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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 association of portal hypertension, although its etiology is unclear. We report the development of severe "primary" pulmonary hypertension after an end-to-side portacaval shunt in a woman with postnecrotic cirrhosis.
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PMID:Primary pulmonary hypertension after portocaval shunt. 688 59

A 62-year-old white woman was found to have pulmonary hypertension; a short time later hepatic insufficiency developed. At the time of autopsy, hepatic cirrhosis, "primary" pulmonary hypertension, and evidence of increased iron absorption and storage were found. Pulmonary hypertension is an unusual complication of portal hypertension that is being reported increasingly often.
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PMID:Pulmonary hypertension. Its association with hepatic cirrhosis and iron accumulation. 689 45

The present report deals with the clinical history and autopsy findings of a 41 year-old male patient with hepatic cirrhosis, surgical splenorenal shunt and severe pulmonary hypertension. Since the original description of Mantz and Craige several series have been reported, both in children and in adults, of the association between pulmonary hypertension and liver disease and/or portal hypertension. However, the type of hepatic alteration associated to pulmonary hypertension and the mechanisms responsible for this association remain unclarified. In the present case prominent plexiform and/or angiomatoid lesions of the pulmonary vasculature were observed, prompting the authors to speculate on the possibility that non-identified vasoconstrictor substances might contribute to the production of morphological lesions in the arterio-venous pulmonary shunts.
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PMID:[Hepatic cirrhosis and primary pulmonary hypertension. A case report (author's transl)]. 725 48

Pulmonary arterial hypertension with severe pulmonary vascular disease is a rare association of portal hypertension in man, be it the result of cirrhosis of the liver or obstruction of the portal vein. We induced portal hypertension in 23 rats by partially ligating the portal vein or by totally occluding it in two stages. The rats were killed between one and 15 months after operation. A collateral circulation of varicose, anastomotic vessels was established, and in six animals well-marked oesophageal varices developed. Despite this evidence of sustained portal hypertension, hypertrophy did not develop in the right ventricle or in the media of the pulmonary trunk or muscular pulmonary arteries in any of the animals. This suggests that mechanical obstruction of the portal vein per se is not responsible for the development of pulmonary hypertension. Other factors, perhaps of a humoral nature, appear to be required to induce this rare association of portal hypertension but we have been unable to identify these. In particular, blood levels of oestrogen were not raised after ligation of the portal vein.
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PMID:Observations on the rare association between portal and pulmonary hypertension. 726 72

The clinical and pathologic findings are reported in seven consecutive patients with progressive and fatal pulmonary hypertension which was not explained by predisposing cardiac or pulmonary diseases. Pulmonary arterial lesions consisted of atherosclerosis of the elastic pulmonary arteries, and medial hypertrophy and concentric laminar fibrosis of the muscular pulmonary arteries in seven patients, plexiform lesions in six patients and necrotizing vasculitis in one patient. Pulmonary emboli were not identified. Five patients had manifestations of autoimmune disease, including laboratory abnormalities (positive antinuclear antibody, positive latex agglutination for rheumatoid factor, hypergammaglobulinemia or antimitochondrial antibody) in four, necrotizing vasculitis in one, Raynaud's phenomenon in two and clinical evidence of multisystem collagen vascular disease in two. Five patients had liver disease which developed prior to or concomitant with the onset of pulmonary hypertension. At autopsy, one patient had prominent periportal fibrosis and four had macronodular (postnecrotic) cirrhosis (active in three and inactive in one). Four of these five patients with liver disease and pulmonary hypertension had evidence of autoimmune phenomena. The findings in the seven patients suggest an association between autoimmune disease, plexogenic pulmonary hypertension and liver disease of the chronic active hepatitis-postnecrotic cirrhosis type.
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PMID:Severe pulmonary hypertension associated with macronodular (postnecrotic) cirrhosis and autoimmune phenomena. 742 41

Despite much interest in plexiform lesions, no published work compares their distribution in different types of pulmonary hypertension. Scattered reports of plexiform lesions in bronchial arteries oppose the consensus view that the lesions develop in pulmonary arteries. To compare the localization of plexiform lesions in different types of pulmonary hypertension, and to assess the role of the bronchial arteries in their formation, we examined by light microscopy lung tissue from five patients with primary plexogenic pulmonary arteriopathy (PPPA), six with pulmonary hypertension secondary to congenital cardiac malformations (CCM), and one with pulmonary hypertension complicating hepatic cirrhosis. We classified the 270 plexiform lesions observed as either preacinar or intra-acinar based on the type of pulmonary artery in which they were located, and computed the frequencies of each type of lesion within each etiologic group. We searched for lesions developing in bronchial arteries. Then, postulating that a close anatomic relationship between plexiform lesions and bronchial arteries would necessitate a clustering of the lesions near sites in the lung subserved by the bronchial circulation, we measured, for 211 of the 270 lesions previously classified, the distance from the lesion to the nearest airway and computed the mean lesion-to-airway distance in each etiologic group. The frequencies of preacinar plexiform lesions were 34% in PPPA, 67% in CCM (P < .01), and 21% in the case of cirrhosis. We found no plexiform lesions within bronchial arteries, and the mean plexiform lesion-to-airway distances were 1,680 +/- 180 microns in PPPA, 1,330 +/- 220 microns in CCM, and 2,050 +/- 1,090 microns in cirrhosis (P > .05). Our data suggest that (1) the distribution of plexiform lesions within the pulmonary arterial tree varies depending on the etiology, (2) plexiform lesions rarely if ever arise in bronchial arteries, and (3) plexiform lesions are not preferentially distributed near parts of the lung subserved by the bronchial circulation.
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PMID:Different distribution of plexiform lesions in primary and secondary pulmonary hypertension. 767 99

Nitric oxide was identified as the relaxing factor derived from the endothelium in 1987. Nitric oxide synthesis allows the vascular system to maintain a state of vasodilation, thereby regulating arterial pressure. Nitric oxide is also found in platelets, where it inhibits adhesion and aggregation; in the immune system, where it is responsible for the cytotoxic action of macrophages; and in the nervous system, where it acts as neurotransmitter. A deficit in endogenous synthesis of nitric oxide contributes to such conditions as essential arterial hypertension, pulmonary hypertension and heart disease. An excess of nitrous oxide induced by endotoxins and cytokinins, meanwhile, is believed to be responsible for hypotension in septic shock and for hyperdynamic circulatory state in cirrhosis of the liver. Nitric oxide has also been implicated in the rejection of transplanted organs and in cell damage after reperfusion. Inhaled nitrous oxide gas reduces pulmonary hypertension without triggering systemic hypotension in both experimental and clinical conditions. It also produces selective vasodilation when used to ventilate specific pulmonary areas, thereby improving the ventilation/perfusion ratio and, hence, oxygenation. Nitric oxide inhalation is effective in pulmonary hypertension-coincident with chronic obstructive lung disease, in persistent neonatal pulmonary hypertension and in pulmonary hypertension with congenital or acquired heart disease. Likewise, it reduces intrapulmonary shunt in acute respiratory failure and improves gas exchange. Under experimental conditions nitric oxide acts as a bronchodilator, although it seems to be less effective for this purpose in clinical use.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Nitric oxide]. 789 26

Gut-liver-lung axis hypothesis in the pulmonary hypertension complicated with the cirrhosis, that some humonal factors escaping the hepatic inactivation caused by the liver cirrhosis were responsible for the changes in the lung vessels and caused the pulmonary hypertension, was set up. To determine the relation between the pulmonary hypertension and the liver cirrhosis, the author studied experimentally the hemodynamics and the pathological changes in the lung in the cirrhotic rats induced with carbon tetrachloride and in the rats with the portacaval anastomosis. Pathologically the intimal and medial thickness of the small lung arteries were created in both grous. The right ventricular systolic pressure in the cirrhotic rats (25.6 +/- 1.4 torr) was higher than that of the rats with the portacaval anastomosis (21.3 +/- 2.0 torr), but the rate of the extrahepatic portasystemic shunt in the cirrhotic rats (3.3 +/- 1.2%), which was measured with using gamma-labelled microsphere, was conversely lower than that in the rats with the portacaval shunt (17.8 +/- 3.9%). The same pathological changes were also created in another cirrhotic rats induced with dimethylnitrosamine. The right ventricular systolic pressure increased as the liver function became severe. It was suggested that not only the extrahepatic portasystemic shunt but also the dysfunction of the reticuloendothelial system in the liver, was responsible for the mechanism of the pulmonary hypertension in the cirrhosis.
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PMID:[Experimental study on changes of the pulmonary vessels in the cirrhotic rats]. 789 30

This paper reviews the effects of pulmonary artery hypertension on gas exchange by exploring three different issues, namely: 1) how does gas exchange behave in diseases characterized by increased vascular tone (primary pulmonary hypertension (PPH), chronic obstructive pulmonary disease (COPD) and interstitial pulmonary fibrosis (IPF)) or decreased vascular tone ("hepatopulmonary syndrome"); 2) how does exercise, as a non-pharmacological tool of increasing pulmonary blood flow, modify gas exchange in these diseases; and 3) how do several drugs that lower (vasodilators) or increase (almitrine) the active component of pulmonary hypertension interact with gas exchange. Available data show that: 1) in PPH a high pulmonary vascular tone enhances gas exchange and when it is lowered, either by oxygen or vasodilators, ventilation perfusion (VA/Q) distributions deteriorate; 2) in COPD a lowered (vasodilators) or augmented (almitrine) active vascular tone is almost invariably paralleled by a deterioration or enhancement of ventilation-perfusion matching, respectively; 3) in IPF an adequate active response of the pulmonary vasculature is essential to maintain gas exchange, both at rest and during exercise; and 4) in patients with liver cirrhosis a low pulmonary vascular tone induces an abnormal VA/Q distribution. In summary, these data show that any situation and/or therapeutic intervention that lowers the active vascular tone deteriorates VA/Q relationships and vice versa. The final effect of pulmonary vascular tone on arterial oxygen tension (PaO2) is less predictable. The reason for this uncertainty is that the actual PaO2 value depends on the interplay of the intra- and extrapulmonary factors that control gas exchange in humans, and not only on the degree of VA/Q mismatching.
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PMID:Effect of pulmonary hypertension on gas exchange. 828 57

We report an uncommon mechanism of severe hypoxemia in two cirrhotic patients under long-term beta-blocker therapy. Our patients presented with profound hypoxemia refractory to oxygen therapy, normal lung radiography and pulmonary function tests, and evidence of right-to-left anatomic shunt. Although these features are highly suggestive of hepatopulmonary syndrome, pulmonary hypertension was present, and a right-to-left shunt through a patent foramen ovale was demonstrated by contrast-enhanced echocardiography. No cause of pulmonary hypertension other than portal hypertension was identified. Pulmonary hypertension and intracardiac right-to-left shunt eventually regressed after discontinuation of beta-blocker therapy. We conclude that "primary" pulmonary hypertension associated with portal hypertension may because of severe hypoxemia during liver cirrhosis. Differential diagnosis of hepatopulmonary syndrome relies upon contrast-enhanced echocardiography and may be of critical importance because of possible therapeutic implications.
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PMID:Refractory hypoxemia during liver cirrhosis. Hepatopulmonary syndrome or "primary" pulmonary hypertension? 863 May 62


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