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[Endocardial fibroelastosis of the left ventricle in a patient with Alagille syndrome].

We present a case of a 6 week old infant with Alagille syndrome. Cardiological evaluation revealed peripheral pulmonary arterial stenosis and left ventricular endocardial fibroelastosis. While peripheral pulmonary arterial stenosis are typical for the syndrome this is the first case reported with endocardial fibroelastosis. Complex cardiac malformations may worsen the prognosis in Alagille syndrome. They require early diagnosis and therapy.

Biliary Atresia↗
PubMed1994

[Natural history and prognostic factors in primary endocardial fibroelastosis in infants].

Over a 33 year period, 127 patients under 2 years of age with dilated cardiomyopathies and appearances compatible with the diagnosis of primary endocardial fibroelastosis were admitted to the paediatric cardiac unit of the CHRU of Lille. The average follow-up was 8.9 +/- 6.7 years. Ninety-four children (74%) were cured, 16 (13%) had persistent cardiomegaly and/or left ventricular dysfunction on echocardiographic examination, and only 17 (13%) patients died (10 in the year following their initial hospital admission including 5 in the first week). The outcome of patients was not related to age at diagnosis, sex, cardiothoracic index, initial shortening fraction of the left ventricle or the period at which the patients were seen for the first time. On the other hand, the presence of a family history of cardiomyopathy was associated with a significantly worse prognosis. A recurrence of symptoms was the factor most closely correlated with a bad prognosis: 12 of the 19 patients (63%) with this evolution died, and 4 others (21%) had persistent myocardial dysfunction at the end of the study. These recurrences were often observed after premature withdrawal or after use of ineffective dosages of digitalis. In the authors' experience, dilated cardiomyopathy in neonates with clinical features of primary endocardial fibroelastosis is associated with a relatively high number of cures. Prolonged treatment with high doses of digitalis seems a determining prognostic factor.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiomyopathy, Dilated↗
PubMed1997

[Invasive aspergillosis. Description of a case in an infant with severe granulocytopenia and endocardial fibroelastosis].

Invasive Aspergillosis in a child with severe granulocitopenia and endocardial fibroelastosis. A case of invasive aspergillosis in a 3-month old baby is reported. At autopsy gross examination revealed the presence of whitish nodules in the liver, spleen, kidneys and suprarenal glands. A black eschar with surrounding erythema involved the left auricle. The heart showed dilated left ventricle with porcelain-like thickening of the endocardium. The histological section of the hepatic nodular lesions showed Aspergillus colonization, vascular invasion with hyphal forms and necrotic granulomatous lesions in the other sites. Histologically, the endocardium is markedly thickened and rich in connective tissue and elastic fibres. Examination of the bone-marrow showed poor cellularity with a striking reduction of cells of the neutrophil granulocyte series. Aspergillosis may complicate the course of the child in immunocompromised state. Granulocitopenia causes an insufficient delimitation of infections and allows metastatic diffusion of fungi, through blood-vessels invasion.

Agranulocytosis↗
PubMed1987

Peripartum congestive cardiomyopathy and endocardial fibroelastosis associated with ritodrine treatment. A case report.

Congestive cardiomyopathy from endocardial fibroelastosis occurred in a 24-year-old primigravida with a twin gestation and preeclampsia. The patient was taking ritodrine for premature labor. Cardiovascular evaluation should be performed during the course of ritodrine treatment, and no patient should be discharged if she does not have normal cardiovascular function.

Adult↗
PubMed1986

Inheritance of familial primary endocardial fibroelastosis.

Two related families with 15 children, seven of whom developed endocardial fibroelastosis (EFE) are described. Three of the children died during infancy, and the disease was confirmed in one of them at autopsy. The survivors, two sisters age 5 years and 15 months (Family A) and two sisters age 17 and 14 years (Family B), are now symptomless and show a decrease in left ventricular hypertrophy. The mode of inheritance of EFE in our two families appears to be either autosomal or X-linked dominant with reduced penetrance.

Carnitine↗
PubMed1990

Dilated and contracted forms of primary endocardial fibroelastosis: a single fetal disease with two stages of development.

Left ventricular endocardial fibroelastosis was diagnosed by echocardiographic scanning in a fetus at 20 weeks' gestation. Repeated prenatal examination over the next 20 weeks' gestation showed the development of the left ventricle from a chamber with a dilated cavity to a small cavity with a very thick wall. These findings were confirmed at necropsy and by the absence of other morphological anomalies. The contracted form of primary fibroelastosis in the fetus must have followed an insult that prevented any further increase in the size of the left ventricular cavity.

Adult↗
PubMed1987

Endocardial fibroelastosis in mucopolysaccharidosis type VI.

This case report describes two siblings less than 1 year of age who presented severely ill with a dilated cardiomyopathy. Full blood examination in both cases revealed marked granularity of neutrophils suggestive of mucopolysaccharidosis type VI. There were no physical features of a mucopolysaccharidosis but biochemical evaluation confirmed mucopolysaccharidosis type VI in both children. Autopsy in one patient confirmed endocardial fibroelastosis and electron microscopy of fibroblasts in the myocardium showed distention with membrane-bound vacuoles, consistent with a mucopolysaccharidosis. These siblings developed endocardial fibroelastosis before other clinical manifestations of the mucopolysaccharidosis. Assessment for metabolic causes of a cardiomyopathy is important, as cardiac disease may be the initial manifestation of a metabolic disease.

Endocardial Fibroelastosis↗
PubMed1983

[Unusual contrast medium distribution in the left ventricular myocardium in infants with a high degree of aortic valve stenosis and endocardial fibroelastosis. Pathologico-morphologic findings].

In 5 newborn infants with severe valvular aortic stenosis, left ventricular angiocardiography revealed conspicuous pathologic-morphologic findings consisting of a fine network of contrast medium opacification in the region of the posterior wall. Subsequently performed histologic examination of tissue removed from the involved regions showed, in addition to endocardial fibroelastosis, two structures which were assumed to be the morphologic substrates for the unusual distribution of contrast medium. On the one hand, antler-like spaces with a well-demarcated lining of fibroelastic tissue, similar to the thickened endocardium, were found in the inner half of the left ventricular wall connected to the lumen of the left ventricle through bottle-necked communications. On the other hand, oval or oblong lumina, substantially larger in caliber than capillaries but showing only a single endothelial layer could be demonstrated. With the sectioning technique employed, anastomoses of the latter spaces with the left ventricle or the previously mentioned system of spaces could not be established. The cause of the development or maintenance of these sinusoidal spaces may be attributable to the elevated diastolic left ventricular pressure, while the elevated systolic pressure may have been a decisive etiologic factor for the endocardial fibroelastosis. Whether the intratrabecular spaces represent persistent embryonic myocardial sinusoids cannot be differentiated unequivocally. In support of the stated contentions, however, are other results indicating that the extent of structural changes in the ventricular wall increases proportionately with the duration of existence of the obstructive lesion in the embryonic period.

Angiocardiography↗
PubMed2000

Secondary endocardial fibroelastosis associated with Pompe disease and multicystic dysplastic kidney.

An infant with secondary endocardial fibroelastosis (EFE) associated with glycogen storage disease II (Pompe disease) and multicystic dysplastic kidney (MCDK) is described. She had had refractory heart and renal failure from the early neonatal period. In spite of administration of cathecholamines and diuretics, ventilator support, and peritoneal dialysis, her heart failure due to reduction of left ventricular contractility progressively worsened. She died on the 40th day after admission. Histological examination of a left ventricular autopsy specimen showed prominent thickening of the endocardium due to fibroelastosis, and a lacework-like structure due to accumulation of glycogen in the cardiomyocytes. The EFE was derived from degeneration of the smooth muscle in the endocardium and cardiomyocytes due to glycogen storage. In addition, we supposed that the renal failure due to MCDK made the preload for the ventricles increase and accelerated her heart failure.

Endocardial Fibroelastosis↗
PubMed1991

Endocardial fibroelastosis with coronary artery thromboembolus and myocardial infarction.

We report a case of an 18-month-old male, born to a woman with third trimester febrile illness, who had a history of congestive heart failure and respiratory distress, cardiomegaly, and electrocardiographic (ECG) findings suggestive of cardiomyopathy and myocarditis. After gradual improvement in heart size and function with pharmacologic therapy, he developed a terminal episode of respiratory distress and cardiogenic shock, with ECG findings of an anterolateral infarct. At autopsy it was found that endocardial fibroelastosis with mural thrombi in the left ventricle had been complicated by thromboembolism to the left anterior descending coronary artery, resulting in transmural infarction of the anteroseptal region of the left ventricle. Myocardial infarction is a potential but unusual thromboembolic complication of endocardial fibroelastosis. A high index of suspicion for coronary artery thromboemboli should be maintained in pediatric patients with cardiomyopathy and suspected myocardial infarction.

Autopsy↗