Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cases of acute cardiac failure which are apparently primary, and occurring in babies or young children have one characteristics clinical picture. There are three etiological agents, which cannot be differentiated clinically: myocarditis, fibroelastosis, and primary non-obstructive cardiomyopathies. When the critical early period, which still carries a high mortality, has been passed, the outlook is relatively favourable. 61 babies or infants of less than 30 months with acute primary cardiac failure were followed up for a period of between 5 and 20 years. 33 of them were considered cured; 22 have minor sequelae (most frequently) left ventricular hypertrophy on X-ray or ECG); 3 have persistent cardiac failure despite treatment; 3 have died after a period of more than 5 years. There is no clinical, X-ray or ECG finding which can predict the longterm outlook in this condition. It is virtually impossible to differentiate between myocarditis and firboelastosis; it seems likely in the majority of cases that we are dealing with different modes of development in the same condition.
 ...
PMID:[Apparently primary acute heart failure in infants. Long-term outcome]. 9 60

We report a case of aortic atresia with biventricular hypertrophy and death due to cardiac insufficiency (at 5 1/2 days of age). In the necropsy a normally sized left ventricle was observed, with endocardiac fibroelastosis, anomalous mitral arcade, intact interventricular septum and atresia of the aortic valve. The ascending aorta was moderately hypoplasic; there was a foramen ovale and persistent ductus arteriosus. We believe that this is the first case that has been reported of this anomaly occurring with a normal left ventricle and intact ventricular septum.
 ...
PMID:Aortic atresia with normal left ventricle and intact ventricular septum. 14 2

Two children with congenital fibroelastosis and recurrent episodes of heart failure had overt proteinuria and hematuria; one also had a reversible nephrotic syndrome. Urinary manifestations persisted during periods of cardiac compensation. Renal biopsies revealed mesangial hyperplasia by light microscopy, identical ultrastructural lesions in the glomerular basement membrane, and deposits of fibrin in one of the biopsy specimens studied by immunofluorscence. These changes detected by electron microscopy may result in an increase in glomerular permeability independent of the renal hemodynamic disturbances associated with cardiac insufficiency. The progression of the lesions appears to be slow, although urinary manifestations may simulate an intercurrent glomerulonephritis. Pulmonary hypertension and renal venous stasis with glomerular intravascular coagulation were discussed as possible pathogenic mechanisms.
 ...
PMID:Glomerular lesions in congenital endocardial fibroelastosis: clinical manifestations and ultrastructural studies in two patients. 58 Aug 81

On the basis of clinico-anatomical analysis of the causes of death in 35 patients following valve prosthetics for rheumatic valvular disease, it was established that cardiac failure was the most frequent cause. Cardiac failure was due to exacerbation of the carditis process on the one hand, and to disorders of the rhythm induced by dystrophy, sclerosis, fibroelastosis, and calcinosis in the cardiac septum on the other. Thromboembolic processes rate second in frequency among the lethal complications. Death from septic complications was rarely encountered, and from causal causes only in individual cases.
 ...
PMID:[Clinical anatomical basis of the pathogenesis of fatal complications in the late periods after heart valve prosthesis]. 59 19

Critical aortic stenosis (CAOS) is not compatible with life when the ductus arteriosus closes. We have treated 11 consecutive cases with isolated CAOS. Symptom presentation was in the early neonatal period and diagnosis was made noninvasively at a mean age of 4 days. All were operated on with transventricular dilation (TVD) at a mean age of 4.7 days. There was no early mortality. There were two late deaths due to fibroelastosis. Both had the smallest aortic anulus diameter (5 mm). Two other patients had aortic root replacement, one at the age of 6.5 weeks due to intractable heart failure, and the other at the age of 3 months due to increasing gradient. In these two cases elective surgery was made possible by a successful TVD in the early neonatal period. TVD in this material was not associated with any early mortality, which makes this procedure a good alternative in the treatment of CAOS.
 ...
PMID:Transventricular dilation for critical aortic stenosis in neonates. 140 43

On Nov 1, 1984, an orthotopic cardiac transplantation was performed in an 8-month-old female infant with subendocardial fibroelastosis. Because of the advanced state of cardiac failure, the operation was done despite a positive tissue crossmatch for antitoxic donor-specific antibodies. Immunosuppression consisted of high doses of cyclosporine (up to 550 mg/m2 or 30 mg/kg) and steroids. Hypertension and tremor of the extremities, which were attributed to cyclosporine, occurred during the first week but resolved after seven days. No signs of nephrotoxic effects have been noted; however, a severe episode of allograft rejection was detected by endomyocardial biopsy on the seventh postoperative day, and a moderate rejection episode was noted on the 22nd postoperative day. Histologic improvement was seen after treatment with conventional steroid pulses. The patient was discharged on Nov 29, 1984. Complications consisted of four episodes of otitis media caused by Staphylococcus aureus and one rejection episode that was treated on an outpatient basis with an intravenous methylprednisolone sodium succinate pulse. Our experience emphasizes both the feasibility and importance of performing endomyocardial biopsies in infant recipients. Through biopsy, episodes of rejection can be discovered when clinical signs are not yet apparent. Eighteen months after transplantation, the child was developing and growing normally.
 ...
PMID:Cardiac transplantation in an 8-month-old female infant with subendocardial fibroelastosis. 352 37

We report a pedigree in which six males died of cardiac failure within the first eight months of life. These males were related through healthy females, as with X linked recessive inheritance. There was no consanguinity. None of the affected boys had an anatomical cardiac abnormality. In two affected brothers, histological evidence for endomyocardial fibroelastosis was documented, and in one of these electron microscopy demonstrated abnormalities of the mitochondria as found in mitochondrial cytopathy. A review of published reports revealed five similar X linked pedigrees, and in two of these mitochondrial abnormalities were found. We suggest that these families may show an X linked recessive cardiomyopathy with mitochondrial abnormalities.
 ...
PMID:Endocardial fibroelastosis: possible X linked inheritance. 358 35

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.
 ...
PMID:Endocardial fibroelastosis and tricuspid valve insufficiency in a calf. 361 Jul 56

A diagnosis of fibroelastosis was made in a 33-week-old fetus, based on the echocardiographic features of a marked impairment of left ventricular contractility and hyperechogenicity of the endocardium. Fibroelastosis was associated with an aortic stenosis. There was no evidence of hydrops fetalis. Prenatal detection of this severe congenital heart disease allowed its early management in an intensive care unit. Heart failure due to closure of the ductus required the use of prostaglandin, then a surgical aortic valvulotomy was performed. There was no postoperative problem, and one year after surgery the infant is doing well. His left ventricular contractility is normal, and echocardiographic features of fibroelastosis are no more present. Prenatal diagnosis, use of prostaglandin and increasing safety of neonatal cardiac surgery have improved the prognosis of this serious association.
 ...
PMID:[Endocardial fibroelastosis: prenatal manifestation of aortic valve stenosis]. 372 98

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.
 ...
PMID:Secondary endocardial fibroelastosis associated with Pompe disease and multicystic dysplastic kidney. 1156 Mar 61


1 2 Next >>