Advertisement
Review Article| Volume 27, ISSUE 1, P71-84, February 2023

Portopulmonary Hypertension

Published:October 18, 2022DOI:https://doi.org/10.1016/j.cld.2022.08.002

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribers receive full online access to your subscription and archive of back issues up to and including 2002.

      Content published before 2002 is available via pay-per-view purchase only.

      Subscribe:

      Subscribe to Clinics in Liver Disease
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Simonneau G.
        • Montani D.
        • Celermajer D.S.
        • et al.
        Haemodynamic definitions and updated clinical classification of pulmonary hypertension.
        Eur Respir J. 2019; 53: 1801913https://doi.org/10.1183/13993003.01913-2018
        • Goldberg D.S.
        • Batra S.
        • Sahay S.
        • et al.
        MELD exceptions for portopulmonary hypertension: current policy and future implementation: transplantation in POPH.
        Am J Transpl. 2014; 14: 2081-2087https://doi.org/10.1111/ajt.12783
        • Krowka M.J.
        • Swanson K.L.
        • Frantz R.P.
        • et al.
        Portopulmonary hypertension: results from a 10-year screening algorithm.
        Hepatology. 2006; 44: 1502-1510https://doi.org/10.1002/hep.21431
        • Shen M.
        • Zhang F.
        • Zhang X.
        Pulmonary hypertension in primary biliary cirrhosis: a prospective study in 178 patients.
        Scand J Gastroenterol. 2009; 44: 219-223https://doi.org/10.1080/00365520802400883
        • Kawut S.M.
        • Krowka M.J.
        • Trotter J.F.
        • et al.
        Clinical risk factors for portopulmonary hypertension.
        Hepatol Baltim Md. 2008; 48: 196-203https://doi.org/10.1002/hep.22275
        • Krowka M.J.
        • Miller D.P.
        • Barst R.J.
        • et al.
        Portopulmonary hypertension: a report from the US-based REVEAL Registry.
        Chest. 2012; 141: 906-915https://doi.org/10.1378/chest.11-0160
        • Sithamparanathan S.
        • Nair A.
        • Thirugnanasothy L.
        • et al.
        Survival in portopulmonary hypertension: outcomes of the United Kingdom national pulmonary arterial hypertension registry.
        J Heart Lung Transpl Off Publ Int Soc Heart Transpl. 2017; 36: 770-779https://doi.org/10.1016/j.healun.2016.12.014
        • Lebrec D.
        • Capron J.P.
        • Dhumeaux D.
        • et al.
        Pulmonary hypertension complicating portal hypertension.
        Am Rev Respir Dis. 1979; 120: 849-856https://doi.org/10.1164/arrd.1979.120.4.849
        • Robalino B.D.
        • Moodie D.S.
        Association between primary pulmonary hypertension and portal hypertension: analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations.
        J Am Coll Cardiol. 1991; 17: 492-498https://doi.org/10.1016/s0735-1097(10)80121-4
        • Medarov B.I.
        • Chopra A.
        • Judson M.A.
        Clinical aspects of portopulmonary hypertension.
        Respir Med. 2014; 108: 943-954https://doi.org/10.1016/j.rmed.2014.04.004
        • Thomas C.
        • Glinskii V.
        • de Jesus Perez V.
        • et al.
        Portopulmonary hypertension: from bench to bedside.
        Front Med. 2020; 7: 569413https://doi.org/10.3389/fmed.2020.569413
        • Vallabhajosula S.
        • Radhi S.
        • Cevik C.
        • et al.
        Hyperthyroidism and pulmonary hypertension: an important association.
        Am J Med Sci. 2011; 342: 507-512https://doi.org/10.1097/MAJ.0b013e31821790f4
        • Baeyens N.
        • Bandyopadhyay C.
        • Coon B.G.
        • et al.
        Endothelial fluid shear stress sensing in vascular health and disease.
        J Clin Invest. 2016; 126: 821-828https://doi.org/10.1172/JCI83083
        • Garg A.
        • Armstrong W.F.
        Echocardiography in liver transplant candidates.
        JACC Cardiovasc Imaging. 2013; 6: 105-119https://doi.org/10.1016/j.jcmg.2012.11.002
        • Taleb M.
        • Khuder S.
        • Tinkel J.
        • et al.
        The diagnostic accuracy of Doppler echocardiography in assessment of pulmonary artery systolic pressure: a meta-analysis.
        Echocardiography. 2013; 30: 258-265https://doi.org/10.1111/echo.12061
        • Silverton N.
        • Meineri M.
        • Djaiani G.
        The controversy of right ventricular systolic pressure: is it time to abandon the pulmonary artery catheter?.
        Anaesthesia. 2015; 70: 241-244https://doi.org/10.1111/anae.12939
        • DeMartino E.S.
        • Cartin-Ceba R.
        • Findlay J.Y.
        • et al.
        Frequency and outcomes of patients with increased mean pulmonary artery pressure at the time of liver transplantation.
        Transplantation. 2017; 101: 101-106https://doi.org/10.1097/TP.0000000000001517
        • Rich J.D.
        • Shah S.J.
        • Swamy R.S.
        • et al.
        Inaccuracy of Doppler echocardiographic estimates of pulmonary artery pressures in patients with pulmonary hypertension.
        Chest. 2011; 139: 988-993https://doi.org/10.1378/chest.10-1269
        • DuBrock H.M.
        • Salgia R.J.
        • Sussman N.L.
        • et al.
        Portopulmonary hypertension: a survey of practice patterns and provider attitudes.
        Transpl Direct. 2019; 5: e456https://doi.org/10.1097/TXD.0000000000000900
        • Martin P.
        Evaluation for liver transplantation in adults.
        Pract Guideline by AASLD Am Soc Transplant. 2013; 98 (Published online 2013)
        • Krowka M.J.
        • Fallon M.B.
        • Kawut S.M.
        • et al.
        International liver transplant society practice guidelines: diagnosis and management of hepatopulmonary syndrome and portopulmonary hypertension.
        Transplantation. 2016; 100: 1440-1452https://doi.org/10.1097/TP.0000000000001229
        • Galiè N.
        • Humbert M.
        • Vachiery J.L.
        • et al.
        2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: 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)endorsed by: association for European paediatric and congenital cardiology (AEPC), international society for heart and lung transplantation (ISHLT).
        Eur Respir J. 2015; 46: 903-975https://doi.org/10.1183/13993003.01032-2015
        • Krowka M.
        Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation.
        Liver Transpl. 2000; 6: 443-450https://doi.org/10.1053/jlts.2000.6356
        • Aithal G.P.
        • Palaniyappan N.
        • China L.
        • et al.
        Guidelines on the management of ascites in cirrhosis.
        Gut. 2021; 70: 9-29https://doi.org/10.1136/gutjnl-2020-321790
        • Provencher S.
        • Herve P.
        • Jais X.
        • et al.
        Deleterious effects of β-blockers on exercise capacity and hemodynamics in patients with portopulmonary hypertension.
        Gastroenterology. 2006; 130: 120-126https://doi.org/10.1053/j.gastro.2005.10.013
        • Peacock A.
        • Ross K.
        Pulmonary hypertension: a contraindication to the use of -adrenoceptor blocking agents.
        Thorax. 2010; 65: 454-455https://doi.org/10.1136/thx.2008.111955
        • Soulaidopoulos S.
        Pulmonary manifestations of chronic liver disease: a comprehensive review.
        Ann Gastroenterol. 2020; https://doi.org/10.20524/aog.2020.0474
        • Klinger J.R.
        • Kadowitz P.J.
        The nitric oxide pathway in pulmonary vascular disease.
        Am J Cardiol. 2017; 120: S71-S79https://doi.org/10.1016/j.amjcard.2017.06.012
        • Reichenberger F.
        • Voswinckel R.
        • Steveling E.
        • et al.
        Sildenafil treatment for portopulmonary hypertension.
        Eur Respir J. 2006; 28: 563-567https://doi.org/10.1183/09031936.06.00030206
        • Hollatz T.J.
        • Musat A.
        • Westphal S.
        • et al.
        Treatment with sildenafil and treprostinil allows successful liver transplantation of patients with moderate to severe portopulmonary hypertension.
        Liver Transpl. 2012; 18: 686-695https://doi.org/10.1002/lt.23407
        • Cartin-Ceba R.
        • Halank M.
        • Ghofrani H.
        • et al.
        Riociguat treatment for portopulmonary hypertension: a subgroup analysis from the PATENT-1/-2 studies.
        Pulm Circ. 2018; 8: 1-4https://doi.org/10.1177/2045894018769305
        • Hynynen M.
        • Khalil R.
        The vascular endothelin system in hypertension - recent patents and discoveries.
        Recent Patents Cardiovasc Drug Discov. 2006; 1: 95-108https://doi.org/10.2174/157489006775244263
        • Faisal M.
        • Siddiqi F.
        • Alkaddour A.
        • et al.
        Effect of PAH specific therapy on pulmonary hemodynamics and six-minute walk distance in portopulmonary hypertension: a systematic review and meta-analysis.
        Pulm Med. 2014; 2014: 1-7https://doi.org/10.1155/2014/528783
      1. Current and emerging therapeutic approaches to pulmonary hypertension.
        Rev Cardiovasc Med. 2020; 21: 163https://doi.org/10.31083/j.rcm.2020.02.597
        • Sitbon O.
        • Bosch J.
        • Cottreel E.
        • et al.
        Macitentan for the treatment of portopulmonary hypertension (PORTICO): a multicentre, randomised, double-blind, placebo-controlled, phase 4 trial.
        Lancet Respir Med. 2019; 7: 594-604https://doi.org/10.1016/S2213-2600(19)30091-8
        • Sitbon O.
        • Vonk Noordegraaf A.
        Epoprostenol and pulmonary arterial hypertension: 20 years of clinical experience.
        Eur Respir Rev. 2017; 26: 160055https://doi.org/10.1183/16000617.0055-2016
        • Gomberg-Maitland M.
        • Olschewski H.
        Prostacyclin therapies for the treatment of pulmonary arterial hypertension.
        Eur Respir J. 2008; 31: 891-901https://doi.org/10.1183/09031936.00097107
        • DuBrock H.M.
        • Goldberg D.S.
        • Sussman N.L.
        • et al.
        Predictors of waitlist mortality in portopulmonary hypertension.
        Transplantation. 2017; 101: 1609-1615https://doi.org/10.1097/TP.0000000000001666
        • Aggarwal M.
        • Li M.
        • Bhardwaj A.
        • et al.
        Predictors of survival in portopulmonary hypertension: a 20-year experience.
        Eur J Gastroenterol Hepatol. 2022; 34: 449-456
        • Jose A.
        • Shah S.A.
        • Anwar N.
        • et al.
        Pulmonary vascular resistance predicts mortality and graft failure in transplantation patients with portopulmonary hypertension.
        Liver Transpl. 2021; 27: 1811-1823https://doi.org/10.1002/lt.26091
        • DuBrock H.M.
        • Runo J.R.
        • Sadd C.J.
        • et al.
        Outcomes of liver transplantation in treated portopulmonary hypertension patients with a mean pulmonary arterial pressure ≥35 mm Hg.
        Transpl Direct. 2020; 6: e630https://doi.org/10.1097/TXD.0000000000001085
        • Cartin-Ceba R.
        • Burger C.
        • Swanson K.
        • et al.
        Clinical outcomes after liver transplantation in patients with portopulmonary hypertension.
        Transplantation. 2020; 105: 2283-2290
        • Navarro-Vergara D.I.
        • Roldan-Valadez E.
        • Cueto-Robledo G.
        • et al.
        Portopulmonary hypertension: prevalence, clinical and hemodynamic features.
        Curr Probl Cardiol. 2021; 46: 100747https://doi.org/10.1016/j.cpcardiol.2020.100747
      2. Enhancements to the national liver review board, OPTN liver and intestinal organ transplantation committee.
        (Available at:) (Accessed June 1, 2022)
        • Deroo R.
        • Trépo E.
        • Holvoet T.
        • et al.
        Vasomodulators and liver transplantation for portopulmonary hypertension: evidence from a systematic review and meta-analysis.
        Hepatology. 2020; 72: 1701-1716https://doi.org/10.1002/hep.31164
        • Barbas A.S.
        • Schroder J.N.
        • Borle D.P.
        • et al.
        Planned initiation of venoarterial extracorporeal membrane oxygenation prior to liver transplantation in a patient with severe portopulmonary hypertension.
        Liver Transpl. 2021; 27: 760-762https://doi.org/10.1002/lt.25871
        • DuBrock H.M.
        • Channick R.N.
        • Krowka M.J.
        What’s new in the treatment of portopulmonary hypertension?.
        Expert Rev Gastroenterol Hepatol. 2015; 9: 983-992https://doi.org/10.1586/17474124.2015.1035647
        • Savale L.
        • Sattler C.
        • Coilly A.
        • et al.
        Long-term outcome in liver transplantation candidates with portopulmonary hypertension: savale et al.
        Hepatology. 2017; 65: 1683-1692https://doi.org/10.1002/hep.28990
        • Savale L.
        • Guimas M.
        • Ebstein N.
        • et al.
        Portopulmonary hypertension in the current era of pulmonary hypertension management.
        J Hepatol. 2020; 73: 130-139https://doi.org/10.1016/j.jhep.2020.02.021
        • Cartin-Ceba R.
        • Burger C.
        • Swanson K.
        • et al.
        Clinical outcomes after liver transplantation in patients with portopulmonary hypertension.
        Transplantation. 2021; 105: 2283-2290