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

Follow-up data for 81 consecutive patients with primary pulmonary hypertension (PPH) and pulmonary hypertension related to connective tissue diseases or liver cirrhosis, entered into a multicenter registry in Berlin between 1/96 and 11/99, are described. At entry into the registry the diagnosis of PPH was known for 22 +/- 32 months. Hemodynamically, these patients were characterized by a right atrial pressure of 8.6 +/- 5 mmHg, a mean pulmonary arterial pressure of 58.9 +/- 17 mmHg, a cardiac index of 1.8 +/- 0.6 l/min/m2 and a pulmonary vascular resistance of 1574 +/- 787 dyn x s x cm-5. In about one third of the patients, a restrictive and/or obstructive pulmonary physiology was found while the majority showed signs compatible with small airway disease. Furthermore, diffusion abnormalities were found in about 65% of the patients. When added to conventional medical therapy the treatment with inhaled or continuously infused prostanoids represents a major improvement in the treatment of patients with PPH. Aerosolized iloprost therapy was started in 51 patients and was continued for 12 and 24 months in 20 and 6 patients, respectively. This therapy was well tolerated without any significant changes in pulmonary function or signs of toxicity. About 25% of these patients had to be switched to continuous intravenous therapy due to progressive clinical and hemodynamic deterioration. About two thirds of these "rescue patients" could be stabilized on intravenous therapy and discharged from the hospital. Based on the currently available evidence, the continuous infusion of prostanoids represents an important part of the standard therapy in the treatment of patients with PPH. These first long-term data on inhaled iloprost therapy in this patient group illustrate the potential value of this well-tolerated and effective treatment within the concept of a differentiated treatment plan for patients with PPH. However, the true importance of prostanoid inhalation in comparison to continuous intravenous therapy in PPH remains to be determined in randomized controlled trials.
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PMID:[Iloprost as inhalational and intravenous long-term treatment of patients with primary pulmonary hypertension. Register of the Berlin Study Group for Pulmonary Hypertension]]. 1114 75

The relationship between grade of pulmonary hypertension and factors associated with human immunodeficiency virus among patients with HIV infection is poorly documented. This report documents the most extensive attempt made thus far to determine whether a relationship exists between degree of pulmonary hypertension and the following conditions: HIV risk factor, degree of immunosuppression, presence or absence of AIDS, and presence or absence of liver cirrhosis. A retrospective study involving a search of the published literature on primary pulmonary hypertension among HIV cases from 1987 to 1998, using the Medline and Aidsline databases was conducted. Patients for whom secondary causes of pulmonary hypertension could be excluded were selected, and the following information for each was recorded: age, gender, risk factors for HIV infection, HIV disease stage according to the Centers for Disease Control, previous opportunistic and neoplastic diseases, CD4+ cell count (cells/L), presence or absence of liver cirrhosis, pulmonary systolic artery pressure level, and lung pathology specimens. Information about the patient's survival time was also recorded. Seventy-six patients were judged to have primary pulmonary hypertension and were included in the study. While no correlation was found between pulmonary systolic artery pressure level and CD4+ cell counts, a statistically significant difference was found between HIV-positive patients with and without AIDS as determined by the Centers for Disease Control criteria with regard to the degree of pulmonary hypertension, expressed as pulmonary systolic artery pressure level (85.4 +/- 17 mm Hg vs 71.8 +/- 15 mm Hg, p < 0.013). Although a higher PAPS was present in HIV cirrhotic patients, a statistically significant difference was not found between degree of pulmonary hypertension and evidence of hepatic cirrhosis (85 +/- 21 mm Hg vs 73.1 +/- 15 mm Hg, p < 0.062). Patients with AIDS and primary pulmonary hypertension present a higher degree of pulmonary hypertension than non-AIDS patients. Pulmonary hypertension associated with HIV seems to be related to a cytokine-related stimulation and proliferation of endothelium. High levels of cytokines present in AIDS patients can favor pulmonary hypertension, but the role of a host response to HIV--determined by one or more HLA subtypes--is suspected to enhance high cytokine production levels.
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PMID:Primary pulmonary hypertension in HIV patients: a systematic review. 1120 29

Portopulmonary hypertension is now recognized as one of the pulmonary complications of chronic liver disease. However, previous studies reported that the incidence ranged from 0.25% to 2%, excluding fortuitous coincidence. In this study, we aimed to determine the variant hemodynamic and clinical features of portopulmonary hypertension in an area with a high prevalence of viral cirrhosis. After reviewing the hemodynamic data of 322 patients with portal hypertension admitted to the Taipei Veterans General Hospital between 1987 and 1999, we found 10 with portopulmonary hypertension. The overall incidence was, therefore, 3.1% in all patients with portal hypertension. Most of the patients with portopulmonary hypertension experienced exertional dyspnea. The survival times ranged from 2 to 86 months. In our series, most of the patients who died, died of complications related to cirrhosis and portal hypertension, but not of complications related to pulmonary hypertension. This study suggested that portopulmonary hypertension was not a frequent complication in cirrhotic patients and was not associated with an adverse outcome.
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PMID:Portopulmonary hypertension: distinctive hemodynamic and clinical manifestations. 1129 81

The role of the cardiac catheterization for diagnosis and treatment of pulmonary hypertension (PH) is very important. When mean pulmonary artery pressure increased more than 25 mmHg, then PH is defined. But this is measured accurately only by the catheterization. And we can discriminate the etiology of PH clearly by pulmonary capillary wedge pressure (Ppcw) or intra-cardiac shunt (L to R) by blood oxygen saturation step-up, and both parameters are obtained by this method. The etiology of PH is diagnosed as left sided heart failure, if Ppcw is increased more than 13 mmHg. PH is produced by congenital heart disease (ASD, VSD, PDA etc.), when the oxygen saturation step-up is recognized. And PH is induced by any pulmonary disease or pulmonary thrombo-embolism or collagen disease or liver cirrhosis or PPH, if Ppcw is normal and no oxygen step-up is recognized.
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PMID:[The role of cardiac catheterization for diagnosis and treatment of pulmonary hypertension]. 1141 Nov 19

Portopulmonary hypertension is a condition with a poor prognosis, which is defined as precapillary pulmonary hypertension complicating portal hypertension mainly due to cirrhosis of various etiologies. A mean pulmonary arterial pressure greater than 25 mmHg at rest with a pulmonary capillary wedge pressure less than 15 mmHg and a pulmonary vascular resistance greater than 120 dynes.sec.cm-5, in the setting of the presence of portosystemic shunting has been proposed as hemodynamic criteria for portopulmonary hypertension. Prevalence of pulmonary hypertension ascertained by right cardiac catheterization was 2% among patients with cirrhosis, and reached to 4% particularly among candidates for liver transplantation. Hyperdynamic systemic circulation seen commonly in patients with cirrhosis appeared to be normalized by complication of pulmonary hypertension with a contraction of circulating plasma volume. Long term treatment by epoprostenol administration or nitric oxide inhalation could induce a gradual decline in pulmonary arterial pressure in patients with poor response to acute vasodilator administration.
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PMID:[Pulmonary hypertension complicating portal hypertension: portopulmonary hypertension]. 1141 Nov 34

We report the case of a patient with liver cirrhosis who was admitted to the emergency room for rapid occurrence of dyspnea and severe hypoxemia at rest. Lung CT-scan and echocardiography did not disclose any right-to-left shunt and right-sided heart catheterization evidenced major precapillary pulmonary hypertension. The present feature supports the hypothesis that the pulmonary complications of cirrhosis, the hepatopulmonary syndrome and the portopulmonary hypertension, which are usually considered as mutually exclusive, may coexist. In such circumstances, the right failing heart is the major determinant to the immediate prognosis.
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PMID:[Clinical case of the month. Porto-pulmonary hypertension syndrome associated with severe hypoxemia]. 1158 37

We report the occurrence of pulmonary hypertension in a 37-year-old male patient with cirrhosis of the liver, portal hypertension and oesophageal varices. Although this is a rare combination, previous reports have shown that the association of portal and pulmonary hypertension is not coincidental; the temporal onset of primary pulmonary hypertension is hard to predict and our patient was asymptomatic for a number of years. The pathogenesis of portal hypertension leading to pulmonary hypertension is not known. Diagnosis is difficult because the clinico-pathological symptoms in both conditions are similar. Treatment is limited to calcium channel blockers, vasodilators, nitrous oxide and prostacyclin, although most patients will eventually require visceral transplantation.
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PMID:Lessons to be learned: a case study approach a case study of the temporal onset of pulmonary hypertension with pre-existent cirrhotic portal hypertension. 1181 Oct 97

In patients with cirrhosis, discrepant findings have been reported on the evolution of pulmonary hemodynamics and gas exchange after liver transplantation. The aim of this study was to evaluate the effects of liver transplantation on pulmonary and systemic hemodynamics and gas exchange in patients transplanted for cirrhosis. Forty-three patients with cirrhosis underwent hemodynamic investigations before and one year after liver transplantation. Mean pulmonary arterial pressures did not significantly change after transplantation (from 17 +/- 4 to 17 +/- 3 mm Hg) whereas pulmonary vascular resistance significantly increased by 62%. Cardiac index significantly decreased by 20%. PaO2 did not change significantly after transplantation (from 88.8 +/- 13.9 to 88.5 +/- 12.1 mm Hg) and PaCO2 significantly increased by 16%. In conclusion, liver transplantation has no effect on pulmonary pressures but normalizes pulmonary vascular resistance in patients with cirrhosis without pulmonary hypertension. Moreover, it has no major effect on gas exchange in patients with cirrhosis without hypoxemia.
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PMID:Pulmonary hemodynamics and gas exchange after liver transplantation in patients with cirrhosis. 1199 3

Abnormal diffusing capacity is the commonest pulmonary dysfunction in liver transplant candidates, but severe hypoxemia secondary to hepatopulmonary syndrome and significant pulmonary hypertension are pulmonary vascular manifestations of cirrhosis that may affect the perioperative course. We prospectively assessed the extent of pulmonary dysfunction in patients referred for liver transplantation. A total of 57 consecutive patients with chronic liver disease were evaluated. All patients had a chest radiograph, standing arterial blood gas on room air, pulmonary function testing, and Doppler echocardiogram. Those patients with arterial hypoxaemia (PaO(2) < 10 kPa) also underwent (99m)Tc-macroaggregated albumin lung scan, and nine patients had agitated normal saline injection during echocardiography to define further the existence of pulmonary vascular dilatation. Reduced diffusing capacity for carbon monoxide less than 75% of the predicted value was found in 29 of 57 (51%) patients. Although elevated alveolar-arterial oxygen tension difference was detected in 35% (20/57) of the patients, only four (7%) patients had hypoxemia. We were unable to find evidence of intrapulmonary vascular dilatation either on the lung scan or saline-enhanced echocardiography in any of these patients. Reduction in diffusing capacity for carbon monoxide was noted in 75% (18/24) of patients who were transplanted for primary biliary cirrhosis and was accompanied by widened alveolar-arterial oxygen tension in 10 out of 18 (56%) of patients. This study shows that in liver transplant candidates, diffusion impairment and widened alveolar-arterial oxygen tension difference were frequently detected, especially in patients with primary biliary cirrhosis.
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PMID:Pulmonary gas exchange abnormalities in liver transplant candidates. 1220 Jul 82

The hepatopulmonary syndrome (HPS), consisting of elevated alveolar-arterial oxygen gradient and intrapulmonary vascular abnormalities in the presence of advanced liver disease, is associated with high mortality. Liver transplantation (LT) has been used for the treatment of HPS; however, the success of LT for the treatment of HPS is not uniformly documented. We reviewed our experience over a 5-year period and identified eight adult patients with incapacitating respiratory symptoms compatible with HPS. Inclusion criteria included hypoxemia, normal lung volumes, reduced oxygen diffusing capacity (D(L)CO), and the presence of intrapulmonary shunting. Underlying liver disease was caused by hepatitis C (2 patients), primary biliary cirrhosis (1 patient), cryptogenic cirrhosis (1 patient), alcohol (2 patients), and hepatitis C with alcohol (2 patients). Six out of eight patients required preoperative oxygen support. Severe hypoxemia was present in seven patients (Pa(O2) 51.5 +/- 8.2 mm Hg). Three patients had complicating pulmonary hypertension. All patients exhibited a severely reduced D(L)CO (44.6 +/- 12.2% of predicted value). Six patients were transplanted, with five requiring oxygen support at the time of discharge. Resolution of oxygen dependency occurred in all patients but was delayed in the two patients exhibiting complicating pulmonary hypertension (288.5 +/- 37.4 v 53.5 +/- 35.7 days). All patients exhibited O2 saturations greater than 98% on room air. Currently, three patients are alive and off oxygen. The current report documents successful resolution of hypoxemia after LT in this pilot cohort. This supports the newly implemented United Network for Organ Sharing (UNOS) criteria, that LT for HPS may be extended to include patients with Pa(O2) < 60 mm Hg.
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PMID:Retrospective analysis of the results of liver transplantation for adults with severe hepatopulmonary syndrome. 1236 Apr 35


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