypertension, while no evidence of myocarditis or idiopathic dilated cardiomyopathy was found. TREATMENT AND COURSE: Symptoms of heart failure improved under medical treatment with digitalis, angiotensin-converting enzyme inhibitor and diuretics. CONCLUSION: Primary endocardial fibroelastosis of the contracted type must be included in the differential diagnosis of heart failure occurring in young adults.

Adult↗
PubMed1986

Assessment of cardiac performance by first pass radionuclide angiocardiography in infants and children with normal heart and endocardial fibroelastosis.

Peak to peak time from the right and to the left ventricle (PPT), left ventricular ejection fraction, left ventricular peak ejection rate and left ventricular peak filling rate were measured by first pass radionuclide angiocardiography in 27 infants and children with normal heart and in 8 patients (18 studies) with endocardial fibroelastosis. In normal subjects, the PPT significantly correlated with heart rate (r = -0.87, p less than 0.001). A PPT corrected by the heart rate (cPPT) was calculated by rotation of the regression equation relating the variables: cPPT = PPT + 0.018 X (heart rate)-2.1. The cPPT averaged 3.0 +/- 0.0 (mean +/- S.E.) sec. Consequently, there was no significant correlation between the cPPT and heart rate, but the cPPT and body surface area are significantly correlated (r = 0.41, p less than 0.05). Left ventricular ejection fraction, peak ejection rate and peak filling rate averaged 68 +/- 2%, 4.2 +/- 0.3/sec and 4.8 +/- 0.3/sec, all of which were independent of the heart rate and body surface area. In patients with endocardial fibroelastosis, the cPPT was prolonged (4.7 +/- 0.4 sec), and left ventricular ejection fraction, peak ejection rate and peak filling rate were all reduced (28 +/- 4%, 1.7 +/- 0.2/sec and 1.8 +/- 0.2/sec). These results indicate that parameters obtained from the radionuclide angiocardiography are useful for evaluating cardiac performance in patients with endocardial fibroelastosis.

Child↗
PubMed1995

New syndrome of hydrocephalus, endocardial fibroelastosis, and cataracts (HEC syndrome).

We report on two unrelated male infants with similar findings of communicating hydrocephalus, endocardial fibroelastosis (EFE) and congenital cataracts, who died at 4 months of age. Both mothers reported an upper respiratory infection during the first trimester of pregnancy which was further complicated by polyhydramnios in the third trimester. The infants were diagnosed with bilateral congenital nuclear cataracts at birth. Serologic tests for toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus, syphilis, and galactosemia screens were negative. Chromosome analyses were normal. Both children developed communicating hydrocephalus between one and three months after birth. Patient 1 died suddenly at 4 months following an upper respiratory infection. Patient 2 developed congestive heart failure and also died at 4 months. At autopsy, both infants had enlarged hearts with endocardial fibroelastosis. No identifiable organism could be isolated. We discuss the association of birth defects in widely separated organ systems in these patients and suggest that this may represent a genetic syndrome; however, a viral etiology cannot entirely be excluded. We believe this is a distinct disorder and propose the acronym HEC for hydrocephalus, EFE and cataracts.

Abnormalities, Multiple↗
PubMed1993

[Diagnosis and treatment of infantile endocardial fibroelastosis].

The diagnosis, treatment and etiology of primary infantile endocardial fibroelastosis were studied in 33 patients from 1989 to 1992. Two dimensional echocardiography with Doppler examination showed that the thickness of the endocardium obviously increased and systolic and diastolic functions of the left ventricles were abnormal. Endomyocardial biopsy proved that the endocardium and myocardium under went pathological changes. The virus probe examination of biopsy tissue showed negative results and the effectiveness of immunosuppressive therapy (total effective rate 96%) indicated that this disease is probably of autoimmune etiology. The facts led us to nominate this disease as infantile endomyocardial disease. Besides, the classification, diagnostic criteria, principles of treatment and prognosis of this disease were discussed.

Child, Preschool↗
PubMed1988

Contracted form of primary endocardial fibroelastosis in a young adult without congestive heart failure.

A case of a 24-year-old man with the contracted form of primary endocardial fibroelastosis diagnosed by left ventricular endomyocardial biopsy showing a markedly thickened endocardium with fibroelastic proliferation is reported. He had no evident symptoms of congestive heart failure except for shortness of breath on moderate exertion. Echocardiogram showed thickened and dense echoes from the left side of the septum and from the posterior left ventricular endocardium. Hemodynamic and angiographic studies revealed marked elevation of right and left ventricular end-diastolic pressures with dip and plateau pressure contours, moderate pulmonary hypertension, left atrial enlargement and mild mitral regurgitation. Further elevation of right and left ventricular diastolic pressures and pulmonary artery pressure was observed at the second evaluation after 5 years. Our patient suggests that primary endocardial fibroelastosis should be included in the differential diagnosis of adult patients with obscure types of cardiac disease.

Adult↗