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

Pulmonary abnormalities are common in patients with advanced chronic liver disease. Two distinct syndromes strictly related to the presence of portal hypertension, but clearly different from a pathophysiologic point of view, have been identified. Portopulmonary hypertension, characterized by an increased pulmonary arterial pressure, is due to a progressive arteriolar vasoconstriction induced by excess local production of vasoconstrictor substances. Hepatopulmonary syndrome results from intrapulmonary microvascular dilation caused by an inadequate synthesis or metabolism of putative pulmonary vasoactive substances leading to a functional vasodilation of the pulmonary vasculature, ultimately leading to hypoxemia. Controversies on pathogenesis imply different tentative therapeutic approaches for the medical management of these conditions. The development of portopulmonary hypertension or the hepatopulmonary syndrome has important clinical and prognostic implications facing the impact of new therapeutic strategies for the management of the main complications of advanced liver diseases on cardiopulmonary function.
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PMID:The pulmonary involvement in portal hypertension: portopulmonary hypertension and hepatopulmonary syndrome. 1639 30

Portopulmonary hypertension (POPH) is regarded as a subtype of pulmonary arterial hypertension (PAH); however, established PAH therapies have not been evaluated for this condition. The current authors treated 14 patients (four male, 10 female; mean (range) age 55 (39-75) yrs) with moderate (n = 1) or severe (n = 13) POPH caused by alcoholic liver disease (n = 7), chronic viral hepatitis (n = 3), autoimmune hepatitis (n = 3), and hepatic manifestation of hereditary haemorrhagic teleangiectasia (n = 1) with oral sildenafil. Eight patients were newly started on pulmonary vasoactive treatment, while six patients were already on treatment with inhaled prostanoids (iloprost, n = 5; treprostinil, n = 1). During treatment with sildenafil, mean +/- sd 6-min walk distance increased from 312 +/- 111 m to 397 +/- 99 m after 3 months, and 407 +/- 97 m after 12 months. Mean +/- sd pro-brain natriuretic peptide levels decreased from 582 +/- 315 ng x mL(-1) to 230 +/- 278 ng x mL(-1), and to 189 +/- 274 ng x mL(-1) after 3 and 12 months, respectively. Two patients died after 1 and 2 months from liver failure and cardiac failure, respectively. There was a similar response to sildenafil treatment after 3 and 12 months in patients on monotherapy and those on combination therapy. In conclusion, sildenafil might be effective in monotherapy and in combination therapy with inhaled prostanoids in portopulmonary hypertension, leading to significant improvement by 3 months and sustained response over 12 months.
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PMID:Sildenafil treatment for portopulmonary hypertension. 1719 88

We present the case of a 9-year-old boy with portal hypertension who died suddenly and unexpectedly due to pulmonary hypertensive crisis during a routine endoscopic procedure. He had known portal hypertension with esophageal varices but had no preceding clinical symptoms suggestive of significant pulmonary hypertensive disease despite postmortem histological evidence of advanced pulmonary vascular changes. Portopulmonary hypertension is a well-described and distinct clinical syndrome that is rare in childhood and is associated with a relatively poor prognosis. Occasional patients with histologically advanced disease may remain asymptomatic but present with pulmonary hypertensive crisis. Children with portopulmonary hypertension should be considered at high risk for surgical procedures, and pulmonary hypertensive complications should be excluded as a cause of death in all children dying suddenly in the setting of portal hypertension.
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PMID:Portopulmonary hypertension in childhood presenting as sudden death. 1680 32

Portopulmonary hypertension is a recognized but uncommon complication of cirrhosis. Liver transplantation may be contraindicated in patients with severe portopulmonary hypertension. In order to decrease the pulmonary arterial pressure, intravenous administration of epoprostenol has been shown to provide substantial beneficial results in these patients. Additionally, a recent case report demonstrated that long-term oral administration of sildenafil decreased pulmonary arterial pressure, but its effects on splanchnic hemodynamics were not measured. We report on a patient with cirrhosis and portopulmonary hypertension and the changes in the hemodynamic status after an oral administration of sildenafil. This case report clearly delineates that sildenafil decreases pulmonary arterial pressure but may exacerbate portal hypertension and hyperdynamic circulation in patients with cirrhosis and portopulmonary hypertension.
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PMID:Sildenafil decreased pulmonary arterial pressure but may have exacerbated portal hypertension in a patient with cirrhosis and portopulmonary hypertension. 1686 9

Portopulmonary hypertension (PPHT) is a rare but devastating complication in patients with portal hypertension, characterized by pulmonary arterial obliterative disease with a concomitant rise in pulmonary vascular resistance. A broad body of evidence has accumulated, indicating that endothelin (ET) peptides and their cognate receptors are causally involved in the pathophysiology of pulmonary arterial hypertension (PAH) owing to different aetiologies, including PPHT. In addition, the ET system may be involved in hepatic fibrotic remodelling and portal hypertension. Several experimental models have provided evidence that ET receptor antagonism may have therapeutic potential in PPHT. Initial experience has accumulated during the last 2 years, suggesting that targeting the ET system may have beneficial effects in the clinical setting. In these studies, the orally active, dual ET receptor antagonist bosentan improved pulmonary haemodynamics and functional capacity. These effects were sustained and occurred in the absence of adverse events. If these observations can be corroborated by controlled clinical trials, bosentan would offer several advantages over available therapies, which have major drawbacks owing to their invasive and demanding mode of application.
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PMID:Endothelin and endothelin receptor antagonism in portopulmonary hypertension. 1691 12

Portopulmonary hypertension (PPHT) is defined as precapillary pulmonary hypertension accompanied by hepatic disease or portal hypertension. Pulmonary hypertension results from excessive pulmonary vascular remodeling and vasoconstriction. These histological alterations have been indistinguishable from those of other forms of pulmonary arterial hypertension. Factors involved in the pathogenesis of PPHT include volume overload, hyperdynamic circulation, and circulating vasoactive mediators. The disorder has a substantial impact on survival and requires focused treatment. Liver transplantation in patients with moderate to severe PPHT is associated with a significantly reduced survival rate. The best medical treatment for patients with PPHT is controversial; most authors currently regard continuous intravenous application of prostacyclin as the treatment of choice for patients with severe PPHT. There is only very limited reported experience with inhaled prostacyclin or its analog, iloprost. Increasing evidence of the efficacy of the endothelin-receptor antagonist bosentan and of the phosphodiesterase-5 inhibitor sildenafil is emerging in highly selected patients with PPHT. In the future, a combination therapy of the above-mentioned agents might become a therapeutic option. Other agents such as beta-blockers seem to be harmful to patients with moderate to severe portopulmonary hypertension. Up-to-date, randomized, double-blind, controlled clinical trials are lacking and are needed urgently.
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PMID:Portopulmonary hypertension. 1704 47

Portopulmonary hypertension is a common condition in patients who have portal hypertension. This article reviews the definition and clinical presentation of this disorder and outlines our current understanding of its pathophysiology. A diagnostic approach is provided , and novel medical therapies that are being investigated to treat this condition are discussed. Finally, the safety of liver transplantation in patients who have portopulmonary hypertension is reviewed.
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PMID:Portopulmonary hypertension. 1716 33

Portopulmonary hypertension is characterized by a chronic liver disease associated with a mean pulmonary artery pressure >25 mmHg at rest, an increased pulmonary vascular resistance and a capillary pulmonary pressure <15 mmHg with portal hypertension. Schistosomiasis may be an aetiology of this syndrome, however, few cases have been reported. We describe the first cases of portopulmonary hypertension with schistosomiasis in Malagasy patients. There were 2 men aged of 18 and 20 from hyperendemic area of schistosomiasis in Madagascar Both had a history of repeated water contact. They presented a dyspnea associated with ascites and oedema. Clinical examination showed portal and pulmonary hypertension with right ventricular heart failure. Cardiac examination revealed a systolic murmur and splint of the second heart pulmonary Pulmonary hypertension was confirmed by cardiac ultrasonogaphy Serology of bilharzias was positive. Parasitological examination showed eggs of S. mansoni. The treatment based on salt-free diet, spironolactone and praziquantel led to a better evolution of symptoms (case 1). Symptoms of right heart failure remained for the second patient even though improvement was noted. In tropical countries, schistosomiasis may be one of the cause of portopulmonary hypertension and may appear in early age. Its treatment remains difficult as the drugs recommended are not affordable.
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PMID:[Portopulmonary hypertension due to schistosomiasis in two Malagasy patients]. 1740 90

Portopulmonary hypertension is an uncommon but treatable pulmonary vascular consequence of portal hypertension, which can lead to significant morbidity and mortality. Portopulmonary hypertension results from excessive pulmonary vasoconstriction and vascular remodeling that eventually leads to right-heart failure and death if left untreated. Although pulmonary vascular disease in these patients may be asymptomatic or associated with subtle and nonspecific symptoms (dyspnea, fatigue and lower extremity swelling), it should be looked for especially if patients are potential candidates for liver transplantation. Patients with clinical suspicion of portopulmonary hypertension should undergo screening testing, specifically echocardiography. Right heart catheterization remains the gold standard for the diagnosis. The existence of moderate to severe disease poses higher risks and challenges for liver transplantation. The disease has a substantial impact on survival and requires focused pharmacological therapy. New and evolving medical therapies, such as prostanoids (intravenous, inhaled or oral), endothelin receptors antagonists, phosphodiesterases inhibitors, combination therapy and other experimental drugs might change the natural course of the disease. Case reports and cases series have been published regarding the efficacy and safety of pharmacological therapy, but randomized, controlled multicenter trials are urgently needed. Liver transplantation is not the treatment of choice for portopulmonary hypertension, but after optimal hemodynamic and clinical improvement with medical therapy as a bridge, liver transplant can be considered an option in selected patients.
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PMID:Portopulmonary hypertension: state of the art. 1903 31

Portopulmonary hypertension (PPH) is clinically defined as the development of pulmonary arterial hypertension complicated by portal hypertension, with or without advanced hepatic disease. Physical signs may be absent in mild to moderate PPH and only appear in a hyperdynamic circulatory state. Similar signs of advanced liver disease can be observed in severe PPH, with ascites and lower extremity edema. Pulmonary hypertension is usually diagnosed after anesthetic induction during liver transplantation (LT). We present intraoperative pulmonary hypertension in a 41-year-old male patient with hepatic cirrhosis. Since this patient had no preoperation laboratory data supporting the diagnosis of pulmonary hypertension and was asymptomatic for a number of years, it was necessary to send him to the intensive care unit after operation. Further study should be focused on the diagnosis and treatment of pulmonary arterial hypertension in order to reduce its mortality.
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PMID:Intraoperative pulmonary hypertension occurred in an asymptomatic patient with pre-existent liver cirrhotic and portal hypertension. 1908 45


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