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Suman Verma, Fiona Hand, Matthew J Armstrong, Marie de Vos, Douglas Thorburn, Terry Pan, John Klinck, Rachel H Westbrook, Georg Auzinger, Andrew Bathgate, Steven Masson, Andrew Holt, Diarmaid D Houlihan, James W Ferguson
: Liver Transplantation(LT) in patients with portopulmonary hypertension(PoPH) has historically resulted in unpredictable and often poor outcomes. The United Kingdom experience for the period 1992-2012 is reported in this paper. METHODS: A retrospective analysis of patients, pre-operatively fulfilling the PoPH European Respiratory Society Task Force on Pulmonary-Hepatic Vascular Disorders diagnostic criteria was conducted across all UK liver transplant centres. Data collection included co-morbidities, use of pre- and post- operative pharmacotherapy, patient survival, and cause of death...
September 4, 2016: Liver Transplantation
Haruka Sato, Masanobu Miura, Nobuhiro Yaoita, Saori Yamamoto, Shunsuke Tatebe, Tatsuo Aoki, Kimio Satoh, Hideki Ota, Kei Takase, Koichiro Sugimura, Hiroaki Shimokawa
Cardiopulmonary abnormalities are often present in patients with liver diseases. We herein report a case of congenital portosystemic shunts complicated by hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH). A 57-year-old woman complained of dyspnea and was subsequently diagnosed with HPS and PoPH caused by congenital portosystemic shunts. Although shunt closure by transcatheter embolization was successfully performed, her dyspnea worsened and pulmonary artery pressure and pulmonary vascular resistance elevated...
2016: Internal Medicine
Michael J Krowka, Michael B Fallon, Steven M Kawut, Valentin Fuhrmann, Julie K Heimbach, Michael A E Ramsay, Olivier Sitbon, Ronald J Sokol
Two distinct pulmonary vascular disorders, hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) may occur as a consequence of hepatic parenchymal or vascular abnormalities. HPS and POPH have major clinical implications for liver transplantation. A European Respiratory Society Task Force on Pulmonary-Hepatic Disorders convened in 2002 to standardize the diagnosis and guide management of these disorders. These International Liver Transplant Society diagnostic and management guidelines are based on that task force consensus and should continue to evolve as clinical experience dictates...
July 2016: Transplantation
Lynn A Fussner, Michael J Krowka
Portopulmonary hypertension (POPH) is a form of pulmonary arterial hypertension occurring in the setting of portal hypertension with or without hepatic cirrhosis. The presence of both portal and pulmonary vascular disease contributes to complicated hemodynamics and therapeutic challenges, though the severities do not appear to correlate directly. Diagnosis of POPH, and distinction from the commonly observed hyperdynamic state of end-stage liver disease, is typically accomplished with an initial screening transthoracic echocardiogram, followed by right heart catheterization for confirmation of hemodynamic parameters...
June 2016: Current Gastroenterology Reports
Xueli Bai, Xiaoyu Zhang, Fangyan Lu, Guogang Li, Shunliang Gao, Jianying Lou, Yun Zhang, Tao Ma, Ji Wang, Wei Chen, Bingfeng Huang, Tingbo Liang
INTRODUCTION: The experience of implementing enhanced recovery after surgery (ERAS) programs in pancreatic surgery is limited. The aim of this study was to evaluate the feasibility of ERAS program in pancreatic surgery in an academic medical center of China. METHODS: Between May 2014 and August 2015, 124 patients managed with an ERAS program following pancreatic surgery (ERAS group), were compared to a historical cohort of 63 patients, treated with traditional perioperative care between August 2013 and April 2014 (no-ERAS group)...
July 2016: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
Yong Lv, Guohong Han, Daiming Fan
Portopulmonary hypertension (PoPH) refers to the condition that pulmonary arterial hypertension (PAH) occur in the stetting of portal hypertension. The development of PoPH is thought to be independent of the severity of portal hypertension or the etiology or severity of liver disease. PoPH results from excessive vasoconstriction, vascular remodeling, and proliferative and thrombotic events within the pulmonary circulation that lead to progressive right ventricular failure and ultimately to death. Untreated PoPH is associated with a poor prognosis...
July 2016: Scandinavian Journal of Gastroenterology
Guozhen Yan, Junfeng He, Yueli Yu, Yang Liu, Yanfen Yuan, Zhiyong Guo
The continuous changes in pulmonary hemodynamic properties in hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) have not been fully characterized in large animal models of HPS and PoPH. Beagle dog models of HPS and PoPH were induced by chronic common bile duct ligation and Sephadex microspheres, respectively. The model was validated by catheter examination and pathological analyses, and the hemodynamic characteristics of the models were observed. The results revealed that the cross-sectional area of the blood vessel was significantly increased in HPS models, but it was significantly decreased in the PoPH models...
January 2016: Biomedical Reports
Masako Terao, Akinobu Takaki, Takayuki Maruyama, Hiroki Oe, Tetsuya Yasunaka, Naofumi Tamaki, Kazufumi Nakamura, Takaaki Tomofuji, Takahito Yagi, Hiroshi Sadamori, Yuzo Umeda, Susumu Shinoura, Ryuichi Yoshida, Kazuhiro Nouso, Daisuke Ekuni, Kazuko Koike, Fusao Ikeda, Hidenori Shiraha, Manabu Morita, Hiroshi Ito, Toshiyoshi Fujiwara, Kazuhide Yamamoto
OBJECTIVE: Hepatopulmonary syndrome (HPS) is characterized by vascular dilatation and hyperdynamic circulation, while portopulmonary hypertension (POPH) is characterized by vasoconstriction with fibrous obliteration of the vascular bed. Vasoactive molecules such as nitric oxide (NO) are candidate factors for cirrhotic complications associated with these diseases. However, oxidative stress balance is not well characterized in HPS and POPH. The present objective is to investigate the oxidative stress and anti-oxidative stress balance and NO pathway balance in patients with potential HPS and POPH...
2015: Internal Medicine
Aparna Dalal
Patients with end stage liver disease (ESLD) have complex problems such as cirrhotic cardiomyopathy, coronary artery disease, hepatopulmonary syndrome (HPS), portopulmonary hypertension (POPH), hepatic encephalopathy, intracranial hypertension, (ICP), left ventricular outflow tract obstruction (LVOTO), high Model of end liver disease (MELD) scores, hyponatremia, and coagulopathies. The anesthesia management for liver transplantation can be very complex, dynamic and challenging. Anesthesia agents affect hepatic blood flow and anesthetic drug distribution, metabolism and elimination maybe altered in end stage liver disease...
January 2016: Transplantation Reviews
Lynn A Fussner, Vivek N Iyer, Rodrigo Cartin-Ceba, Grace Lin, Kymberly D Watt, Michael J Krowka
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are pulmonary vascular complications of portal hypertension with divergent clinicopathologic features and management. The presence of intrapulmonary vascular dilatations (IPVDs), detected by agitated saline contrast-enhanced transthoracic echocardiography (cTTE), is an essential feature of HPS but is not typically characteristic of POPH. Although IPVDs have been reported rarely in POPH, the prevalence and significance of this finding have not been systematically studied...
November 2015: Liver Transplantation
T Horvatits, A Drolz, K Rutter, K Roedl, S Kluge, V Fuhrmann
Interactions between the hepatic portal and cardiovascular systems are frequently found in patients with liver disease. Cirrhotic cardiomyopathy (CCMP) is defined as reduced cardiac function in patients with liver cirrhosis in the absence of other known causes of cardiac disease. The typical hyperdynamic circulatory state by means of increased cardiac output and reduced systemic vascular resistance may mask left ventricular failure. Portopulmonary hypertension (POPH) is defined as increased pulmonary arterial pressure and the presence of portal hypertension, and is associated with increased mortality...
June 2016: Medizinische Klinik, Intensivmedizin und Notfallmedizin
Amy C Chan-Dominy, Sarfaraz N Rahiman, Marc Anders, Warwick Butt
BACKGROUND: Patients with severe portopulmonary hypertension (PoPH) responsive to medical therapy may be considered for liver transplantation. We present a case of extracorporeal membrane oxygenation (ECMO) resuscitation for PoPH crisis in a child following liver transplantation (LT), and review the literature on management of this challenging setting. CASE REPORT: A 7-year-old girl, with previous Kasai portoenterostomy and subsequent severe PoPH responsive to pulmonary vasodilator therapy, underwent orthotopic LT...
June 2015: International Journal of Artificial Organs
Amita Singh, Ankur Girdhar, Faisal Usman, James Cury, Abubakr Bajwa
Pulmonary syndromes in the setting of hepatic disease with portal hypertension include portopulmonary hypertension (POPH), hepatopulmonary syndrome (HPS) and hepatic hydrothorax. POPH is defined as pulmonary arterial hypertension with portal hypertension in the absence of other causes of pulmonary arterial hypertension. HPS is a defect in arterial oxygenation as a result of pulmonary micro vascular dilatation in the setting of liver disease. We discuss a case of 63-year-old female with liver cirrhosis, exertional dyspnea and hypoxia associated with coexistence of POPH and HPS...
2012: Respiratory Medicine Case Reports
Emmanuelle Ecochard-Dugelay, Virginie Lambert, Jean-Marc Schleich, Mathieu Duché, Emmanuel Jacquemin, Olivier Bernard
OBJECTIVE: Portopulmonary hypertension (POPH) is a known complication of cirrhosis in adults, but there is little information on its incidence and outcome in children with liver disease. We report 14 patients with POPH and present a synthesis of the medical literature. METHODS: Diagnosis of POPH in the 14 patients was based on right-sided heart catheterization displaying mean pulmonary artery pressure (mPAP) >25 mmHg, indexed pulmonary vascular resistances >3 Wood units · m, and pulmonary wedge pressure <15 mmHg...
September 2015: Journal of Pediatric Gastroenterology and Nutrition
Hilary M DuBrock, Richard N Channick, Michael J Krowka
Portopulmonary hypertension (POPH) is a complication of portal hypertension characterized by pulmonary vasoconstriction and vascular remodeling that can lead to right heart failure and death. Differentiation of POPH from other causes of pulmonary hypertension, such as volume overload or a hyperdynamic high flow state, is critical because a diagnosis of POPH has significant implications for liver transplant risk stratification, Model for End Stage Liver Disease exception points, and the use of pulmonary arterial hypertension-(PAH) specific therapy...
July 2015: Expert Review of Gastroenterology & Hepatology
Rodrigo Liberal, Charlotte R Grant, Rui Baptista, Guilherme Macedo
Porto-pulmonary hypertension (PoPH) is a rare but threatening vasculopathy, defined by the presence of pulmonary arterial hypertension (PAH) in the setting of portal hypertension. Although most commonly observed in cirrhotic patients, those with non-cirrhotic portal hypertension are also at risk of developing it. Little is known about the mechanisms by which PAH develop in patients with portal hypertension, but genetic factors, pulmonary vascular wall shear stress, and a dysregulation of vasoactive, proliferative and inflammatory mediators might be involved...
April 2015: Clinics and Research in Hepatology and Gastroenterology
Muhammad Faisal, Furqan Siddiqi, Ahmad Alkaddour, Abubakr A Bajwa, Adil Shujaat
BACKGROUND: Little is known about the effect of pulmonary arterial hypertension (PAH) specific therapy on pulmonary hemodynamics and exercise capacity in patients with portopulmonary hypertension (PoPH) because such patients are usually excluded from randomized clinical trials (RCT) of such therapy. METHODS: We searched PUBMED using the terms "(Therapy/Broad (filter)) AND (portopulmonary hypertension)." We included studies that met the following criteria: ≥5 patients, AND PoPH confirmed by right heart catheterization (RHC), AND follow-up RHC data, AND/OR baseline and follow-up 6MWD available...
2014: Pulmonary Medicine
Yu Yamashita, Ichizo Tsujino, Takahiro Sato, Asuka Yamada, Taku Watanabe, Hiroshi Ohira, Masaharu Nishimura
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 (PoPH) 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 PoPH patients who were successfully treated with a combination of two oral vasodilators used to treat PAH: ambrisentan and tadalafil...
November 27, 2014: World Journal of Hepatology
Yu Yang, Tzu-Ping Ko, Long Liu, Jianghua Li, Chun-Hsiang Huang, Jian Chen, Rey-Ting Guo, Guocheng Du
Oxidized polyvinyl alcohol hydrolase (OPH) catalyzes the cleavage of C-C bond in β-diketone. It belongs to the α/β-hydrolase family and contains a unique lid region that covers the active site. The lid is the most variable region when pOPH from Pseudomonas sp. VM15C and sOPH from Sphingopyxis sp. 113P3 are compared. The wild-type enzymes and the pOPH mutants W255A, W255Y and W255F were analyzed for lipase activity by using p-nitrophenyl (pNP) esters as the substrates. The wild-type enzymes showed increased Km and decreased kcat/Km with the acyl chain length, and the mutants showed reduced kcat/Km for pNP acetate, indicating the importance of Trp255 in sequestering the active site from solvent...
September 26, 2014: Biochemical and Biophysical Research Communications
Laurent Savale, Caroline Sattler, Olivier Sitbon
Portopulmonary hypertension (PoPH) is defined by the combination of portal hypertension and precapillary pulmonary hypertension (mPAP ≥ 25 mmHg, PCWP < 15 mmHg and PVR > 3 Wood units). PoPH is characterised by pathobiological mechanisms that are similar to other forms of pulmonary arterial hypertension. Prevalence of PoPH is estimated at 0.5-5% among patients with portal hypertension with or without cirrhosis. Treatment strategies most commonly employed for PoPH patients are based on recommendations for idiopathic PAH management...
September 2014: La Presse Médicale
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