Abstract
Portopulmonary hypertension (PPHT) is defined as precapillary pulmonary hypertension accompanied by hepatic disease or portal hypertension. Pulmonary hypertension results from excessive pulmonary vascular remodeling and vasoconstriction. These histological alterations have been indistinguishable from those of other forms of pulmonary arterial hypertension. Factors involved in the pathogenesis of PPHT include volume overload, hyperdynamic circulation, and circulating vasoactive mediators. The disorder has a substantial impact on survival and requires focused treatment. Liver transplantation in patients with moderate to severe PPHT is associated with a significantly reduced survival rate. The best medical treatment for patients with PPHT is controversial; most authors currently regard continuous intravenous application of prostacyclin as the treatment of choice for patients with severe PPHT. There is only very limited reported experience with inhaled prostacyclin or its analog, iloprost. Increasing evidence of the efficacy of the endothelin-receptor antagonist bosentan and of the phosphodiesterase-5 inhibitor sildenafil is emerging in highly selected patients with PPHT. In the future, a combination therapy of the above-mentioned agents might become a therapeutic option. Other agents such as β-blockers seem to be harmful to patients with moderate to severe portopulmonary hypertension. Up-to-date, randomized, double-blind, controlled clinical trials are lacking and are needed urgently.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
DN Amarapurkar AD Amarapurkar (2002) ArticleTitleExtrahepatic manifestations of viral hepatitis Ann Hepatol 1 192–5 Occurrence Handle15280806
RA Wilson (1997) ArticleTitleExtrahepatic manifestations of chronic viral hepatitis Am J Gastroenterol 92 4–17
H Mujagic B Prnjavorac Z Mujagic G Festa (2003) ArticleTitleAlcohol in liver disease is a causative factor for development of allergic skin manifestations Med Arh 57 273–8 Occurrence Handle15022578
R Rodriguez-Roisin MJ Krowka Herve Ph MB Fallon ERS Task (2004) ArticleTitleForce Pulmonary-Hepatic Vascular Disorders (PHD) Scientific Committee. Pulmonary-hepatic vascular disorders (PHD) Eur Respir J 24 861–80 Occurrence Handle15516683 Occurrence Handle1:STN:280:DC%2BD2crksFSjsQ%3D%3D Occurrence Handle10.1183/09031936.04.00010904
R Budhiraja PM Hassoun (2003) ArticleTitlePortopulmonary hypertension: a tale of two circulations Chest 123 562–76 Occurrence Handle12576381 Occurrence Handle10.1378/chest.123.2.562
MJ Krowka (1997) ArticleTitleHepatopulmonary syndrome versus portopulmonary hypertension: distinctions and dilemmas Hepatology 25 1282–4 Occurrence Handle9141454 Occurrence Handle1:STN:280:DyaK2s3os1artQ%3D%3D Occurrence Handle10.1002/hep.510250540
AL Gerbes S Moller V Gulberg JH Henriksen (1995) ArticleTitleEndothelin-1 and -3 plasma concentrations in patients with cirrhosis: a role of splanchnic and renal passage and liver function Hepatology 21 735–9 Occurrence Handle7875671 Occurrence Handle1:CAS:528:DyaK2MXltlent7s%3D Occurrence Handle10.1016/0270-9139(95)90526-X
G Cremona TW Higenbottam V Mayoral G Alexander E Demoncheaux C Borland et al. (1995) ArticleTitleElevated exhaled nitric oxide in patients with hepatopulmonary syndrome Eur Respir J 8 1883–5 Occurrence Handle8620957 Occurrence Handle1:STN:280:DyaK287pt1Giug%3D%3D Occurrence Handle10.1183/09031936.95.08111883
Y Katsuta H Honma XJ Zhang M Ohsuga H Komeichi S Shimizu et al. (2005) ArticleTitlePulmonary blood transit time and impaired arterial oxygenation in patients with chronic liver disease J Gastroenterol 40 57–63 Occurrence Handle15692790 Occurrence Handle1:CAS:528:DC%2BD2MXhtVamsb0%3D Occurrence Handle10.1007/s00535-004-1495-6
FA Mantz SuffixJr E Craige (1951) ArticleTitlePortal axis thrombosis with spontaneous portacaval shunt and resultant cor pulmonale AMA Arch Pathol 52 91–7 Occurrence Handle14837570
PJ McDonnel PA Toye GM Hutchins (1983) ArticleTitlePrimary pulmonary hypertension and cirrhosis: are they related? Am Rev Respir Dis 127 437–41
E Cheng H Woehlck (1992) ArticleTitlePulmonary artery hypertension complicating anesthesia for liver transplantation Anesthesiology 77 389–92 Occurrence Handle1642361 Occurrence Handle1:STN:280:DyaK38zlt1Sruw%3D%3D
M Castro MJ Krowka R Schroeder KC Beck DJ Plevak SR Rettke et al. (1996) ArticleTitleFrequency and clinical implications of increased pulmonary pressures in liver transplant patients Mayo Clin Proc 71 543–51 Occurrence Handle8642882 Occurrence Handle1:STN:280:DyaK283jt1altg%3D%3D Occurrence Handle10.4065/71.6.543
FS Benjaminov M Prentice KW Sniderman S Siu P Liu F Wong (2003) ArticleTitlePortopulmonary hypertension in decompensated cirrhosis with refractory ascites Gut 52 1355–62 Occurrence Handle12912870 Occurrence Handle1:STN:280:DC%2BD3svgsVWhtA%3D%3D Occurrence Handle10.1136/gut.52.9.1355
S Rich (1998) Primary pulmonary hypertension: executive summary from the world symposium on primary pulmonary hypertension 1998 World Health Organization Geneva
LJ Rubin (1997) ArticleTitlePrimary pulmonary hypertension N Engl J Med 336 111–7 Occurrence Handle8988890 Occurrence Handle1:STN:280:DyaK2s7jtVSlug%3D%3D Occurrence Handle10.1056/NEJM199701093360207
R Naeije (2003) Pulmonary Circulation CJ Gibson DM Geddes U Costabel PJ Sterk B Corris (Eds) Respiratory medicine Vol. I EditionNumber3rd ed Saunders London 147–57
MJ Krowka (2000) ArticleTitleHepatopulmonary syndromes Gut 46 1–4 Occurrence Handle10601042 Occurrence Handle1:STN:280:DC%2BD3c%2FmvFCrtQ%3D%3D Occurrence Handle10.1136/gut.46.1.1
MJ Krowka (2005) ArticleTitleHepatopulmonary syndrome and portopulmonary hypertension: implications for liver transplantation Clin Chest Med 26 587–97 Occurrence Handle16263398
P Herve D Lebrec F Brenot G Simonneau M Humbert O Sitbon et al. (1998) ArticleTitlePulmonary vascular disorders in portal hypertension Eur Respir J 11 1153–66 Occurrence Handle9648972 Occurrence Handle1:STN:280:DyaK1czhtFejtA%3D%3D Occurrence Handle10.1183/09031936.98.11051153
D Chemla V Castelain P Herve Y Lecarpentier S Brimioulle (2002) ArticleTitleHaemodynamic evaluation of pulmonary hypertension Eur Respir J 20 1314–31 Occurrence Handle12449189 Occurrence Handle1:STN:280:DC%2BD38nosFaqsg%3D%3D Occurrence Handle10.1183/09031936.02.00068002
MS Mandell (2000) ArticleTitleCritical care issues: portopulmonary hypertension Liver Transpl 6 IssueID4 Suppl 1 S36–43 Occurrence Handle10915190 Occurrence Handle1:STN:280:DC%2BD3cvot1ehtg%3D%3D
IO Colle R Moreau E Godinho J Belghiti F Ettori A Cohen-Solal et al. (2003) ArticleTitleDiagnosis of portopulmonary hypertension in candidates for liver transplantation: a prospective study Hepatology 37 401–9 Occurrence Handle12540791 Occurrence Handle10.1053/jhep.2003.50060
M McGoon D Gutterman V Steen R Barst DC McCrory TA Fortin et al. (2004) ArticleTitleScreening, early detecting, and diagnosis of pulmonary arterial hypertension Chest 126 14S–34S Occurrence Handle15249493 Occurrence Handle10.1378/chest.126.1_suppl.14S
M Sakuma S Souma O Kitamukai J Demachi T Takahashi J Suzuki et al. (2005) ArticleTitlePortopulmonary hypertension—hemodynamics, pulmonary angiography, and configuration of the heart Circ J 69 1386–93 Occurrence Handle16247216 Occurrence Handle10.1253/circj.69.1386
PC Kuo JS Plotkin LB Johnson CD Howell JM Laurin ST Bartlett et al. (1997) ArticleTitleDistinctive clinical features of portopulmonary hypertension Chest 112 980–6 Occurrence Handle9377962 Occurrence Handle1:STN:280:DyaK1c%2Fit1Whuw%3D%3D
MM Hoeper MJ Krowka CP Strassburg (2004) ArticleTitlePortopulmonary hypertension and hepatopulmonary syndrome Lancet 363 1461–8 Occurrence Handle15121411 Occurrence Handle10.1016/S0140-6736(04)16107-2
R Naeije (2003) ArticleTitleHepatopulmonary syndrome and portopulmonary hypertension Swiss Med Wkly 133 163–9 Occurrence Handle12715285
A Hadengue MK Benhayoun D Lebrec JP Benhamou (1991) ArticleTitlePulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics Gastroenterology 100 520–8 Occurrence Handle1985048 Occurrence Handle1:STN:280:DyaK3M%2FosFyisw%3D%3D
M Auletta U Oliviero L Iasiuolo G Scherillo S Antoniello (2000) ArticleTitlePulmonary hypertension associated with liver cirrhosis: an echocardiographic study Angiology 51 1013–20 Occurrence Handle11132993 Occurrence Handle1:STN:280:DC%2BD3M%2FotVOitw%3D%3D Occurrence Handle10.1177/000331970005101206
V Shah M Toruner F Haddad G Cadelina A Papapetropoulos K Choo et al. (1999) ArticleTitleImpaired endothelial nitric oxide synthase activity associated with enhanced caveolin binding in experimental cirrhosis in the rat Gastroenterology 117 1222–8 Occurrence Handle10535886 Occurrence Handle1:CAS:528:DyaK1MXnsVCqtrY%3D Occurrence Handle10.1016/S0016-5085(99)70408-7
DC Rockey RA Weisiger (1996) ArticleTitleEndothelin induced contractility of stellate cells from normal and cirrhotic rat liver: implications for regulation of portal pressure and resistance Hepatology 24 233–40 Occurrence Handle8707268 Occurrence Handle1:CAS:528:DyaK28XkvFSju7s%3D Occurrence Handle10.1002/hep.510240137
PS Kamath GM Tyce VM Miller BS Edwards DK Rorie (1999) ArticleTitleEndothelin-1 modulates intrahepatic resistance in a rat model of noncirrhotic portal hypertension Hepatology 30 401–7 Occurrence Handle10421647 Occurrence Handle1:CAS:528:DyaK1MXlt1ajsrg%3D Occurrence Handle10.1002/hep.510300235
MS Mandell BM Groves (1996) ArticleTitlePulmonary hypertension in chronic liver disease Clin Chest Med 17 17–33 Occurrence Handle8665788 Occurrence Handle1:STN:280:DyaK283lsVymtA%3D%3D Occurrence Handle10.1016/S0272-5231(05)70296-3
MJ Krowka WD Edwards (2000) ArticleTitleA spectrum of pulmonary vascular pathology in portopulmonary hypertension Liver Transpl 6 241–2 Occurrence Handle10719028 Occurrence Handle1:STN:280:DC%2BD3c7osFOktw%3D%3D
MJ Krowka MS Mandell MAE Ramsay SM Kawut MB Fallon C Manzarbeitia et al. (2004) ArticleTitleHepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant database Liver Transpl 10 174–82 Occurrence Handle14762853 Occurrence Handle10.1002/lt.20016
MM Hoeper M Halank C Marx G Hoeffken HJ Seyfarth J Schauer et al. (2005) ArticleTitleBosentan therapy for portopulmonary hypertension Eur Resp J 25 502–8 Occurrence Handle1:CAS:528:DC%2BD2MXivVymu70%3D Occurrence Handle10.1183/09031936.05.00080804
S Provencher P Herve X Jais D Lebrec M Humbert G Simonneau et al. (2006) ArticleTitleDeleterious effects of β-blockers on exercise capacity and hemodynamics in patients with portopulmonary hypertension Gastroenterology 130 120–6 Occurrence Handle16401475 Occurrence Handle1:CAS:528:DC%2BD28XhtFCgtbs%3D Occurrence Handle10.1053/j.gastro.2005.10.013
KL Swanson MJ Krowka (2002) ArticleTitleArterial oxygenation associated with portopulmonary hypertension Chest 121 1869–75 Occurrence Handle12065351 Occurrence Handle10.1378/chest.121.6.1869
SM Kawut DB Taichman VN Ahya S Kaplan CL Archer-Chicko SE Kimmel et al. (2005) ArticleTitleHemodynamics and survival of patients with portopulmonary hypertension Liver Transpl 11 1107–11 Occurrence Handle16123953 Occurrence Handle10.1002/lt.20459
MJ Krowka RP Frantz MD McGoon C Severson DJ Plevak RH Wiesner (1999) ArticleTitleImprovement in pulmonary hemodynamics during intravenous epoprostenol (prostacyclin): a study of 15 patients with moderate to severe portopulmonary hypertension Hepatology 30 641–8 Occurrence Handle10462369 Occurrence Handle1:CAS:528:DyaK1MXmtVSktrY%3D Occurrence Handle10.1002/hep.510300307
M Huonker YO Schumacher A Ochs S Sorichter J Keul M Rossle (1999) ArticleTitleCardiac function and haemodynamics in alcoholic cirrhosis and effects of the transjugular intrahepatic portosystemic stent shunt Gut 44 743–8 Occurrence Handle10205217 Occurrence Handle1:STN:280:DyaK1M3itFKqug%3D%3D Occurrence Handle10.1136/gut.44.5.743
L Blendis F Wong (2001) ArticleTitleThe hyperdynamic circulation in cirrhosis: an overview Pharmacol Ther 89 221–31 Occurrence Handle11516477 Occurrence Handle1:CAS:528:DC%2BD3MXksVGruro%3D Occurrence Handle10.1016/S0163-7258(01)00124-3
RM Tuder CD Cool M Yeager L Toraseviciene-Stewart TM Bull NF Voelkel (2001) ArticleTitleThe pathobiology of pulmonary hypertension. Endothelium Clin Chest Med 22 405–18 Occurrence Handle11590837 Occurrence Handle1:STN:280:DC%2BD3MrktlWqtA%3D%3D Occurrence Handle10.1016/S0272-5231(05)70280-X
N Nagasue DK Dahr A Yamanoi Y Emi J Udagawa A Yamamoto et al. (2000) ArticleTitleProduction and release of endothelin-1 from the gut and spleen in portal hypertension due to cirrhosis Hepatology 31 1107–14 Occurrence Handle10796886 Occurrence Handle1:CAS:528:DC%2BD3cXjtlyltbc%3D Occurrence Handle10.1053/he.2000.6596
Y Ling J Zhang B Luo D Song L Liu L Tang et al. (2004) ArticleTitleThe role of endothelin-1 and endothelin B receptor in the pathogenesis of experimental hepatopulmonary syndrome Hepatology 39 1593–602 Occurrence Handle15185300 Occurrence Handle1:CAS:528:DC%2BD2cXlsVWrtr0%3D Occurrence Handle10.1002/hep.20244
M Halank S Miehlke G Höffken A Schmeisser M Schulze RH Strasser (2004) ArticleTitleUse of oral endothelin-receptor antagonist bosentan in the treatment of portopulmonary hypertension Transplantation 77 1775–6 Occurrence Handle15201684 Occurrence Handle10.1097/01.TP.0000122420.86904.89
L Hinterhuber IW Graziadei CM Kahler W Jaschke W Vogel (2004) ArticleTitleEndothelin-receptor antagonist treatment of portopulmonary hypertension Clin Gastroenterol Hepatol 2 1039–42 Occurrence Handle15551258 Occurrence Handle10.1016/S1542-3565(04)00466-5
BD Robalino DS Moodie (1991) ArticleTitleAssociation between primary pulmonary hypertension and portal hypertension: analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations J Am Coll Cardiol 17 492–8 Occurrence Handle1991908 Occurrence Handle1:STN:280:DyaK3M7itlGkuw%3D%3D Occurrence Handle10.1016/S0735-1097(10)80121-4
SS Salvi (1999) ArticleTitleAlpha 1-adrenergic hypothesis for pulmonary hypertension Chest 115 1708–19 Occurrence Handle10378571 Occurrence Handle1:STN:280:DyaK1MzgsV2itA%3D%3D Occurrence Handle10.1378/chest.115.6.1708
RM Tuder CD Cool MW Geraci J Wang SH Abman L Wright et al. (1999) ArticleTitleProstacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension Am J Respir Crit Care Med 159 1925–32 Occurrence Handle10351941 Occurrence Handle1:STN:280:DyaK1M3otVKmsQ%3D%3D
KB Lane RD Machado MW Pauciulo JR Thomson JA Phillips Suffix3rd JE Loyd et al. (2000) ArticleTitleHeterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension: the International PPH Consortium Nat Genet 26 81–4 Occurrence Handle10973254 Occurrence Handle1:CAS:528:DC%2BD3cXmsVKku7Y%3D Occurrence Handle10.1038/79226
RD Machado MW Pauciulo JR Thomson KB Lane NV Morgan L Wheeler et al. (2001) ArticleTitleBMPR2 haploinsufficiency as the inherited molecular mechanism for primary pulmonary hypertension Am J Hum Genet 69 92–102 Occurrence Handle10.1086/316947
JR Thomson RD Machado MW Pauciulo NV Morgan M Humbert GC Elliot et al. (2000) ArticleTitleSporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family J Med Genet 37 741–5 Occurrence Handle11015450 Occurrence Handle1:CAS:528:DC%2BD3cXnvVOisrs%3D Occurrence Handle10.1136/jmg.37.10.741
YY Yang HC Lin WC Lee MC Hou FY Lee FY Chang (2001) ArticleTitlePortopulmonary hypertension: distinctive hemodynamic and clinical manifestations J Gastroenterol 36 181–6 Occurrence Handle11291881 Occurrence Handle1:STN:280:DC%2BD38%2FgslCitA%3D%3D Occurrence Handle10.1007/s005350170126
M Halank C Marx G Hoeffken (2004) ArticleTitleLong term survival in primary pulmonary hypertension Heart 90 e40 Occurrence Handle15201266 Occurrence Handle1:STN:280:DC%2BD2c3pvFynsA%3D%3D Occurrence Handle10.1136/hrt.2004.035725
J Ribas J Angrill JA Barbera JC Garcia-Pagan J Roca J Bosch et al. (1999) ArticleTitleIsosorbide-5-mononitrate in the treatment of pulmonary hypertension associated with portal hypertension Eur Respir J 13 210–2 Occurrence Handle10836350 Occurrence Handle1:STN:280:DC%2BD3czos1Glug%3D%3D Occurrence Handle10.1034/j.1399-3003.1999.13a38.x
M Halank S Miehlke M Kolditz G Hoeffken (2005) ArticleTitlePortopulmonary hypertension (in German with English abstract) Z Gastroenterol 43 677–85 Occurrence Handle16001350 Occurrence Handle1:STN:280:DC%2BD2MzkvFaisA%3D%3D Occurrence Handle10.1055/s-2005-857926
K Ota H Shilo H Kokawa K Kubara T Kim N Akiyoshi et al. (1995) ArticleTitleEffects of nifedipine on hepatic venous pressure gradient and portal vein blood flow in patients with cirrhosis J Gastroenterol Hepatol 10 198–204 Occurrence Handle7787167 Occurrence Handle1:STN:280:DyaK2Mzgs1Oksw%3D%3D
PC Kuo LB Johnson JS Plotkin CD Howell ST Bartlett LJ Rubin (1997) ArticleTitleContinuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension Transplantation 63 604–6 Occurrence Handle9047158 Occurrence Handle1:CAS:528:DyaK2sXhvFKqsL4%3D Occurrence Handle10.1097/00007890-199702270-00020
JS Plotkin PC Kuo LJ Rubin S Gaine CD Howell J Laurin et al. (1998) ArticleTitleSuccessful use of chronic epoprostenol as a bridge to liver transplantation in severe portopulmonary hypertension Transplantation 65 457–9 Occurrence Handle9500616 Occurrence Handle1:CAS:528:DyaK1cXitVGksb8%3D Occurrence Handle10.1097/00007890-199802270-00001
MJ Krowka (2002) ArticleTitleHepatopulmonary syndrome and portopulmonary hypertension Curr Treat Options Cardiovasc Med 4 267–73 Occurrence Handle12003725
JY Findlay DJ Plevak MJ Krowka EM Sack MK Porayko (1999) ArticleTitleProgressive splenomegaly after epoprostenol therapy in portopulmonary hypertension Liver Transpl Surg 5 362–5 Occurrence Handle10477835 Occurrence Handle1:STN:280:DyaK1MvgvV2lsQ%3D%3D Occurrence Handle10.1002/lt.500050517
RA Schroeder AA Rafii JS Plotkin LB Johnson VK Rustgi PC Kuo (2000) ArticleTitleUse of aerosolized inhaled epoprostenol in the treatment of portopulmonary hypertension Transplantation 70 548–50 Occurrence Handle10949204 Occurrence Handle1:CAS:528:DC%2BD3cXmtFKht78%3D Occurrence Handle10.1097/00007890-200008150-00028
M Halank C Marx S Miehlke G Hoeffken (2004) ArticleTitleUse of aerosolized inhaled iloprost in the treatment of portopulmonary hypertension J Gastroenterol 39 1222–3 Occurrence Handle15622492 Occurrence Handle10.1007/s00535-004-1478-7
C Kuntzen V Guelberg AL Gerbes (2005) ArticleTitleUse of a mixed receptor antagonist in portopulmonary hypertension: a safe and effective therapy? Gastroenterology 128 164–8 Occurrence Handle15633133 Occurrence Handle10.1053/j.gastro.2004.09.005
LJ Rubin DB Badesch R Barst N Galie CM Black A Keogh et al. (2002) ArticleTitleBosentan therapy for pulmonary arterial hypertension N Engl J Med 346 896–903 Occurrence Handle11907289 Occurrence Handle1:CAS:528:DC%2BD38Xit1ekur0%3D Occurrence Handle10.1056/NEJMoa012212
H Makisalo A Koivusalo A Vakkuri K Hockerstedt (2004) ArticleTitleSildenafil for portopulmonary hypertension in a patient undergoing liver transplantation Liver Transpl 10 945–50 Occurrence Handle15237383 Occurrence Handle10.1002/lt.20153
R Chua A Keogh M Miyashita (2005) ArticleTitleNovel use of sildenafil in the treatment of portopulmonary hypertension J Heart Lung Transplant 24 498–500 Occurrence Handle15797756 Occurrence Handle10.1016/j.healun.2004.01.017
JL Callejas Rubio J Salmeron Escobar J Gonzalez-Calvin N Ortego Centeno (2006) ArticleTitleSuccessful treatment of severe portopulmonary hypertension in a patient with Child C cirrhosis by sildenafil Liver Transpl 12 690–1 Occurrence Handle16555327 Occurrence Handle10.1002/lt.20748
F Reichenberger R Voswinckel E Steveling B Enke A Kreckel H Olschewski et al. (2006) ArticleTitleSildenafil treatment for portopulmonary hypertension Eur Respir J 28 563–7 Occurrence Handle16807265 Occurrence Handle1:CAS:528:DC%2BD28XhtVWhu7fP Occurrence Handle10.1183/09031936.06.00030206
MM Hoeper I Markevych W Spiekerkoetter T Welte J Niedermeyer (2005) ArticleTitleGoal-oriented treatment and combination therapy for pulmonary arterial hypertension Eur Respir J 26 858–63 Occurrence Handle16264047 Occurrence Handle1:STN:280:DC%2BD2MrosFShtQ%3D%3D Occurrence Handle10.1183/09031936.05.00075305
M Halank M Kolditz S Miehlke S Schiemanck A Schmeisser G Hoeffken (2005) ArticleTitleCombination therapy for portopulmonary hypertension with intravenous iloprost and oral bosentan Wien Med Wochenschr 155 376–80 Occurrence Handle16160926 Occurrence Handle10.1007/s10354-005-0196-y
HP Tan JS Markowitz RA Montgomery WT Merritt AS Klein PJ Thuluvath et al. (2001) ArticleTitleLiver transplantation in patients with severe portopulmonary hypertension treated with preoperative chronic intravenous epoprostenol Liver Transpl 7 745–9 Occurrence Handle11510023 Occurrence Handle1:STN:280:DC%2BD3MvmsVOrsg%3D%3D Occurrence Handle10.1053/jlts.2001.26057
Levy MT, Torzillo P, Bookallil M, Sheil AG, McCaughan GW. Case report: delayed resolution of severe pulmonary hypertension after isolated liver transplantation in a patient with cirrhosis. J Gastroenterol Hepatol 1996;11:734–7
R Schott A Chaouat A Launoy T Pottecher E Weitzenblum (1999) ArticleTitleImprovement of pulmonary hypertension after liver transplantation Chest 115 1748–9 Occurrence Handle10378581 Occurrence Handle1:STN:280:DyaK1MzgsV2htA%3D%3D Occurrence Handle10.1378/chest.115.6.1748
B Koneru S Ahmed AB Weisse GB Grant KA McKim (1994) ArticleTitleResolution of pulmonary hypertension of cirrhosis after liver transplantation Transplantation 58 1133–5 Occurrence Handle7974722 Occurrence Handle1:STN:280:DyaK2M%2FmsFSmsQ%3D%3D
P Taura JC Garcia-Valdecasas J Beltran E Izquierdo M Navasa J Sala-Blanch et al. (1996) ArticleTitleModerate primary pulmonary hypertension in patients undergoing liver transplantation Anesth Analg 83 675–80 Occurrence Handle8831302 Occurrence Handle1:STN:280:DyaK28vivVWquw%3D%3D Occurrence Handle10.1097/00000539-199610000-00003
MA Ramsay BR Simpson AT Nguyen KJ Ramsay C East GB Klintmalm (1997) ArticleTitleSevere pulmonary hypertension in liver transplant candidates Liver Transpl Surg 3 494–500 Occurrence Handle9346791 Occurrence Handle1:STN:280:DyaK1c%2FgtlCisg%3D%3D Occurrence Handle10.1002/lt.500030503
MJ Krowka DJ Plevak JY Findlay CB Rosen RH Wiesner RA Krom (2000) ArticleTitlePulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation Liver Transpl 6 443–50 Occurrence Handle10915166 Occurrence Handle1:STN:280:DC%2BD3M%2FitVOjtQ%3D%3D Occurrence Handle10.1053/jlts.2000.6356
P Starkel A Vera B Gunson D Mutimer (2002) ArticleTitleOutcome of liver transplantation for patients with pulmonary hypertension Liver Transpl 8 382–8 Occurrence Handle11965583 Occurrence Handle10.1053/jlts.2002.31343
PC Kuo JS Plotkin S Gaine RA Schroeder VK Rustgi LJ Rubin et al. (1999) ArticleTitlePortopulmonary hypertension and the liver transplant candidate Transplantation 67 1087–93 Occurrence Handle10232556 Occurrence Handle1:STN:280:DyaK1M3kslGitA%3D%3D Occurrence Handle10.1097/00007890-199904270-00001
J Pirenne G Verleden F Nevens M Delcroix D Van Raemdonck B Meyns et al. (2002) ArticleTitleCombined liver and (heart) lung transplantation in liver transplant candidates with refractory portopulmonary hypertension Transplantation 73 140–2 Occurrence Handle11792993 Occurrence Handle10.1097/00007890-200201150-00025
CM Dennis KD McNeil J Dunning S Stewart PJ Friend G Alexander et al. (1996) ArticleTitleHeart-lung-liver transplantation J Heart Lung Transplant 15 536–8 Occurrence Handle8771510 Occurrence Handle1:STN:280:DyaK28zmsleqtA%3D%3D
D Azoulay Y Castaing A Dennison W Martino D Eyraud H Bismuth (1994) ArticleTitleTransjugular intrahepatic portosystemic shunt worsens the hyperdynamic circulatory state of the cirrhotic patient Hepatology 19 129–32 Occurrence Handle8276348 Occurrence Handle1:STN:280:DyaK2c7gt1KrsA%3D%3D Occurrence Handle10.1016/0270-9139(94)90063-9
P Van der Linden O Le Moine M Ghysels M Ortinez J Deviere (1996) ArticleTitlePulmonary hypertension after transjugular intrahepatic portosystemic shunt: effects on right ventricular function Hepatology 23 982–7 Occurrence Handle8621179 Occurrence Handle1:STN:280:DyaK283gs1Ghsw%3D%3D Occurrence Handle10.1002/hep.510230507
Author information
Authors and Affiliations
Additional information
An erratum to this article is available at http://dx.doi.org/10.1007/s00535-006-1971-2.
Rights and permissions
About this article
Cite this article
Halank, M., Ewert, R., Seyfarth, HJ. et al. Portopulmonary hypertension. J Gastroenterol 41, 837–847 (2006). https://doi.org/10.1007/s00535-006-1879-x
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/s00535-006-1879-x