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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary hypertension is a rare complication of portal hypertension. Reports of childhood cases especially rare. This report describes an autopsy case of a 6-year-old boy with congenital biliary atresia followed by liver cirrhosis in whom severe hypertensive pulmonary arterial changes, including medial hypertrophy, intimal fibrosis and plexiform lesions were demonstrated. Fresh and organizing fibrin-platelet thrombi as well as probable organized thrombi with recanalization were occasionally found in the pulmonary vasculature, but it was thought that they had probably been formed locally as a late complication rather than being of thromboembolic origin. Retrospectively, the chest roentgenograms had revealed abnormalities suggestive of pulmonary hypertension since infancy, but the patient showed no apparent symptoms of it during life. Previously reported childhood cases of pulmonary hypertension associated with portal hypertension were briefly reviewed. Although the mechanism is presently not known, it is suggested that patients with portal hypertension, even in early childhood, are at risk of developing this unusual complication.
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PMID:Pulmonary hypertension associated with portal hypertension in childhood. Case report of a 6-year-old child and review of the literature. 305 10

Pulmonary hypertension is a rare but well-established complication of portal hypertension with a prevalence of 0.73% in one large autopsy series. In this report, we review the findings on chest radiographs in eight patients with this complication. Portal hypertension in these patients was evidenced by the presence of esophageal varices and/or ascites. The causes of portal hypertension were liver cirrhosis in seven patients and portal venous thrombosis in one patient. Pulmonary hypertension was established by right heart catheterization and pressure measurement. Qualitative assessment of the radiographs showed that four patients had the classic findings of pulmonary hypertension including prominent central pulmonary arteries and right ventricular enlargement, three had subtle abnormalities, and one had only cardiomegaly. Measurements of the width of the right descending pulmonary artery and the pulmonary lobar diameter/transverse thoracic diameter ratio were made in five of the patients in whom postero-anterior radiographs were available. The results confirmed our qualitative analysis, although they did not establish the diagnosis in borderline cases. We also observed that pulmonary vascular redistribution to the upper lobes was present in four patients, a finding that has been reported in patients with other causes of pulmonary hypertension. We conclude that the possibility of pulmonary hypertension should be raised in patients with portal hypertension, even when only subtle chest radiographic findings are present to suggest that diagnosis.
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PMID:Pulmonary hypertension complicating portal hypertension: findings on chest radiographs. 326 20

Two autopsy cases of pulmonary hypertension (PH) associated with liver cirrhosis are presented. Both patients were hepatitis B (HB) virus carriers and suffered from type B cirrhosis during the clinical course. The first patient was a 52-year-old male with type B cirrhosis. He died of hepatic encephalopathy but did not have any specific symptoms for PH except abnormal laboratory findings. Chest roentgenograms displayed prominence of the central pulmonary artery. Cardiac catheterization indicated marked increment of pulmonary arterial pressure. Autopsy revealed dilatation and sclerosis of the main pulmonary artery and right ventricular hypertrophy. Microscopically, the pulmonary arteries showed intimal fibrosis, medial hypertrophy, and plexiform lesions throughout the lungs. The second patient, a 15-year-old boy, had PH with juvenile liver cirrhosis which had existed for 8 years prior to the onset of PH. He complained of severe dyspnea and dizziness before death. Electrocardiogram indicated right ventricular hypertrophy. Autopsy disclosed cardiomegaly, type B cirrhosis and sclerotic pulmonary arteries. Grade VI pulmonary plexogenic arteriopathy including plexiform lesions and necrotizing arteritis was observed. HBsAg was detected in both the hepatocytes and the pulmonary arterial walls. We discuss the possible relationship between persistent HB viral infection and PH with liver cirrhosis.
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PMID:Pulmonary hypertension in hepatitis B virus carriers. 344 51

Patients with portal hypertension of varying etiology may develop pulmonary artery hypertension. In the present autopsy study, pulmonary and hepatic tissue was studied in 12 patients in whom pulmonary and portal hypertension coexisted. Plexogenic pulmonary arteriopathy was present in 10 patients, 7 of whom had coexistent thromboembolic lesions. One patient had isolated medial hypertrophy, which may be an early stage in the plexogenic category, whereas isolated thromboembolic pulmonary vascular disease was observed in one subject. Hepatic disease was consistent with alcoholic cirrhosis in seven patients, cryptogenic cirrhosis in four and extrahepatic portal hypertension without cirrhosis in one. Thrombocytopenia was present in all 10 patients whose platelet count was determined. This study suggests that pulmonary hypertension associated with portal hypertension commonly has a plexogenic appearance on histologic examination. However, thrombosis (whether embolic or in situ) may also contribute to vascular obstruction.
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PMID:Coexistent pulmonary and portal hypertension: morphologic and clinical features. 368 Jul 90

An autopsy case of pulmonary hypertension in a 29-year-old Japanese female with macronodular, posthepatic liver cirrhosis and hepatitis-B antigenemia was presented. No recognizable known cardio-pulmonary disease or portal thrombosis was obtained. Hepatitis-B antigen was demonstrated in the cirrhotic hepatocytes by a specific peroxidase antiperoxidase method. Characteristic pulmonary arterial changes including plexiform lesions with varying developmental stages were widely observed throughout the lungs. Complication of these two distinct disease processes seems to be rarely encountered in the literature. Discussion was focused on the possible interrelationship between the liver cirrhosis with hepatitis-B antigenemia and pulmonary hypertension. Proposed were presumptive underlying humoral, particularly immunological, abnormalities common to these diseases rather than mere incidental complications.
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PMID:Pulmonary hypertension associated with liver cirrhosis and hepatitis-B antigenemia. 634 Feb 45

Monocrotaline, a pyrrolizidine alkaloid derived from Crotalaria spectabilis, is known to be toxic to a variety of domestic and laboratory animals and to humans. Major pathological effects induced by monocrotaline poisoning include hepatic cirrhosis and megalocytosis, venocclusive disease, pulmonary hypertension, and right ventricular hypertrophy. The present investigation explored the structural and functional relationships that exist between pulmonary artery pressure, small pulmonary artery medial thickness, and right ventricular hypertrophy. The results of this physiological and histological study on monocrotaline-intoxicated rats has demonstrated that there is a positive correlation between progressive pulmonary hypertension, thickening of the medical wall of small pulmonary vessels, and right ventricular hypertrophy as a function of time.
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PMID:Changes in pulmonary structure and function induced by monocrotaline intoxication. 645 Nov 83

Histological changes of the pulmonary vasculature in comparison with that of the portal vein in necropsy cases of severe hepatic injury that had not been associated with clinical pulmonary hypertension were analysed qualitatively and quantitatively. The main changes of the intra-hepatic portal vein were dilatation (88.3 per cent), thickening of the wall (58.5 per cent) and thrombi (22.3 per cent). The changes of the pulmonary vasculature were limited to muscular type arteries, which showed dilatation (31.9 per cent), thickening of the wall (30.9 per cent) and thrombi (10.6 per cent). Association of wall thickening of the portal vein and the small pulmonary artery was observed in 30.9 per cent, these associations being statistically significant. Coincidence of thrombus formation in the portal vein and pulmonary vasculature was observed only in 4.3 per cent, which was not statistically significant. After the circumferences of the internal and external elastic laminae of small pulmonary arteries and the cross-sectional area of the media were measured, the anatomical radius and wall thickness of each artery were calculated according to Furuyama 's method. The relations between the anatomical radii and wall thickness of small pulmonary arteries in controls and hepatic cirrhosis groups were investigated by analysis of covariance to compare the equality of the two corresponding regression lines. The wall thickness and the ratio of wall thickness to the anatomical radius of small pulmonary arteries were significantly larger in the cirrhotic group than in the control group.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Histometrical investigation of the pulmonary artery in severe hepatic disease. 673 14

The finding of a rise in VD/VT or failure of VD/VT to fall during exercise has been proposed as a useful noninvasive indicator of pulmonary vaso-occlusive disease in patients with unexplained dyspnea. However, we previously reported a normal fall in VD/VT during exercise in patients with pulmonary hypertension at rest and/or during exercise due to collagen vascular disease. To investigate further the relationship between pulmonary vascular abnormalities and VD/VT responses to exercise, we studied 4 subjects with severe hypoxemia due to cirrhosis or diffuse telangiectasia. We found an abnormal VD/VT response to exercise in these subjects despite normal pulmonary hemodynamics which effectively excluded hemodynamically significant pulmonary vascular obstruction. These findings provide further support for the lack of utility of the VD/VT measurement at rest and during exercise as a screening method for detecting pulmonary vaso-occlusive disease, since the VD/VT cannot only fall appropriately during exercise in patients with pulmonary hypertension but can also remain unchanged or rise during exercise in patients with normal pulmonary hemodynamics.
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PMID:Abnormal responses of wasted ventilation fraction (VD/VT) during exercise in patients with pulmonary vascular abnormalities. 682 5

Marked pulmonary hypertension developed in a 40-year-old man with known cirrhosis and a previous portosystemic shunt. Terminally, he also showed signs of microangiopathic hemolytic anemia. At postmortem examination, he had severe plexiform dilatation lesions in the pulmonary vasculature, with deposition of fibrin in the vasculature channels. It is suggested that the site of microangiopathic red cell damage was the pulmonary microvasculature.
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PMID:Portal and pulmonary hypertension with microangiopathic hemolytic anemia. 685 60

Hypertensive pulmonary vascular disease secondary to chronic alveolar hypoxia is the result of vasoconstriction and then muscularization of the terminal portions of the pulmonary arterial tree. Ultrastructurally these two phases are characterized respectively by muscular evaginations and the hyperplasia of smooth muscle cells. Plexogenic pulmonary arteriopathy secondary to congenital cardiac shunts, primary pulmonary hypertension or rare cases of cirrhosis of the liver are characterized by concentric-laminar proliferation of myofibroblasts. Later there is a development of plexiform lesions which consist of vascular channels separated by a matrix of proteoglycan containing fibrillary cells and myofibroblasts. Fibrinoid necrosis results from the passage of fibrinogen from the vascular lumen into the arterial wall.
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PMID:Electron microscopy of hypertensive pulmonary vascular disease. 686 May 54


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