Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Portopulmonary hypertension (POPH), or pulmonary arterial hypertension associated with cirrhosis, carries a high mortality and often precludes liver transplantation. Many POPH patients have preserved or increased cardiac output; therefore, decreasing pulmonary artery pressure rather than improving cardiac output is more important in reducing liver transplant risk, and this makes sildenafil an attractive therapeutic option. We assessed the clinical response of patients with POPH treated with sildenafil monotherapy. We retrospectively reviewed the charts of 10 patients with POPH and sildenafil monotherapy. Eight of 10 patients had hepatitis C virus infection. Patients took 31 +/- 14 mg (mean +/- standard deviation) thrice daily and were followed for 21 +/- 16 months. The World Health Organization functional class improved from 3.0 +/- 0.0 to 2.3 +/- 0.5 at 1 year (P < 0.05). Four of 8 patients increased the 6-minute walk distance at 1 year by 30 m or more. Three patients became transplant-eligible, 1 of whom underwent successful transplantation, and 3 patients have been stable without progression of liver disease or POPH. The remainder were not transplant candidates because of refractory POPH (n = 2) or other comorbidities (n = 2). We conclude that sildenafil may be an effective therapy for POPH that can stabilize or improve hemodynamics in patients with POPH and thereby facilitate liver transplantation.
 ...
PMID:Sildenafil monotherapy in portopulmonary hypertension can facilitate liver transplantation. 1910 43

Portopulmonary hypertension (PPHTN) is pulmonary arterial hypertension (PAH) associated with portal hypertension. It is a common condition among liver transplantation candidates; however, its association with congenital absence of the portal vein (CAPV) has not yet been established. CAPV is a very rare developmental anomaly, which is usually accompanied by abnormal mesenteric drainage that bypasses the liver. Here, we report a rare case of severe PPHTN secondary to CAPV.
 ...
PMID:Portopulmonary hypertension associated with congenital absence of the portal vein treated with bosentan. 1936 55

Portopulmonary hypertension (PPHTN) refers to the development of pulmonary arterial hypertension in the setting of portal hypertension with or without chronic hepatic failure. This syndrome is characterized by marked alternations of pulmonary vascular tone and obstruction of pulmonary arterial blood flow. An increased pulmonary blood flow, which is a hallmark of the hyperdynamic circulation of cirrhotic patients, seems to be present in almost all patients who develop PPHTN. The elevations of pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) along with the transpulmonary gradient (TPG) have been considered in diagnosing PPHTN. Only a high TPG reflects the severity of obstruction to pulmonary blood flow and differentiates an elevated PAP with concomitant elevated PVR from the situation where the increase in PAP is due only to the hyperdynamic flow and elevated volume. A considerable risk for cardiovascular death arises when PAP increases significantly; this may occur in rapidly evolving syndromes, in very advanced disease, or during a complicated liver transplantation. The distinction between PPHTN and elevated PAP in the context of a hyperdynamic state is of great importance; a PAP increase of hyperkinetic origin, as opposed to PPHTN, is apparently not associated with a high risk for adverse effects during and following liver transplantation.
 ...
PMID:Hemodynamic profile of portopulmonary hypertension. 1946 May 27

Recent years have witnessed a significant increase in the knowledge about the pathophysiology of pulmonary arterial hypertension (PAH) and the availability of multiple drugs specifically aimed at pulmonary circulation. Although most of this is related to the idiopathic form of PAH, this development has also turned attention to other forms of pulmonary hypertension such as schistosomiasis-associated PAH (Sch-PAH), portopulmonary hypertension (POPH), and hemolysis-associated pulmonary hypertension. The importance of these different forms of pulmonary hypertension spans their epidemiology and the multiple pathophysiological mechanisms associated with their development and complications. Taken together, Sch-PAH and hemolysis-associated pulmonary hypertension may represent the most prevalent forms of PAH worldwide. Portopulmonary hypertension is particularly important if the morbidity and mortality that it adds to patients with liver disease is considered. Although clear progress has been reached in these various forms of PAH, there are many different aspects yet to be addressed that may contribute to the basis of specific treatment applied to these subgroups of patients.
 ...
PMID:Other causes of PAH (schistosomiasis, porto-pulmonary hypertension and hemolysis-associated pulmonary hypertension). 1963 84

The pulmonary complications of end-stage liver disease include hepatopulmonary syndrome and portopulmonary hypertension. The etio-pathogenesis of these conditions is as yet unclear. Hepatopulmonary syndrome is a gas exchange abnormality and usually manifests as hypoxemia secondary to intra-pulmonary vascular shunts. These shunts can be demonstrated by echocardiography using agitated saline injections, and quantitated by lung perfusion scans. Liver transplantation is the treatment of choice for hepatopulmonary syndrome, and there are no effective pharmacological therapies. Portopulmonary hypertension is a hemodynamic problem which manifests as fatigue and right sided cardiac failure. Several vasoactive agents have been used to lower mean pulmonary arterial pressures. Portopulmonary hypertension is a relative contraindication to liver transplantation.
 ...
PMID:Hepatopulmonary syndrome and portopulmonary hypertension. 1979 40

Portopulmonary hypertension is a rare and severe complication of patients with cirrhosis. Sarcoidosis, a disease of unknown etiology, is also a cause of pulonary hypertension and right heart dysfunction. We report the case of a 51-year-old male patient, suffering from cirrhosis due to Wilson's disease, portal hypertension and pulmonary hypertension (PH), who developed severe pericarditis. Wilson's disease was diagnosed 8 years before his last admission to our hospital and was being successfully treated with D-penicillamine. PH was recognized 2 years before admission and being treated with bosentan. The patient complained for dyspnea at rest and the 2D echocardiogram revealed a significant amount of pericardial fluid. All other causes of acute pericarditis were excluded and his laboratory, imaging and histopathological investigation showed evidence of sarcoidosis. He underwent a therapy with corticosteroids (methylprednisolone) and his follow-up examination showed remarkable decrease of the levels of mean pulmonary artery pressure and pericardial fluid.
 ...
PMID:Sarcoidosis-induced pericarditis in a patient with portopulmonary hypertension: a case report. 1991 93

Portopulmonary hypertension (PoPH) is a rare but life-threatening complication of portal hypertension that is characterised by proliferative changes in the pulmonary microvasculature indistinguishable from other forms of pulmonary arterial hypertension (PAH). Although PoPH is most commonly observed in the setting of cirrhosis, patients with non-cirrhotic portal hypertension are also at risk of developing the disorder. A definitive diagnosis requires invasive haemodynamic confirmation by right heart catheterisation and screening for PoPH should be routinely performed in all patients being considered for liver transplantation. Although severe PoPH is considered a contraindication to liver transplantation, there is now compelling data supporting the use of PAH-specific therapies with the aim of improving pulmonary haemodynamics to allow transplantation to be successfully performed. This review explores possible relevant aetiological factors and summarises current diagnostic and therapeutic approaches for PoPH patients.
 ...
PMID:Current management approaches to portopulmonary hypertension. 2117 11

Portopulmonary hypertension is defined as the combination of pulmonary arterial hypertension with portal hypertension and presents management complications in patients awaiting liver transplantation. The combination of these vascular disorders has a marked impact on mortality. At present the recommendations for management are limited because of the paucity of definitive clinical trials. We have reviewed the available data on prevalence, diagnosis and treatment. It is clearly time to more formally approach the study of this patient population.
 ...
PMID:Portopulmonary hypertension: challenges in diagnosis and management. 2118 May 56

The development of pulmonary arterial hypertension in the setting of portal hypertension is known as portopulmonary hypertension. Portal hypertension is thought to predispose patients to disturbances in the homeostatic regulation of numerous neurohumoral and vasoactive mediators that induce the development of pulmonary arterial hypertension. Portopulmonary hypertension is pathologically indistinguishable from idiopathic pulmonary arterial hypertension and is characterized by the development of vasoconstriction, vascular remodeling, and thrombosis within the pulmonary vasculature. Although described in patients with both cirrhotic and noncirrhotic portal hypertension, portopulmonary hypertension is most prevalent among patients with end-stage liver disease, and its severity seems to be independent of the etiology or severity of liver disease. All liver transplant candidates must be screened for the presence of portopulmonary hypertension because of the high perioperative mortality risk of liver transplantation associated with this condition. Primary screening for portopulmonary hypertension consists of Doppler-estimated pulmonary artery systolic pressure measurement during echocardiography. However, the diagnosis of portopulmonary hypertension is based on unique hemodynamic criteria as determined by right heart catheterization. Untreated portopulmonary hypertension portends a poor prognosis, and the efficacy of current treatment modalities is limited. At present, the primary goals of therapy are to provide symptomatic relief, prolong survival, and improve pulmonary hemodynamics to facilitate safe and successful liver transplantation.
 ...
PMID:Portopulmonary hypertension. 2132 52

Portopulmonary hypertension is a complication of chronic liver disease, which has significant effects on survival and prognosis. Although the pathogenesis of pulmonary arterial hypertension has been well known, portopulmonary hypertension is often underestimated in patients with chronic liver disease. Every clinician who manages patients with chronic liver disease complaining of dyspnea should consider portopulmonary hypertension because this disorder requires special treatment. Herein, a 40-year-old woman with liver cirrhosis who complained of dyspnea on exercise is presented. She was diagnosed with portopulmonary hypertension by echocardiography and right-heart catheterization. Beraprost was used to reduce the pulmonary arterial pressure and improve the symptoms. Her symptoms were improved after 2 weeks, and improved symptoms and reduced pulmonary arterial pressure were sustained for 18 months.
 ...
PMID:Successful management of portopulmonary hypertension with beraprost. 2138 99


<< Previous 1 2 3 4 5 6 Next >>