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PubMed1977

Histologic and ultrastructural features of primary and secondary endocardial fibroelastosis.

The average size of elastic fibers in thickened left ventricular endocardium was much larger in four patients with congenital endocardial fibroelastosis (EFE) than in six patients with acquired EFE (secondary to ischemic heart disease in two patients, to prosthetic cardiac valves in three, and to irradiation of the chest in one). Both components of normal elastic tissue (central, amorphous cores, and peripheral microfibrils) were present in endocardial elastic fibers of each patient. Ultrastructural identification of elastic fibers was greatly facilitated by staining with silver tetraphenylporphin sulfonate.

Adult↗
PubMed2005

Fetal Doppler echocardiographic diagnosis and successful steroid therapy of Luciani-Wenckebach phenomenon and endocardial fibroelastosis related to maternal anti-Ro and anti-La antibodies.

BACKGROUND: Complete fetal heart block (HB) and endocardial fibroelastosis (EFE) are known to be associated with maternal anti-Ro and anti-La antibodies. Complete fetal HB is irreversible. OBJECTIVES: We sought to (1) assess the value of the superior vena cava/ascending aorta Doppler approach in the early detection of abnormal delay in the fetal atrioventricular (AV) time of conduction, before appearance of complete fetal HB; and (2) report the effect of prenatal steroid therapy on EFE, HB, or both. RESULTS: The clinical history, echocardiographic, and Doppler investigations of 3 fetuses and children born to mothers positive for anti-Ro and anti-La antibodies are reported. Two fetuses presented with EFE either isolated (29 weeks) or associated with AV block (25 weeks). In this last case, the superior vena cava/ascending aorta approach allowed the identification of a Luciani-Wenckebach phenomenon. In a third fetus, 2:1 AV block was noted at 23 weeks of gestation. Dexamethasone (4 mg/day) was administered to all 3 patients. Complete regression of the EFE and conduction abnormalities was documented in all cases. CONCLUSION: Early prenatal detection of abnormal delay in fetal AV time conduction is possible with the Doppler superior vena cava/ascending aorta approach. Steroid therapy can cure fetal EFE and AV conduction delays associated with maternal anti-Ro and anti-La antibodies.

Adult↗
PubMed2002

Maternal anti-Ro and anti-La antibody-associated endocardial fibroelastosis.

BACKGROUND: Maternal anti-Ro and anti-La antibodies are associated with congenital heart block (CHB). Although endocardial fibroelastosis (EFE) has been described in isolated cases of autoantibody-mediated CHB, the natural history and pathogenesis of this disease are poorly understood. METHODS AND RESULTS: We retrospectively reviewed the clinical history, echocardiography, and pathology of fetuses and children with EFE associated with CHB born to mothers positive for anti-Ro or anti-La antibodies at 5 centers. Thirteen patients were identified, 6 with a prenatal and 7 with a postnatal diagnosis. Six mothers were positive for anti-Ro and anti-La antibodies, and 7 were positive for anti-Ro antibodies only. Only 1 mother had autoimmune disease. Severe ventricular dysfunction was seen in all fetal and postnatal cases. Four fetal and 3 postnatal cases had EFE at initial presentation. However, 2 fetal and 4 postnatal cases developed EFE 6 to 12 weeks and 7 months to 5 years from CHB diagnosis, respectively, even despite ventricular pacing in 6 postnatal cases. Eleven (85%) either died (n=9) or underwent cardiac transplantation (n=2) secondary to the EFE. Pathologic assessment of the explanted heart, available in 10 cases, revealed moderate to severe EFE in 7 and mild EFE in 3 cases, predominantly involving the left ventricle. Immunohistochemistry in 4 cases (including 3 fetuses) demonstrated deposition of IgG in 4 and IgM in 3 and T-cell infiltrates in 3 cases, suggesting an immune response by the affected fetus or child. CONCLUSIONS: EFE occurs in the presence of autoantibody-mediated CHB despite adequate ventricular pacing. Autoantibody-associated EFE has a very high mortality rate, whether developing in fetal or postnatal life.

Adult↗
PubMedDate not supplied

Dilated endocardial fibroelastosis: unusual late finding with congenital hydrops.

The case of a baby born with severe nonimmune hydrops fetalis in whom endocardial fibroelastosis was a late finding is reported. Left ventricular dimensions and systolic function were normal at presentation. After recovery from the hydrops, at 2 months of age, a dilated, poorly contracting left ventricle was documented and eventually led to the infant's death.

Echocardiography↗
PubMed1980

Endocardial fibroelastosis, neurologic dysfunction and unusual facial appearance in two brothers, coincidentally associated with dominantly inherited macrocephaly.

We describe two brothers with endocardial fibroelastosis, unusual facial appearance, and cryptorchidism. The surviving brother has mental retardation, seizures and possible hypothalamic dysfunction. Both brothers have a head size greater than two standard deviations above normal; this appears to be related to superimposed presence of coincidental autosomal dominant macrocephaly in this family.

Cryptorchidism↗
PubMedDate not supplied

[Radiological and angiocardiographical aspects of primary endocardial fibroelastosis in children (author's transl)].

In this article we try to show the value of radiography and angiocardiography in primary endocardial fibroelastosis. The 20 CASEs studied in this report, were angiocardiographically and/or necropsically proved. In spite of different opinions, we think that it is possible to reach an approximate diagnosis of this condition if we use the clinical and angiohemodinamic examinations. On the other hand, it is necessary to point out that pathology is characteristic. The most important radiological signs were cardiomegaly, together with venocapilar congestion, both of variable magnitude, and dependent on the degree of cardiac insufficiency. By means of angiocardiography we could appreciate a great dilatation and hypertrophy of the left ventricle and a reduction of the mobility and distensibility of this cavity along the cardiac cycle.

Angiocardiography↗
PubMed1984

Endocardial fibroelastosis. Clinico-pathological study of 38 cases.

A clinico-pathological study was carried out based on 38 autopsies of patients with endocardial fibroelastosis ( EFE ): 82% of the cases were below the age of one year. The primary EFE was established in 21 (55%) of the cases, and in the last 17 (44%) it was associated with different congenital heart malformations (secondary EFE ). Different pathological conditions during pregnancy were present in 24% of the cases. Most often the left ventricle and atrium were involved, but in most of the cases the endocardium of the right cardiac chambers appeared by histological examination to be also affected. The varying morphological pictures of the heart suggest that EFE is a reaction of endocardium to different damaging factors.

Adult↗
PubMed1987

Endocardial fibroelastosis and tricuspid valve insufficiency in a calf.

A 5-month-old Holstein heifer had clinical signs of tricuspid valve insufficiency and histopathologic evidence of endocardial fibroelastosis. The calf had a 3-week history of weight loss, abdominal distention, dyspnea, and decreased appetite. Physical examination revealed signs of right-sided heart failure, and a systolic murmur (II/VI) was heard best over the right heart base. Results of cardiac catheterization and echocardiography indicated tricuspid valve insufficiency and right-sided heart failure. The calf was euthanatized after not responding to treatment with penicillin, furosemide, and removal of fluid from the thorax and abdomen. Necropsy findings included multifocal areas of thickening and opacification of the endocardium of the left and right ventricles. Excessive elastic fibers, consistent with fibroelastosis, were seen by use of special stains applied to sections of endocardium.

Animals↗
PubMed1975

[Endocardial fibroelastosis in infants observed at the children's ward of the County Hospital in Włocławek (author's transl)].

The authors have observed eight children suffering from endocardial fibroelastosis at the Children's Ward of the Ziemia Kujawska county hospital in Włocławek. The diagnosis was confirmed by autopsy. In five cases it was isolated fibroelastosis in the remaining three cases fibroelastosis was complicated by other heart malformations. In most of the patients severe symptoms appeared within the first six months of life. All the observed children died in the first two years of life. Death occurred within one day to six months after the appearance of symptoms. The main symptoms were present in ECG, X-rays and laboratory tests.

Adolescent↗
PubMed1978

Endocardial fibroelastosis: myocardial and vascular alterations associated with viral-like nuclear particles.

Although clinical, immunologic, and experimental evidence exists implicating in utero viral infection of the myocardium in the development of primary endocardial fibroelastosis, the infectious etiology of this condition remains somewhat controversial. To date, specific features of viral myocarditis and morphological demonstration of viral particles have not been described in EFE. The present case is the first in which extensive light microscopic and ultrastructural analysis of the myocardium revealed abnormalities consistent with a primary viral myocarditis associated with typical EFE. These alterations consisted of chronic myocardial inflammation, extensive interstitial fibrosis, severe degenerative changes in myocardial cells, and a marked proliferation of endothelial cells in large nad small intramyocardial vessels leading to vascular occlusions. In support of the infectious etiology of this disease, similar features were noted in skeletal muscle. Most interestingly, viral-like particles were observed in many myocardial and endothelial nuclei. Although we are not absolutely certain of the viral nature of these particles, their appearance suggests viral assoicated material. We propose that the presence of these particles in this case in association with the other morphological alterations in support for the viral etiology of EFE. The prominent vascular occlusion observed in the myocardium may be an important clue to the pathogenesis of fibroelastosis as either a primary or secondary disease.

Cell Nucleus↗
PubMed2002

Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block.

OBJECTIVES: This study was designed to document the association of endocardial fibroelastosis (EFE) and maternal autoantibodies. BACKGROUND: Neonatal lupus erythematosus is associated with the transplacental passage of maternal anti-Ro and anti-La antibodies, leading to complete atrioventricular block (CAVB). In some cases, CAVB is associated with EFE. Isolated EFE may be independently related to maternal anti-Ro and anti-La antibodies. METHODS: We identified three cases (one fetus and two infants, all female) of isolated EFE in infants born to autoantibody-positive mothers in the absence of CAVB. Demographics, echocardiograms, and pathology were reviewed. Immunohistochemical analyses for immunoglobulin (Ig)G, IgM, IgA, T-cell, B-cell, and terminal deoxynucleoleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) (test for cell apoptosis) staining were performed on multiple sections of the heart in each case and compared with negative controls. RESULTS: Two cases died and one received a cardiac transplant. All three cases had histologically confirmed EFE. All cases demonstrated significant diffuse IgG infiltration. To a lesser extent, myocardial tissue was also positive for IgM, CD43, and Granzyme B. None of the specimens were TUNEL positive. CONCLUSIONS: These are the first reported cases of isolated EFE associated with maternal anti-Ro and anti-La antibodies in the absence of CAVB. The diffuse deposition of IgG and the presence of a T-cell infiltrate throughout the myocardium suggest that the transplacental passage of maternal autoantibodies induces an immune reaction within the myocardium, leading to isolated EFE. Autoantibody-mediated EFE may be an etiologic factor in cases of fetal and neonatal "idiopathic" dilated cardiomyopathy.

Antibodies, Antinuclear↗