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

This review presents the cardiac and non-cardiac malformations in 60 cases with asplenia and polysplenia with special reference to distinguishing factors which may be helpful in the clinical recognition of these syndromes. The asplenia cases were predominantly male and presented with cyanosis. They frequently had transposition of the great arteries (72%) with pulmonary stenosis or atresia (88%) and total anomalous pulmonary venous drainage (72%). Deaths were caused by cardiac failure and anoxia in 57 per cent of cases. Most of the patients died in the first year of life (79%), but longer survival is possible in the asplenia syndrome. The polysplenia cases were predominantly female and survived longer. The characteristic clinical findings were the relatively more benign presenting signs and the leftward or superiorly orientated P wave axis on the electrocardiogram. Conotruncal abnormalities were less common and total anomalous pulmonary venous drainage did not occur. On angiography the inferior vena caval drainage via the azygos system was clearly identified and this was present in all cases at surgery. Our study indicated that the cardiac anomalies in polysplenia were less severe than they were in asplenia and therefore the prognosis in the former syndrome is likely to be more favourable. Three families had two affects sibs but no single genetic factor could be identified. The aetiology of these syndromes remains undetermined.
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PMID:Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis. 119 45

Five patients of asplenia syndrome with pulmonary venous obstruction underwent TAPVC repair in the period of neonates (4 patients) and infant (1 patient). They also underwent associated procedures to reconstruct or adjust pulmonary blood flow (systemic-pulmonary shunts in 2, bilateral PDA banding in 1, pulmonary artery banding in 1) in accordance with individual anatomy of the pulmonary arteries. In four of them, they required subsequent surgical procedures for reduction of pulmonary blood flow because of intractable heart failure due to increase in pulmonary blood flow mostly at the early postoperative period. The increasing pulmonary blood flow was successfully controlled by early reduction procedures (1 patient: extrathoracically adjustable PA banding, 1 patient: tightening of shunt graft) in two patients. However, one patient died in the early postoperative period because of moribund preoperative condition. The remaining one patient underwent PA banding three month after the first operation, but died in late period due to heart failure. These results suggest that the adjustment of pulmonary blood flow is critically important for management of the patients of asplenia syndrome with TAPVC after the operation, and early decision of PA band re-adjustment (or other procedures to decrease pulmonary blood flow) is mandatory to improve the results.
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PMID:[Relief of pulmonary venous obstruction for asplenia syndrome associated with total anomalous pulmonary venous connection in neonates and infants]. 817 95

We reported a successful palliative operation for asplenia syndrome with total anomalous pulmonary venous return (TAPVR Ia) in an infant. The boy was suffering from cyanosis and tachypnea. He was diagnosed as asplenia syndrome with TAPVR and hiatus hernia. After he was admitted to our hospital, pulmonary congestion gradually progressed in a month. At 58 days of age, a palliative operation (repair of TAPVR and pulmonary artery banding with band of 20 mm in length) was performed. The postoperative course was uneventful. At 114 days of age, he underwent curative operation for hiatus hernia without cardiac failure. Postoperative cardiac catheterization at 179 days of age showed appropriate pulmonary artery pressure. We emphasize that pulmonary artery banding which is tighter than usual well controls pulmonary blood flow, although the length of the band in each case should be considered individually.
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PMID:[A case of successful palliative operation of asplenia syndrome with total anomalous pulmonary venous return in young infant]. 1047 51

The absence of a spleen is a well-known risk factor for severe bacterial infections, especially due to encapsulated bacteria. Congenital asplenia can be part of multiple congenital abnormalities as in heterotaxy including Ivemark syndrome with congenital anomalies of the heart or great vessels, or it can be isolated, which is extremely rare. In these cases, asplenia is an important factor effecting mortality. In this report, the clinical courses of five children with asplenia and concomitant minor or complex cardiac anomalies are presented. The ages of the children ranged between 1.5 and 17 months at the time of diagnosis. All of the cases had had a history of hospitalisation for infectious diseases before the diagnosis. The patient who was diagnosed at 17 months old had a history pneumonia, urinary tract infection, and bacterial meningitis beginning at five months old. Three children had complex cardiac anomalies, one child had ventricular septal defect, and one child had atrial septal defect. Howell-Jolly bodies were determined in peripheral blood smear in all of the patients. The diagnoses of asplenia were confirmed with spleen scintigraphy. One of the patients with complex cardiac anomalies died a short time after diagnosis, because of cardiac failure. The rest of the four patients were vaccinated for encapsulated bacteria and were taken under antibiotic prophylaxis. These children did not need hospitalisation for infectious diseases during the follow-up period (5-40 months). In asplenic children, early diagnosis, antibiotic prophylaxis, and immunisation for encapsulated bacteria can decrease the risk of morbidity and mortality.
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PMID:Asplenia in children with congenital heart disease as a cause of poor outcome. 2655 43