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Management of portopulmonary hypertension:New perspectives

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World Journal of Gastroenterology 2013年第45期19卷 8252-8257页

作者： Luigi Mancuso Francesca Scordato Michela Pieri Eliana Valerio Andrea Mancuso Department of Cardiologia Ospedale Cervello90100 Palermo Italy Cardiologia Clinica Candela 90100 Palermo Italy Epatologia e Gastroenterologia Ospedale Niguarda Cà Granda Milano 20162 Milano Italy Medicina Interna 1 ARNAS Civico 90100 Palermo Italy

portopulmonary hypertension(PPHTN)is a known complication of ***-to-severe PPHTN implies an extremely poor *** occurs in 5%-10%of patients referred for liver transplantation(LT),and probably with an higher incidence in patients with large portosystemic *** with moderate-tosevere pulmonary hypertension have been previously excluded from LT because of the extremely high surgical risk and since the post-transplant outcome reported was ***,new perspectives in the management of patients with portopulmonary hypertension are *** fact,some pulmonary vasoactive drugs have become routine in the treatment of patients with idiopathic pulmonary *** drugs,particularly epoprostenol,have been recently introduced in the treatment of patients with PPHTN,and have been shown to be effective in reducing pulmonary artery pressure as well as pulmonary vascular ***,recent studies seem to demonstrate that treatment with pulmonary vasoactive drugs could allow liver transplantation with acceptable surgical risks and excellent *** there are not large series nor prospective studies addressing this topic,the clinical scenario of patients with PPHTN seems to be positively changing.

关键词： portopulmonary hypertension Cirrhosis Liver transplantation Management Epoprostenol

Liver transplantation for severe portopulmonary hypertension: A case report and literature review

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World Journal of Clinical Cases 2019年第21期7卷 3569-3574页

作者： Xiao-Jie Chen Zhi-Jun Zhu Li-Ying Sun Lin Wei Zhi-Gui Zeng Ying Liu Wei Qu Liang Zhang Liver Transplantation Center National Clinical Research Center for Digestive DiseasesBeijing Friendship HospitalCapital Medical UniversityBeijing 100050China Clinical Center for Pediatric Liver Transplantation Capital Medical UniversityBeijing 100050China Beijing Key Laboratory of Tolerance Induction and Organ Protection in Transplantation Beijing 100050China Department of Intensive Care Unit Beijing Friendship HospitalCapital Medical UniversityBeijing 100050China

BACKGROUND portopulmonary hypertension(PoPH) is not uncommon in patients waiting for liver transplantation(LT).Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for *** patients with liver disease require but cannot receive LT due to severe PoPH and eventually *** report a patient with severe PoPH who underwent successful LT and had near normal pulmonary pressure without drug *** SUMMARY A 39-year-old woman was hospitalized with the chief complaint of jaundice and exertional dyspnea and *** disease and liver cirrhosis was diagnosed 6 years *** liver disease met the criteria for ***, right heart catheterization showed that her mean pulmonary artery pressure was increased at 50 mmHg, pulmonary vascular resistance was 460 dyn?s/cm^5 and pulmonary artery wedge pressure was 20 mmHg, which may have been the reasons for her chief *** patient was diagnosed with severe PoPH and was not listed for LT *** 5 mo of pharmacotherapy, her severe PoPH was moderate, and she underwent successful *** artery pressure steadily decreased according to post-operative echocardiographic monitoring and drugs have been discontinued for a *** The safety of LT can be greatly improved by reducing mean pulmonary artery pressure to a low level, and LT may cure PoPH.

关键词： portopulmonary hypertension Pharmacotherapy Liver transplantation Pulmonary hypertension crisis Treatment Case report

Hemodynamic effects of ambrisentan-tadalafil combination therapy on progressive portopulmonary hypertension

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World Journal of Hepatology 2014年第11期6卷 825-829页

作者： Yu Yamashita Ichizo Tsujino Takahiro Sato Asuka Yamada Taku Watanabe Hiroshi Ohira Masaharu Nishimura First Department of Medicine Hokkaido University Hospital

Intravenous epoprostenol is recommended for World Health Organization functional class(WHO-FC) Ⅳ patients with pulmonary arterial hypertension(PAH) in the latest guidelines. However, in portopulmonary hypertension(PoP H) patients, advanced liver dysfunction and/or thrombocytopenia often makes the use of intravenous epoprostenol challenging. Here we report the cases of two WHO-FC Ⅳ PoP H patients who were successfully treated with a combination of two oral vasodilators used to treat PAH: ambrisentan and tadalafil. Oral vasodilator therapy using a combination of ambrisentan and tadalafil may be a safe and effective therapeutic option for WHO-FC Ⅳ PoP H patients and should be considered for selected patients with severe and rapidly progressing PoP H.

关键词： portopulmonary hypertension Ambrisentan Tadalafil Thrombocytopenia

portopulmonary Hypertension in Patients Undergoing Liver Transplantation: A Case Report

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Surgical Science 2014年第2期5卷 53-56页

作者： Emica Shimozono Cristina A. A. Caruy Adilson R. Cardoso Ilka F. S. F. Boin State University of Campinas UNICAMP Liver Transplant Unit of Clinics Hospital Campinas Brazil

portopulmonary hypertension (PPH) is a form of pulmonary arterial hypertension, characterized by elevation of pulmonary arterial pressure due to increased resistance to pulmonary blood flow. It is a consequence of portal hypertension, associated or not with liver disease. A retrospective study of patients undergoing liver transplantation was carried out in the Liver Transplantation Unit of the Clinics Hospital of the State University of Campinas (UNICAMP) from 1991 to 2012, covering 389 transplants. Although 50 (12.9%) patients presented with mean pulmonary arterial pressure (MPAP) > 25 mmHg, only one case (0.25%) met the diagnostic criteria for PPH: a 43-year-old female patient with advanced liver disease, caused by primary biliary cirrhosis with portal hypertension, diagnosed as mild portopulmonary hypertension and diagnosed in the operating room. PPH is a serious complication of portal hypertension. It is related to the great difficulty in diagnosis and the high mortality rate due to right heart failure. PPH is an entity with a low frequency of occurrence in individuals undergoing liver transplantation, although there is a wide variation in the prevalence rate presented in the literature and the definitive diagnosis should be confirmed with right heart catheterization.

关键词： portopulmonary Hypertension Portal Hypertension Survival Liver Transplantation

Thoracic perspective revisited in chronic liver disease

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Gastroenterology Report 2015年第3期3卷 194-200页

作者： Binit Sureka Kalpana Bansal Yashwant Patidar Sachin Kumar Ankur Arora Department of Radiology/Interventional Radiology Department of Pulmonary Medicine Institute of Liver and Biliary SciencesNew DelhiIndia

A variety of chest manifestations are seen in patients with chronic liver diseases,namely hepatopulmonary syndrome,portopulmonary hypertension,intrathoracic portosystemic collaterals,hepatic hydrothorax,infections,drug-induced changes,manifestations of hepatocellular carcinoma,gynecomastia,acute respiratory distress syndrome,autoimmune changes,aspiration pneumonitis and changes due to a1-antitrypsin *** and radiologists should be aware of these entities;knowledge of the imaging findings specific to each condition is of prime importance for managing such patients.

关键词： cirrhosis infection portosystemic portopulmonary computed tomography