Bosentan therapy for portopulmonary hypertension

Hoeper, Marius M.; Halank, Michael; Marx, Christian; Hoeffken, Gert; Seyfarth, HJ; Schauer, J.; Niedermeyer, J; Winkler, Jörg

doi:10.1183/09031936.05.00080804

Cited by 183 publications

(123 citation statements)

References 31 publications

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“…In the current retrospective study, a significant clinical, functional and haemodynamic improvement was found over 3 months' treatment with oral sildenafil in patients with severe POPH. Successful treatment of POPH has been reported using prostanoids or endothelin-receptor antagonists, with observation times of 3-12 months [9][10][11][12][13]. Subcutaneous, i.v.…”

Section: Discussionmentioning

confidence: 99%

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Sildenafil treatment for portopulmonary hypertension

Reichenberger

Voswinckel²,

Steveling³

et al. 2006

European Respiratory Journal

193

121

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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 (n51) or severe (n513) POPH caused by alcoholic liver disease (n57), chronic viral hepatitis (n53), autoimmune hepatitis (n53), and hepatic manifestation of hereditary haemorrhagic teleangiectasia (n51) with oral sildenafil. Eight patients were newly started on pulmonary vasoactive treatment, while six patients were already on treatment with inhaled prostanoids (iloprost, n55; treprostinil, n51). 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?mL -1 to 230¡278 ng?mL, and to 189¡274ng?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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Section: Discussionmentioning

confidence: 99%

“…So far, case reports and a case series have been published showing clinical, functional and haemodynamic benefit on treatment with intravenous and inhaled prostanoids, oral bosentan or combination therapy [9][10][11][12][13].…”

mentioning

confidence: 99%

Sildenafil treatment for portopulmonary hypertension

Reichenberger

Voswinckel²,

Steveling³

et al. 2006

European Respiratory Journal

193

121

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“…Conventional treatment consists of fluid overload with diuretics and oxygen but does not improve survival rate [15]. Inhibitors of 1-endothelin as bosentan are an effective treatment in stable hepatic illness, however there is insufficient evidence of its effectiveness in patients with altered hepatic dysfunction [16]. Furthermore, patients treated with bosentan have shown better results in hemodynamic parameters and the 6 minute-walk distance test (6MWD) compared with patients treated with inhaled iloprost [17].…”

Section: Discussionmentioning

confidence: 99%

Hepatopulmonary Syndrome and Portopulmonary Hypertension in the Same Patient

Chertcoff¹

2016

J Clin Microbiol Biochem Technol

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A 71 year-old woman with alcoholic cirrhosis presented to the emergency room and referred effort dyspnea for the last six months. She had a past medical history of ex-smoker (10 pack/year), glaucoma and hypothyroidism. She had been abstinent from alcohol for one year and showed no admissions for edematous-ascites syndrome, esophageal varices or spontaneous bacterial peritonitis. Her Model for End-Stage Liver Disease (MELD) score at the time of presentation to our center was 17.On physical exam, her breath sounds were clear and did not show signs of fluid overload. Cyanosis, clubbing or platypnea were not present, however orthodeoxia was detected: SaO2 of 93% (0.21) in supine position and SaO2 of 89% in upright position. Computer tomography scans showed ground glass areas with and thickening of interlobular septa. Laboratory evaluation showed: hematocrit = 41%, hemoglobin = 14.5 g/dl, platelet count = 83000/mm3, total bilirubin = 2.7 g/dl, alkaline phosphatase =118 U/l, serum albumine = 4.1 g/ dl, aspartate aminotransferase = 30UI/l and alanine aminotransferase = 43UI/l. HIV serology was negative and her immunological profile inconsistent with collagen diseases.Pulmonary function, respiratory gas exchange and ventilationperfusion relationships were studied. Both forced spirometry results and lung volumes by plethysmography were normal. A severely reduced DLCO (37%) after adequate correction for anaemia was detected (Table 1). Arterial blood gas (ABG) on room air revealed a pH of 7.43, partial pressure of oxygen (PaO2) was 57.7 mmHg, partial pressure of dioxide (PaCO2) was 30 mmHg, and an alveolar arterial gradient of 43 mmHg. Contrast enhanced transesophageal echocardiography Abbreviations

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“…[30][31] Use of the endothelin A/B receptor antagonist bosentan currently is under investigation. Hoeper and associates showed the effectiveness of bosentan as monotherapy 32 and in combination with inhaled iloprost 33 for the treatment of POPH. Approximately 10% patients treated with bosentan develop hepatotoxicity due to intracellular bile salt accumulation.…”

Section: Discussionmentioning

confidence: 99%

Untitled

Reid

McQuillan²,

Kadry³

et al. 2017

Exp Clin Transplant

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Good right ventricular function and responsiveness to vasodilator therapy are the most important prerequisites for successful liver transplant in patients with portopulmonary hypertension. A patient with portopulmonary hypertension and good right ventricular function presented for deceased-donor liver transplant. Pulmonary arterial pressure was controlled with epoprostenol and sildenafil preoperatively. After anesthesia induction, pulmonary arterial pressure increased significantly and the procedure was aborted. Additional medical treatment included aggressive vasodilator therapy and the transplant was successfully performed 1 month later. During the procedure, elevations in pulmonary arterial pressure responded to a combination of inhaled nitric oxide, intravenous milrinone and nitroglycerin, and optimization of mechanical ventilation.

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Bosentan therapy for portopulmonary hypertension

Cited by 183 publications

References 31 publications

Sildenafil treatment for portopulmonary hypertension

Sildenafil treatment for portopulmonary hypertension

Hepatopulmonary Syndrome and Portopulmonary Hypertension in the Same Patient

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