{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T15:45:10Z","timestamp":1792079110805,"version":"4.4.0"},"reference-count":38,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:sec>\n            <jats:title>Background.<\/jats:title>\n            <jats:p>Portopulmonary hypertension is present in an estimated 5.3% to 8.5% of liver transplant candidates. Untreated, 5-year survival is estimated between 14% and 28%. Moderate-severe disease is a contraindication to liver transplant due to the high perioperative mortality, but patients optimized with pulmonary vasodilator therapy can become eligible for transplant. There is minimal data regarding posttransplant outcomes and ability to discontinue pulmonary vasodilator therapy posttransplant.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods.<\/jats:title>\n            <jats:p>We performed a single-center retrospective analysis to evaluate long-term outcomes of patients with moderate-severe portopulmonary hypertension who were optimized with pulmonary vasodilator therapy, became eligible for liver transplant, and subsequently underwent transplant. We identified 24 patients optimized with pulmonary vasodilator therapy who underwent subsequent liver transplantation and 25 patients who were treated with pulmonary vasodilator therapy alone.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results.<\/jats:title>\n            <jats:p>In the transplanted cohort, 1-year survival from portopulmonary hypertension diagnosis date: 95.8%, 3-year survival: 90.9%, and 5-year survival: 90.9%. Posttransplant; 1-, 3-, and 5-year survival was 86.9%. Among transplanted patients, 41.6% (10\/24) were optimized with nonparenteral therapy. Following transplantation, 100% (14\/14) of the surviving patients were able to discontinue parenteral therapy; median time: 7.2 months (interquartile range: 5.1\u20138.9 mo), while 61.9% (13\/21) were able to discontinue pulmonary vasodilator therapy altogether; median time: 13.9 months (interquartile range: 5.1\u201317.6 mo).<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions.<\/jats:title>\n            <jats:p>Patients who are optimized with pulmonary vasodilator therapy before liver transplant can have excellent long-term outcomes posttransplant. Oral pulmonary vasodilator therapy can be effective treatment to qualify a patient for transplant, and the majority are able to wean from pulmonary vasodilator therapy entirely posttransplant.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1097\/tp.0000000000003248","type":"journal-article","created":{"date-parts":[[2020,4,1]],"date-time":"2020-04-01T17:41:54Z","timestamp":1585762914000},"page":"346-353","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":34,"title":["Long-term Outcomes and Survival in Moderate-severe Portopulmonary Hypertension After Liver Transplant"],"prefix":"10.1097","volume":"105","author":[{"given":"Corey J.","family":"Sadd","sequence":"first","affiliation":[{"name":"Department of Medicine, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fauzia","family":"Osman","sequence":"additional","affiliation":[{"name":"Department of Medicine, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhanhai","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Medicine, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Chybowski","sequence":"additional","affiliation":[{"name":"Department of Medicine, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Decker","sequence":"additional","affiliation":[{"name":"School of Pharmacy, University of Wisconsin-Madison, Madison, WI."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruce","family":"Henderson","sequence":"additional","affiliation":[{"name":"Department of Medicine, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kara N.","family":"Goss","sequence":"additional","affiliation":[{"name":"Department of Medicine, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura L.","family":"Hammel","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James R.","family":"Runo","sequence":"additional","affiliation":[{"name":"Department of Medicine, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2020,3,30]]},"reference":[{"key":"R1-20250604","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1164\/rccm.200510-1668OC","article-title":"Pulmonary arterial hypertension in France: results from a national registry.","volume":"173","author":"Humbert","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"key":"R2-20250604","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1378\/chest.09-1140","article-title":"Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry.","volume":"137","author":"Badesch","year":"2010","journal-title":"Chest"},{"issue":"12 Suppl 3","key":"R3-20250604","doi-asserted-by":"crossref","first-page":"S114","DOI":"10.1002\/lt.20975","article-title":"Model for end-stage liver disease (MELD) exception for portopulmonary hypertension.","volume":"12","author":"Krowka","year":"2006","journal-title":"Liver 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The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS).","volume":"46","author":"Gali\u00e8","year":"2015","journal-title":"Eur Respir J"},{"key":"R9-20250604","doi-asserted-by":"crossref","first-page":"1440","DOI":"10.1097\/TP.0000000000001229","article-title":"International liver transplant society practice guidelines: diagnosis and management of hepatopulmonary syndrome and portopulmonary hypertension.","volume":"100","author":"Krowka","year":"2016","journal-title":"Transplantation"},{"key":"R10-20250604","doi-asserted-by":"crossref","first-page":"1801913","DOI":"10.1183\/13993003.01913-2018","article-title":"Haemodynamic definitions and updated clinical classification of pulmonary hypertension.","volume":"53","author":"Simonneau","year":"2019","journal-title":"Eur Respir J"},{"key":"R11-20250604","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1053\/jlts.2000.6356","article-title":"Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation.","volume":"6","author":"Krowka","year":"2000","journal-title":"Liver Transpl"},{"key":"R12-20250604","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1053\/jlts.2001.26057","article-title":"Liver transplantation in patients with severe portopulmonary hypertension treated with preoperative chronic intravenous epoprostenol.","volume":"7","author":"Tan","year":"2001","journal-title":"Liver Transpl"},{"key":"R13-20250604","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1002\/lt.21174","article-title":"Long-term follow-up of portopulmonary hypertension: effect of treatment with epoprostenol.","volume":"13","author":"Fix","year":"2007","journal-title":"Liver Transpl"},{"key":"R14-20250604","doi-asserted-by":"crossref","first-page":"1144","DOI":"10.1002\/hep.26972","article-title":"Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation.","volume":"59","author":"Martin","year":"2014","journal-title":"Hepatology"},{"key":"R15-20250604","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.1002\/lt.20494","article-title":"Portopulmonary hypertension and the issue of survival.","volume":"11","author":"Krowka","year":"2005","journal-title":"Liver Transpl"},{"key":"R16-20250604","doi-asserted-by":"crossref","first-page":"2445","DOI":"10.1111\/j.1600-6143.2008.02384.x","article-title":"Survival in portopulmonary hypertension: Mayo Clinic experience categorized by treatment subgroups.","volume":"8","author":"Swanson","year":"2008","journal-title":"Am J Transplant"},{"key":"R17-20250604","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1378\/chest.11-0160","article-title":"Portopulmonary hypertension: a report from the US-based REVEAL Registry.","volume":"141","author":"Krowka","year":"2012","journal-title":"Chest"},{"key":"R18-20250604","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1016\/j.healun.2016.12.014","article-title":"Survival in portopulmonary hypertension: outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry.","volume":"36","author":"Sithamparanathan","year":"2017","journal-title":"J Heart Lung Transplant"},{"key":"R19-20250604","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1002\/lt.23870","article-title":"Long-term follow-up of portopulmonary hypertension patients after liver transplantation.","volume":"20","author":"Khaderi","year":"2014","journal-title":"Liver Transpl"},{"key":"R20-20250604","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1097\/TP.0000000000001981","article-title":"Does portopulmonary hypertension impede liver transplantation in cirrhotic patients? A French multicentric retrospective study.","volume":"102","author":"Reymond","year":"2018","journal-title":"Transplantation"},{"key":"R21-20250604","doi-asserted-by":"crossref","first-page":"1683","DOI":"10.1002\/hep.28990","article-title":"Long-term outcome in liver transplantation candidates with portopulmonary hypertension.","volume":"65","author":"Savale","year":"2017","journal-title":"Hepatology"},{"key":"R22-20250604","doi-asserted-by":"crossref","first-page":"1258","DOI":"10.1111\/j.1600-6143.2006.01701.x","article-title":"The impact of treatment of portopulmonary hypertension on survival following liver transplantation.","volume":"7","author":"Ashfaq","year":"2007","journal-title":"Am J Transplant"},{"key":"R24-20250604","doi-asserted-by":"crossref","first-page":"2081","DOI":"10.1111\/ajt.12783","article-title":"MELD exceptions for portopulmonary hypertension: current policy and future implementation.","volume":"14","author":"Goldberg","year":"2014","journal-title":"Am J 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