Portopulmonary Hypertension in Patients Undergoing Liver Transplantation: A Case Report


Portopulmonary hypertension (PPH) is a form of pulmonary arterial hypertension, characterized by elevation of pulmonary arterial pressure due to increased resistance to pulmonary blood flow. It is a consequence of portal hypertension, associated or not with liver disease. A retrospective study of patients undergoing liver transplantation was carried out in the Liver Transplantation Unit of the Clinics Hospital of the State University of Campinas (UNICAMP) from 1991 to 2012, covering 389 transplants. Although 50 (12.9%) patients presented with mean pulmonary arterial pressure (MPAP) > 25 mmHg, only one case (0.25%) met the diagnostic criteria for PPH: a 43-year-old female patient with advanced liver disease, caused by primary biliary cirrhosis with portal hypertension, diagnosed as mild portopulmonary hypertension and diagnosed in the operating room. PPH is a serious complication of portal hypertension. It is related to the great difficulty in diagnosis and the high mortality rate due to right heart failure. PPH is an entity with a low frequency of occurrence in individuals undergoing liver transplantation, although there is a wide variation in the prevalence rate presented in the literature and the definitive diagnosis should be confirmed with right heart catheterization.

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E. Shimozono, C. Caruy, A. Cardoso and I. Boin, "Portopulmonary Hypertension in Patients Undergoing Liver Transplantation: A Case Report," Surgical Science, Vol. 5 No. 2, 2014, pp. 53-56. doi: 10.4236/ss.2014.52012.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] F. A. Mantz and E. Craige, “Portal Axis Thrombosis with Spontaneous Portocaval Shunt and Resultant cor Pulmonale,” Archives of Pathology, Vol. 52, No. 1, 1951, pp. 91-97.
[2] P. Hervé, D. Liberec, F. Brenot, G. Simonneau, O. Sitbon and P. Duroux, “Pulmonary Vascular Disorders in Portal Hypertension,” European Respiratory Journal, Vol. 11, No. 5, 1998, pp. 1153-1166. http://dx.doi.org/10.1183/09031936.98.11051153
[3] L. J. Rubin, “Primary Pulmonary Hypertension ACCP Consensus Statement,” Chest, Vol. 104, No. 1, 1993, pp. 236-250. http://dx.doi.org/10.1378/chest.104.1.236
[4] N. Galiè, M. M. Hoeper, M. Humbert, et al., “ESC Committee for Practice Guidelines. 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),” European Heart Journal, Vol. 30, No. 20, 2009, pp. 2493-2537.
[5] R. Rodriguez-Roisin, M. J. Krowka, P. Hervè and M. B. Fallon, “Pulmonary-Hepatic Vascular Disorders: A Task Force Report,” European Respiratory Journal, Vol. 24, No. 5, 2004, pp. 861-880.
[6] Z. Safdar, S. Bartolome and N. Sussman, “Portopulmonary Hypertension: An Update,” Liver Transplantation, Vol. 18, No. 8, 2012, pp. 881-891. http://dx.doi.org/10.1002/lt.23485
[7] S. Giusca, M. Jinga, J. Ciprian, et al., “Portopulmonary Hypertension: From Diagnosis to Treatment,” European Journal of Internal Medicine, Vol. 22, No. 5, 2011, pp. 441-447.
[8] M. Castro, M. J. Krowka, D. R. Schroeder, et al., “Frequency and Clinical Implications of Increased Pulmonary Artery Pressures in Liver Transplant Patients,” Mayo Clinic Proceedings, Vol. 71, No. 6, 1996, pp. 543-551. http://dx.doi.org/10.4065/71.6.543
[9] P. C. Kuo, J. S. Plotkin, S. Gaine, R. A. Schroeder, V. K. Rustgi, L. J. Rubin and L. B. Johnson, “Portopulmonary Hypertension and the Liver Transplantation Candidate,” Transplantation, Vol. 67, No. 8, 1999, pp. 1087-1093.
[10] M. J. Krowka, R. P. Swanson, M. D. MacGoon and R. H. Wiesner, “Portopulmonary Hypertension: Results from a 10-Year Screening Algorithm,” Hepatology, Vol. 44, No. 6, 2006, pp. 1502-1510. http://dx.doi.org/10.1002/hep.21431
[11] S. Raevens, I. Colle, K. Reyntjens, et al., “Echocardiography for the Detection of Portopulmonary Hypertension in Liver Transplant Candidates: An Analysis of Cutoff Values,” Liver transplantation, Vol. 19, No. 6, 2013, pp. 602-610. http://dx.doi.org/10.1002/lt.23649
[12] A. Silva, “Anesthesia Monitoring,” In: R. D. Miller and M. C. Pardo Jr., Eds., Basics of Anesthesia, 6th Edition, Elsevier, Rio de Janeiro, 2012.
[13] S. M. Mandell and B. M. Groves, “Pulmonary Hypertension in Chronic Liver Disease,” Clinics in Chest Medicine, Vol. 17, No. 1, 1996, pp. 17-33. http://dx.doi.org/10.1016/S0272-5231(05)70296-3
[14] S. M. Kawut, M. J. Krowka, J. F. Trotter, et al., “Clinical Risk Factors for Portopulmonary Hypertension,” Hepatology, Vol. 48, No. 1, 2008, pp. 196-203. http://dx.doi.org/10.1002/hep.22275
[15] B. Surawitz, “Eletrocardiographic Diagnosis of Chamber Enlargement,” Journal of the American College of Cardiology, Vol. 8, No. 3, 1986, pp. 711-724. http://dx.doi.org/10.1016/S0735-1097(86) 80207-8
[16] N. D. Pilatis, L. E. Jacobs, P. Rerkpattanapipat, N. K. Morris, A. Owen, C. Manzarbeitia, D. Reich, K. Rothstein and S. J. Munoz, “Clinical Predictors of Pulmonary Hypertension in Patients Undergoing Liver Transplant Evaluation,” Liver Transplantation, Vol. 6, No. 1, 2000, pp. 85-91.
[17] J. M. Golbin and M. J. Krowka, “Portopulmonary Hypertension,” Clinics in Chest Medicine, Vol. 28, No. 1, 2007, pp. 203-218. http://dx.doi.org/10.1016/j.ccm.2006.11.004
[18] I. O. Colle, R. Moreau, E. Godinho, et al., “Diagnosis of Portopulmonary Hypertension in Candidates for Liver Transplantation: A Prospective Study,” Hepatology, Vol. 37, No. 2, 2003, pp. 401-409. http://dx.doi.org/10.1053/jhep.2003.50060
[19] K. L. Swanson, R. H. Wiesner, S. L. Nyberg, C. B. Rosen and M. J. Krowka, “Survival in Portopulmonary Hypertension: Mayo Clinic Experience Categorized by Treatment Subgroups,” American Journal of Transplantation, Vol. 8, No. 11, 2008, pp. 2445-2453. http://dx.doi.org/10.1111/j.1600-6143.2008. 02384.x
[20] M. J. Krowka, D. J. Plevak, J. Y. Findlay, C. B. Rosen, R. H. Wiesner and R. A. F. Krom, “Pulmonary Hemodynamics and Perioperative Cardiopulmonary-Related Mortality in Patients with Portopulmonary Hypertension Undergoing Liver Transplantation,” Liver Transplantation, Vol. 6, No. 4, 2000, pp. 443-450.
[21] I. F. S. F. Boin, M. I. Leonardi, E. Y. Udo, T. Sevá-Pereira, R. B. Stucchi and L. S. Leonardi, “Aplica??o do Escore MELD em Pacientes Submetidos a Transplante de Fígado: Análise Retrospectiva da Sobrevida e dos Fatores Preditivos a Curto e Longo Prazo,” Arquivos de Gastroenterologia, Vol. 45, No. 4, 2008, pp. 275-283. http://dx.doi.org/10.1590/S0004-28032008000400004

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