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P Rajaram, A Parekh, M Fisher, J Kempker, R Subramanian
BACKGROUND: In potential liver transplant candidates, pulmonary vascular diseases, including portopulmonary hypertension (PoPH) and pulmonary venous hypertension (PVH), can be associated with high morbidity and mortality. Although there are clear guidelines regarding management and transplant listing criteria for patients with PoPH, the listing criteria for PVH are not well defined. OBJECTIVE: The aim of this study was to describe and compare the perioperative and postoperative morbidity and mortality associated with PoPH and PVH in patients undergoing liver transplantation...
March 2017: Transplantation Proceedings
Hilary M DuBrock, David S Goldberg, Norman L Sussman, Sonja D Bartolome, Zakiyah Kadry, Reena J Salgia, David C Mulligan, Walter K Kremers, Steven M Kawut, Michael J Krowka, Richard N Channick
BACKGROUND: The current Organ Procurement Transplantation Network (OPTN) policy grants Model for End Stage Liver Disease (MELD) exception points to patients with portopulmonary hypertension (POPH), but potentially important factors, such as severity of liver disease and pulmonary hypertension, are not included in the exception score, and may affect survival. The purpose of this study was to identify significant predictors of waitlist mortality in patients with POPH. METHODS: We performed a retrospective cohort study of patients in the OPTN database with hemodynamics consistent with POPH [defined as mean pulmonary arterial pressure (mPAP) >25mmHg and pulmonary vascular resistance (PVR) ≥240 dynes-s-cm] who were approved for a POPH MELD exception between 2006 and 2014...
February 15, 2017: Transplantation
Sasiharan Sithamparanathan, Arun Nair, Logan Thirugnanasothy, J Gerry Coghlan, Robin Condliffe, Konstantinos Dimopoulos, Charlie A Elliot, Andrew J Fisher, Sean Gaine, J Simon R Gibbs, Michael A Gatzoulis, Clive E Handler, Luke S Howard, Martin Johnson, David G Kiely, James L Lordan, Andrew J Peacock, Joanna Pepke-Zaba, Benjamin E Schreiber, Karen K K Sheares, Stephen J Wort, Paul A Corris
BACKGROUND: Portopulmonary hypertension (PoPH) is a rare condition associated with poor survival, and the effect of modern therapies that target pulmonary arterial hypertension (PAH) on long-term outcome is unknown. This study investigated the baseline characteristics and survival in the cohort of patients diagnosed with PoPH in the United Kingdom National Pulmonary Hypertension Service. METHODS: A retrospective review was conducted of all incident treatment-naïve patients with PoPH within the United Kingdom national registry diagnosed between January 2001 and December 2010...
December 31, 2016: Journal of Heart and Lung Transplantation
Yoshito Ogihara, Norikazu Yamada, Kaoru Dohi, Akimasa Matsuda, Satoshi Ota, Ken Ishikura, Mashio Nakamura, Masaaki Ito
The present study reported a case of portopulmonary hypertension (POPH) that was secondary to underlying liver cirrhosis in a 58-year-old woman, who was successfully treated with low-dose tolvaptan. The patient had suffered from refractory peripheral edema and electrolyte abnormalities, including severe hypokalemia, under the combination therapy of sildenafil, ambrisentan, furosemide and spironolactone. Subsequent to the initiation of low-dose tolvaptan at 3.75 mg/day with concurrent de-escalation of the dose of furosemide, the daily urine volume increased, peripheral edema improved and the serum potassium level increased immediately...
January 2017: Experimental and Therapeutic Medicine
Hilary M DuBrock, Josanna M Rodriguez-Lopez, Barbara L LeVarge, Michael P Curry, Paul A VanderLaan, Zsuzsanna K Zsengeller, Elizabeth Pernicone, Ioana R Preston, Paul B Yu, Ivana Nikolic, Dihua Xu, Ravi I Thadhani, Richard N Channick, S Ananth Karumanchi
Portopulmonary hypertension (POPH) is a poorly understood complication of liver disease associated with significant morbidity and mortality. We sought to identify novel biomarkers of POPH disease presence and severity. We performed a prospective, multicenter, case-control study involving patients with liver disease undergoing right heart catheterization. POPH cases were defined as a mean pulmonary arterial pressure (mPAP) ≥25 mmHg and pulmonary vascular resistance (PVR) >240 dynes˙s˙cm(-5). Plasma samples were collected from the systemic and pulmonary circulation, and antibody microarray was used to identify biomarkers...
December 2016: Pulmonary Circulation
Laurent Savale, Caroline Sattler, Audrey Coilly, Filoména Conti, Sébastien Renard, Claire Francoz, Hélène Bouvaist, Cyrille Feray, Patrick Borentain, Xavier Jaïs, David Montani, Florence Parent, Caroline O'Connell, Philippe Hervé, Marc Humbert, Gérald Simonneau, Didier Samuel, Yvon Calmus, Christophe Duvoux, François Durand, Jean Charles Duclos-Vallée, Olivier Sitbon
Portopulmonary hypertension (PoPH) is diagnosed in 2-6% of liver transplantation (LT) candidates. We studied outcomes of candidates for LT suffering from PoPH. Data were collected retrospectively from a prospective registry. Pulmonary hemodynamic variables were collected at the time of PoPH diagnosis, at last evaluation before LT, and within 6 months and beyond 6 months after LT. Forty-nine patients (35 males, 48 ± 8 years) were analyzed (median Model for End-Stage Liver Disease score 20). At baseline, mean pulmonary artery pressure (mPAP) was 44 ± 10 mm Hg (range 26-73 mm Hg), cardiac index was 3...
May 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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
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