Have a personal or library account? Click to login
Portopulmonary Hypertension in Compensated Cirrhosis: When Haemoptysis Tells the Story Cover

Portopulmonary Hypertension in Compensated Cirrhosis: When Haemoptysis Tells the Story

Open Access
|Oct 2025

References

  1. Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: developed by 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 for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG). <em>European Heart Journal</em>. 2022;43(38): 3618–3731. doi:<a href="https://doi.org/10.1093/eurheartj/ehac237" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1093/eurheartj/ehac237</a>
  2. Maron BA. Revised definition of pulmonary hypertension and approach to management: a clinical primer. <em>Journal of the American Heart Association</em>. 2023;12(8): e029024. doi:<a href="https://doi.org/10.1161/JAHA.122.029024" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1161/JAHA.122.029024</a>
  3. Jasso-Baltazar EA, Peña-Arellano GA, Aguirre-Valadez J, Ruiz I, Papacristofilou-Riebeling B, Jimenez JV, et al. Portopulmonary hypertension: an updated review. <em>Transplantation Direct</em>. 2023;9(8): e1517. doi:<a href="https://doi.org/10.1097/TXD.0000000000001517" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1097/TXD.0000000000001517</a>
  4. Weinfurtner K, Forde K. Hepatopulmonary syndrome and portopulmonary hypertension: current status and implications for liver transplantation. <em>Current Hepatology Reports</em>. 2020;19(3): 174–185. doi:<a href="https://doi.org/10.1007/s11901-020-00532-y" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1007/s11901-020-00532-y</a>
  5. Soulaidopoulos S, Goulis I, Cholongitas E. Pulmonary manifestations of chronic liver disease: a comprehensive review. <em>Annals of Gastroenterology</em>. 2020;33(3): 237–249. doi:<a href="https://doi.org/10.20524/aog.2020.0474" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.20524/aog.2020.0474</a>
  6. Tamura Y, Tamura Y, Taniguchi Y, Atsukawa M. Current clinical understanding and effectiveness of portopulmonary hypertension treatment. <em>Frontiers in Medicine (Lausanne)</em>. 2023;10: 1142836. doi:<a href="https://doi.org/10.3389/fmed.2023.1142836" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.3389/fmed.2023.1142836</a>
  7. Gioia S, Nardelli S, Ridola L, Riggio O. Causes and management of non-cirrhotic portal hypertension. <em>Current Gastroenterology Reports</em>. 2020;22(12): 56. doi:<a href="https://doi.org/10.1007/s11894-020-00792-0" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1007/s11894-020-00792-0</a>
  8. Shao Y, Yin X, Qin T, Zhang R, Zhang Y, Wen X. Prevalence and associated factors of portopulmonary hypertension in patients with portal hypertension: a case-control study. <em>BioMed Research International</em>. 2021;2021(1): 5595614. doi:<a href="https://doi.org/10.1155/2021/5595614" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1155/2021/5595614</a>
  9. Ishikawa T, Egusa M, Kawamoto D, Nishimura T, Sasaki R, Saeki I, et al. Screening for portopulmonary hypertension using computed tomography-based measurements of the main pulmonary artery and ascending aorta diameters in patients with portal hypertension. <em>Hepatology Research</em>. 2022;52(3): 255–268. doi:<a href="https://doi.org/10.1111/hepr.13735" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1111/hepr.13735</a>
  10. Sartoris R, Vilgrain V, Wang N. Quantitative computed tomography-based approaches for noninvasive diagnosis of portal hypertension in patients with cirrhosis. <em>Portal Hypertension and Cirrhosis</em>. 2023;2(2): 92–97. doi:<a href="https://doi.org/10.1002/poh2.48" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1002/poh2.48</a>
  11. Sterling RK, Asrani SK, Levine D, Duarte-Rojo A, Patel K, Fiel MI, et al. AASLD Practice Guideline on noninvasive liver disease assessment of portal hypertension. <em>Hepatology (Baltimore, MD)</em>. 2025;81(3): 1060–1085. doi:<a href="https://doi.org/10.1097/HEP.0000000000000844" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1097/HEP.0000000000000844</a>
  12. Kumar A, Maruyama H, Arora A, Sharma P, Anikhindi SA, Bansal N, et al. Diagnostic accuracy of transient elastography in diagnosing clinically significant portal hypertension in patients with chronic liver disease: a systematic review and meta-analysis. <em>Journal of Medical Ultrasonics (2001)</em>. 2022;49(3): 333–346. doi:<a href="https://doi.org/10.1007/s10396-022-01239-x" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1007/s10396-022-01239-x</a>
  13. Tsoris A, Marlar CA. <em>Use of the child Pugh score in liver disease</em>. Treasure Island, FL: StatPearls Publishing; 2025. p. 2025.
  14. Krige J, Spence RT, Jonas E, Hoogerboord M, Ellsmere J. A new recalibrated four-category child–Pugh score performs better than the original child–Pugh and MELD scores in predicting in-hospital mortality in decompensated alcoholic cirrhotic patients with acute variceal bleeding: a real-world cohort analysis. <em>World Journal of Surgery</em>. 2020;44(1): 241–246. doi:<a href="https://doi.org/10.1007/s00268-019-05211-8" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1007/s00268-019-05211-8</a>
  15. Ishii S, Minatsuki S, Hatano M, Saito A, Yagi H, Shimbo M, et al. The ratio of TAPSE to PASP predicts prognosis in lung transplant candidates with pulmonary arterial hypertension. <em>Scientific Reports</em>. 2023;13(1): 3758. doi:<a href="https://doi.org/10.1038/s41598-023-30924-1" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1038/s41598-023-30924-1</a>
  16. Kovacs G, Bartolome S, Denton CP, Gatzoulis MA, Gu S, Khanna D, et al. Definition, classification and diagnosis of pulmonary hypertension. <em>The European Respiratory Journal</em>. 2024;64: 2401324. doi:<a href="https://doi.org/10.1183/13993003.01324-2024" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1183/13993003.01324-2024</a>
  17. Thomas C, Glinskii V, de Jesus Perez V, Sahay S. Portopulmonary hypertension: from bench to bedside. <em>Frontiers in Medicine (Lausanne)</em>. 2020;7: 569413. doi:<a href="https://doi.org/10.3389/fmed.2020.569413" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.3389/fmed.2020.569413</a>
  18. Rodríguez-Roisin R, Krowka MJ, Hervé P, Fallon MB; ERS Task Force Pulmonary-Hepatic Vascular Disorders (PHD) Scientific Committee. Pulmonary–hepatic vascular disorders (PHD). <em>The European Respiratory Journal</em>. 2004;24(5): 861–880. doi:<a href="https://doi.org/10.1183/09031936.04.00010904" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1183/09031936.04.00010904</a>
  19. Sigal SH, Sherman Z, Jesudian A. Clinical implications of thrombocytopenia for the cirrhotic patient. <em>Hepatic Medicine: Evidence and Research</em>. 2020;12(null): 49–60. doi:<a href="https://doi.org/10.2147/HMER." target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.2147/HMER.</a> S244596
  20. Silva CLM, Puthanveetil PN, Oliveira SD. Liver bypass in the development of pathogen-associated pulmonary vascular disease: contribution of mesocaval and portosystemic shunts. <em>American Journal of Physiology. Gastrointestinal and Liver Physiology</em>. 2025;328(6): G791–G800. doi:<a href="https://doi.org/10.1152/ajpgi.00409.2024" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1152/ajpgi.00409.2024</a>
  21. Kumar A, Gonzalez G, Wilkinson L, Mohammed TL, Castro-Pavia F, Glockner J, et al. Computed tomography findings of spontaneous porto-pulmonary shunts in 3 patients with portal hypertension. <em>Journal of Thoracic Imaging</em>. 2010;25(3): W70–W74. doi:<a href="https://doi.org/10.1097/RTI.0b013e3181a652fa" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1097/RTI.0b013e3181a652fa</a>
  22. Talwalkar JA, Swanson KL, Krowka MJ, Andrews JC, Kamath PS. Prevalence of spontaneous portosystemic shunts in patients with portopulmonary hypertension and effect on treatment. <em>Gastroenterology</em>. 2011;141(5): 1673–1679. doi:<a href="https://doi.org/10.1053/j." target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1053/j.</a> gastro.2011.06.053
  23. Baroutidou A, Arvanitaki A, Hatzidakis A, Pitsiou G, Ziakas A, Karvounis H, et al. Haemoptysis in pulmonary arterial hypertension associated with congenital heart disease: insights on pathophysiology, diagnosis and management. <em>Journal of Clinical Medicine [Internet]</em>. 2022;11(3): 633. doi:<a href="https://doi.org/10.3390/jcm11030633" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.3390/jcm11030633</a>
  24. Certain MC, Baron A, Turpin M, Ebstein N, Boucly A, Beurnier A, et al. Outcomes of cirrhotic patients with pre-capillary pulmonary hypertension and pulmonary vascular resistance between 2 and 3 Wood Units. <em>European Respiratory Journal</em>. 2022;60(2): 2200107. doi:<a href="https://doi.org/10.1183/13993003.00107-2022" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1183/13993003.00107-2022</a>
  25. Segraves JM, Cartin-Ceba R, Leise MD, Krowka MJ. Relationship between portopulmonary hypertension and splenectomy: Mayo Clinic experience and review of published works. <em>Hepatology Research: the Official Journal of the Japan Society of Hepatology</em>. 2018;48(3): E340–E346. doi:<a href="https://doi.org/10.1111/hepr.12930" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1111/hepr.12930</a>
  26. Huang L, Li W, Yang T, Xiong C, Ni X, Gu Q, et al. Association between splenectomy and portal hypertension in the development of pulmonary hypertension. <em>Pulmonary Circulation</em>. 2020;10(1): 2045894019895426. doi:<a href="https://doi.org/10.1177/2045894019895426" target="_blank" rel="noopener noreferrer" class="text-signal-blue hover:underline">10.1177/2045894019895426</a>
DOI: https://doi.org/10.2478/pneum-2025-0026 | Journal eISSN: 2247-059X | Journal ISSN: 2067-2993
Language: English
Page range: 38 - 47
Published on: Oct 9, 2025
Published by: Romanian Society of Pneumology
In partnership with: Paradigm Publishing Services
Publication frequency: 1 times per year

© 2025 Ruxandra Stirbu, Bogdan Moldoveanu, Elena Cristina Moldoveanu, Raul Eduard Dabija, Radu Crisan-Dabija, published by Romanian Society of Pneumology
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.