Skip to main content
Log in

Pulmonale Komplikationen der Leberzirrhose

Hepatopulmonales Syndrom, portopulmonale Hypertonie und hepatischer Hydrothorax

Pulmonary complications of liver cirrhosis

Hepatopulmonary syndrome, portopulmonary hypertension and hepatic hydrothorax

  • Schwerpunkt
  • Published:
Der Internist Aims and scope Submit manuscript

Zusammenfassung

Das hepatopulmonale Syndrom, die portopulmonale Hypertonie und der hepatische Hydrothorax stellen typische pulmonale Komplikationen bei Patienten mit einer Leberzirrhose dar. Während es sich bei den ersten beiden Entitäten um pulmonalvaskuläre Krankheitsbilder handelt, sind beim hepatischen Hydrothorax typischerweise Aszites und Zwerchfellläsionen für die Entstehung der Erkrankung verantwortlich. Beim schweren hepatopulmonalen Syndrom und beim therapierefraktären hepatischen Hydrothorax stellt die Lebertransplantation die Therapie der Wahl dar, wohingegen bei der schweren portopulmonalen Hypertonie eine medikamentöse Therapie angestrebt wird. Neben intravenösen Prostazyklinderivaten stellen orale Endothelinrezeptorantagonisten und Phosphodiesterase-5-Hemmer eine Therapieoption bei ausgewählten Patienten dar.

Abstract

Hepatopulmonary syndrome, portopulmonary hypertension and hepatic hydrothorax are typical pulmonary complications in patients with liver cirrhosis. Whereas hepatopulmonary syndrome and portopulmonary hypertension represent pulmonary vascular diseases, the development of hepatic hydrothorax is associated with the presence of ascites and phrenic lesions. For severe hepatopulmonary syndrome and refractory hepatic hydrothorax, liver transplantation is the treatment of choice. In severe portopulmonary hypertension specific medical treatment is indicated. In selected patients, beside intravenous prostanoids, oral endothelin receptor antagonists and phosphodiesterase type-5 inhibitors are possible treatment options.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Abb. 1

Literatur

  1. Badesch DB, Champion HC, Sanchez MA et al. (2009) Diagnosis and assessment of pulmonary hypertension. J Am Coll Cardiol 54: S55–S66

    Article  PubMed  Google Scholar 

  2. Cerfolio RJ, Bryant AS (2006) Efficacy of video-assisted thoracoscopic surgery with talc pleurodesis for porous diaphragm syndrome in patients with refractory hepatic hydrothorax. Ann Thorac Surg 82: 457–459

    Article  PubMed  Google Scholar 

  3. Chalasani N, Clark WS, Martin LG (2000) Determinants of mortality in patients with advanced cirrhosis after transjugular intrahepatic portosystemic shunting. Gastroenterology 118: 138–144

    Article  CAS  PubMed  Google Scholar 

  4. Galie N, Hoeper MM, Humbert M et al. (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension. The 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 the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30: 2493–2537

    Article  PubMed  Google Scholar 

  5. Gomez FP, Martinez-Palli G, Barbera JA et al. (2004) Gas exchange mechanism of orthodeoxia in hepatopulmonary syndrome. Hepatology 40: 660–666

    Article  PubMed  Google Scholar 

  6. Grannas G, Neipp M, Hoeper MM et al. (2008) Indications for and outcomes after combined lung and liver transplantation: a single-center experience on 13 consecutive cases. Transplantation 85: 524–531

    Article  PubMed  Google Scholar 

  7. Halank M, Ewert R, Seyfarth HJ, Hoeffken G (2006) Portopulmonary hypertension. J Gastroenterol 41: 837–847

    Article  PubMed  Google Scholar 

  8. Halank M, Miehlke S, Höffken G et al. (2004) Use of oral endothelin-receptor antagonist bosentan in the treatment of portopulmonary hypertension. Transplantation 77: 1775–1776

    Article  PubMed  Google Scholar 

  9. Halank M, Miehlke S, Kolditz M, Hoeffken G (2005) Portopulmonary hypertension. Z Gastroenterol 43: 677–685

    Article  CAS  PubMed  Google Scholar 

  10. Hoeper MM, Krowka MJ, Strassburg CP (2004) Portopulmonary hypertension and hepatopulmonary syndrome. Lancet 363: 1461–1468

    Article  PubMed  Google Scholar 

  11. Hoeper MM, Seyfarth HJ, Hoeffken G et al. (2007) Experience with inhaled iloprost and bosentan in portopulmonary hypertension. Eur Respir J 30: 1096–1102

    Article  CAS  PubMed  Google Scholar 

  12. Ibi T, Koizumi K, Hirata T et al. (2008) Diaphragmatic repair of two cases of hepatic hydrothorax using video-assisted thoracoscopic surgery. Gen Thorac Cardiovasc Surg 56: 229–232

    Article  PubMed  Google Scholar 

  13. Kawut SM, Krowka MJ, Trotter JF et al. (2008) Clinical risk factors for portopulmonary hypertension. Hepatology 48: 196–203

    Article  PubMed  Google Scholar 

  14. Kiafar C, Gilani N (2008) Hepatic hydrothorax: Current concepts of pathophysiology and treatment options. Ann Hepatol 7: 313–320

    CAS  PubMed  Google Scholar 

  15. Le Pavec J, Souza R, Herve P et al. (2008) Portopulmonary hypertension: survival and prognostic factors. Am J Respir Crit Care Med 178: 637–643

    Article  Google Scholar 

  16. Leschke M, Wädlich A, Waldenmaier S, Faehling M (2009) Diagnostik der pulmonaren Hypertonie. Internist (Berl) 50: 1086–1100

  17. Light RW (2002) Clinical practice. Pleural effusion. N Engl J Med 346: 1971–1977

    Article  PubMed  Google Scholar 

  18. Luh SP, Chen CY (2009) Video-assisted thoracoscopic surgery (VATS) for the treatment of hepatic hydrothorax: report of twelve cases. J Zhejiang Univ Sci B 10: 547–551

    Article  PubMed  Google Scholar 

  19. Northup PG, Harmon RC, Pruett TL at al. (2009) Mechanical pleurodesis aided by peritoneal drainage: procedure for hepatic hydrothorax. Ann Thorac Surg 87: 245–250

    Article  PubMed  Google Scholar 

  20. Roberts KE, Fallon MB, Krowka MJ et al. (2009) Genetic risk factors for portopulmonary hypertension in patients with advanced liver disease. Am J Respir Crit Care Med 179: 835–842

    Article  PubMed  Google Scholar 

  21. Rodriguez-Roisin R, Krowka MJ (2008) Hepatopulmonary syndrome – a liver-induced lung vascular disorder. N Engl J Med 358: 2378–2387

    Article  CAS  PubMed  Google Scholar 

  22. Rodriguez-Roisin R, Krowka MJ, Herve PH, Fallon MB (2004) Pulmonary-hepatic vascular hisorders (PHD). Eur Respir J 24: 861–880

    Article  CAS  PubMed  Google Scholar 

  23. Runyon BA, Greenblatt M, Ming RH (1986) Hepatic hydrothorax is a relative contraindication to chest tube insertion. Am J Gastroenterol 81: 566–567

    CAS  PubMed  Google Scholar 

  24. Schenk P, Schöniger-Hekele M, Fuhrmann V et al. (2003) Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis. Gastroenterology 125: 1042–1052

    Article  PubMed  Google Scholar 

  25. Simmoneau G, Robbins IM, Beghetti M et al. (2009) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 54: S43–S54

    Article  Google Scholar 

  26. Strassburg CP, Manns MP (2009) Lebertransplantation: Indikationen und Ergebnisse. Internist (Berl) 50: 550–560

  27. Swanson KL, Krowka MJ (2008) Screen for portopulmonary hypertension, especially in liver transplant candidates. Cleve Clin J Med 75: 121–133

    Article  PubMed  Google Scholar 

  28. Swanson KL, Wiesner RH, Nyberg SL (2008) Survival in portopulmonary hypertension: Mayo Clinic experience categorized by treatment subgroups. Am J Transplant 8: 2445–2453

    Article  CAS  PubMed  Google Scholar 

  29. Wilputte JY, Goffette P, Zech F et al. (2007) The outcome after transjugular intrahepatic portosystemic shunt (TIPS) for hepatic hydrothorax is closely related to liver dysfunction: a long-term study in 28 patients. Acta Gastroenterol Belg 70: 6–10

    PubMed  Google Scholar 

  30. Xiol X, Castellote J, Cortes-Beut R et al. (2001) Usefulness and complications of thoracentesis in cirrhotic patients. Am J Med 111: 67–69

    Article  CAS  PubMed  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to M. Halank.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Halank, M., Strassburg, C. & Hoeper, M. Pulmonale Komplikationen der Leberzirrhose. Internist 51 (Suppl 1), 255–263 (2010). https://doi.org/10.1007/s00108-009-2503-y

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00108-009-2503-y

Schlüsselworte

Keywords

Navigation