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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

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 a case of portopulmonary hypertension in which the pulmonary hypertension resolved after initial orthotopic liver transplantation. Portopulmonary hypertension recurred when the transplanted liver failed and again resolved after a second liver transplantation. Intravenous epoprostenol was administered perioperatively to control the pulmonary hypertension in both instances.
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PMID:Recurrent portopulmonary hypertension after liver transplantation: management with epoprostenol and resolution after retransplantation. 1146 Feb 34

Portopulmonary hypertension occurs in 2-8% of liver recipients. However, new onset of pulmonary hypertension following liver transplantation has been reported only once. We report de novo occurrences of portopulmonary hypertension in two liver recipients following successful liver transplantation. Although both patients had recurrent hepatitis C after the transplant, both had excellent clinical graft function. In one patient, upper endoscopy and aortogram showed evidence of persistent venous collaterals in the abdomen. Both patients presented with shortness of breath. Portopulmonary hypertension was diagnosed late, thus contributing directly to their deaths. Autopsy in one patient confirmed the absence of significant liver pathology and failed to demonstrate any source of deep venous thrombosis. This, and our earlier case report, highlights the potential for the occurrence of pulmonary hypertension following liver transplantation. Further studies are needed to determine the scope of the problem and identify patients at risk for this complication.
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PMID:De novo diagnosis of portopulmonary hypertension following liver transplantation. 1239 97

Portopulmonary hypertension represents a major risk factor for transplantation; therefore, preoperative detection is crucial. The aims of this study were to determine (1) whether Doppler echocardiography performed at evaluation is a reliable tool for detecting portopulmonary hypertension and (2) the incidence of acquired portopulmonary hypertension profile after evaluation. One hundred sixty-five patients had Doppler echocardiography and right heart catheterization at evaluation over a 9-year period. All patients had a prospective follow-up, and the results of catheterization at evaluation were compared with those obtained at the time of transplantation. Seventeen of 165 patients met the criteria for portopulmonary hypertension on Doppler echocardiography. Portopulmonary hypertension was confirmed by catheterization in 10 patients and ruled out in 7. There were no false negatives for echocardiography. Mean pulmonary artery pressure was significantly higher during the initial phase of transplantation than at evaluation (17.8 +/- 4.3 vs. 20.3 +/- 5.5 mm Hg, respectively, P <.0001), and there was no significant correlation between values obtained at these 2 time points. Three patients showed to have acquired portopulmonary hypertension profile while waiting for a graft within time intervals ranging from 2.5 to 5 months. In conclusion, Doppler echocardiography is a highly sensitive tool for detecting portopulmonary hypertension. However, because this technique has a poor positive predictive value, right heart catheterization is recommended for confirming portopulmonary hypertension. In addition, the absence of portopulmonary hypertension at evaluation does not exclude the occasional occurrence of acquired portopulmonary hypertension profile after listing.
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PMID:Diagnosis of portopulmonary hypertension in candidates for liver transplantation: a prospective study. 1462 36

Liver disease affects the lungs. The majority of patients exhibit mild to moderate arterial hypoxaemia essentially attributable to an alteration in ventilation/perfusion matching and limited by an increase in ventilation. A minority (some 10%) of patients exhibit a "hepatopulmonary syndrome" defined by severe hypoxaemia with arterial PO2 below 60 mm Hg, dyspnoea, cyanosis, digital clubbing, orthodeoxia, platypnoea and demonstrable pulmonary vascular dilatations causing a true pulmonary shunt and a diffusion/perfusion imbalance. The hepatopulmonary syndrome is incurable but resolves over time after liver transplantation. An even lower proportion of patients, approximately 1%, develop pulmonary hypertension. Clinically this "portopulmonary hypertension" resembles primary pulmonary hypertension, with dyspnoea and fatigue as the main symptoms, histopathology and response to prostacyclin therapy. Portopulmonary hypertension is irreversible. Liver transplantation mortality in patients with portopulmonary hypertension ranges from 50 to 100%. The common cause of the hepatopulmonary syndrome and portopulmonary hypertension is portal hypertension and portosystemic shunting, indicating that vasoactive and angiogenetic factors originating from the liver normally control the pulmonary circulation.
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PMID:Hepatopulmonary syndrome and portopulmonary hypertension. 1271 85

Portopulmonary hypertension is a rare complication of portal hypertension. Although epoprostenol infusion, nitric oxide inhalation, isosorbide-5-mononitrate, nitroglycerin, and calcium channel blockers may reduce pulmonary artery pressure in patients with portopulmonary hypertension, the prognosis remains poor. We present a case of congenital hepatic fibrosis associated with pulmonary hypertension. A 42-year-old man with congenital hepatic fibrosis visited our hospital with syncope. The man had suffered from breathlessness on exertion for 2 weeks before the episode of syncope. He also had a history of portal hypertension with documented gastric cardiac varices at the age of 28 years. Despite undergoing intensive care, the patient died 1 week after admission owing to severe right-sided heart failure. Autopsy revealed dilatation of the right atrium and right ventricle grossly and plexogenic pulmonary arteriopathy microscopically. Accurate diagnosis of portopulmonary hypertension requires awareness of the disease and a high index of suspicion when examining patients with portal hypertension and dyspnea.
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PMID:Severe portopulmonary hypertension in congenital hepatic fibrosis. 1279 Feb 24

Primary or non-hemodynamic pulmonary hypertension is characterized by the increased pulmonary arterial pressure, higher than 3.32 kPa, and normal pulmonary capillary pressure less than 1.99 kPa. Primary pulmonary hypertension is a rare complication of portal hypertension, which significantly increases surgical morbidity and mortality, and it is not reversible after liver transplantation. As a rule, clinical manifestations of pulmonary hypertension are disguised by complications of portal hypertension. The symptoms of chronic pulmonary heart, such as peripheral edema and ascites, are usually ascribed to liver insufficiency and/or portal hypertension. Clinical significance of portopulmonary hypertension follows from the fact that the failure of the right heart is most commonly the direct cause of death in these patients. The majority of authors concurs that the right heart catheterization is the most valid method for diagnosis of portopulmonary hypertension. Doppler echocardiography represents quite valuable non-invasive diagnostic method, especially in patients with extensive spontaneous and surgical porto-systemic collaterals, rendering the high risk group of cases. Epoprostenol (prostacyclin), administered via continuous infusion, diminishes the platelet aggregation and causes the intense pulmonary and systemic vasodilatation. Good hemodynamic effect of this substance was verified in patients with primary pulmonary hypertension. Several articles have reported that long-term, continual epoprostenol administration in dose of 10 to 28 ng/kg/min. Has significantly reduced pulmonary vascular pressure and pulmonary vascular resistance. Portopulmonary hypertension represented the absolute contraindication for liver transplantation. Huge clinical significance of epoprostenol reflects in the fact that, along with the improvement of pulmonary hemodynamics, it provides the prerequisite for liver transplantation in patients with portopulmonary hypertension.
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PMID:[Epoprostenol in the treatment of portopulmonary hypertension]. 1469 37

Portopulmonary hypertension (PPHTN) is associated with poor prognosis and high perioperative mortality after orthotopic liver transplantation. This study documents the first case of a patient with PPHTN who was successfully bridged to orthotopic liver transplantation with i.v. iloprost, a stable prostacyclin analogue. The PPHTN had resolved completely 4 months after successful transplantation. In conclusion, portopulmonary hypertension is a relative contraindication to orthotopic liver transplantation, which should be attempted only if pulmonary haemodynamics improve with prostanoids. In this context, iloprost may be a valuable alternative to epoprostenol.
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PMID:Intravenous iloprost bridging to orthotopic liver transplantation in portopulmonary hypertension. 1545 52

Portopulmonary hypertension is a poorly understood and uncommon complication of advanced chronic liver disease. Current therapy is based largely on treatment options proven in idiopathic pulmonary hypertension. The severity of the portopulmonary hypertension should best be attenuated medically before attempting combined liver and lung transplantation to avoid increased peri-operative mortality. This case report describes the successful use of sildenafil to decrease the pulmonary vascular resistance in a patient with hepatitis-C cirrhosis who was preparing for liver transplantation.
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PMID:Novel use of sildenafil in the treatment of portopulmonary hypertension. 1579 56


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