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Current Cardiology Reviews

Editor-in-Chief

ISSN (Print): 1573-403X
ISSN (Online): 1875-6557

Case Report

When Pulmonary Arterial Hypertension may be Associated with Portal Hypertension: A Case Report of Two Different Hepatic Disorders in One Patient with Pulmonary Hypertension

Author(s): Ganna D. Radchenko* and Yuriy M. Sirenko

Volume 20, Issue 1, 2024

Published on: 20 October, 2023

Article ID: e201023222476 Pages: 7

DOI: 10.2174/011573403X267162231011154808

Price: $65

Abstract

Background: Pulmonary arterial hypertension (PAH) is a rare complication of hepatic diseases with portal hypertension that, however, has a significant influence on prognosis. We present a mini-review of how to diagnose and treat it based on a clinical case.

Case Presentation: In early childhood, a patient had portal hypertension associated with cavernous transformation of the portal vein. It was successfully treated by reno-splenic surgery. At the age of 20 years, this patient experienced increased dyspnea at minimal physical activity after the hepatic biopsy due to a hepatocellular adenoma. The examination in the specialized unit showed PAH, which was evaluated as associated with portal hypertension (PAH-PoH). The specific two-drug combination therapy was started with prominent improvement in patient’s state. Successful surgical tumor treatment was provided some months later. The practical and clinical approaches to the diagnosis and treatment of PAH-PoH are discussed. It was emphasized that not all patients with portal hypertension have pulmonary hypertension, which needs to be treated. A lot of evidence gaps exist in management of these patients.

Conclusion: All patients, even with past history of portal hypertension, should be monitored closely and screened for PAH earlier, for better results of treatment.

[1]
Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; 43(38): 3618-731.
[http://dx.doi.org/10.1093/eurheartj/ehac237] [PMID: 36017548]
[2]
Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2016; 37(1): 67-119.
[http://dx.doi.org/10.1093/eurheartj/ehv317] [PMID: 26320113]
[3]
Porres-Aguilar M, Altamirano JT, Torre-Delgadillo A, Charlton MR, Duarte-Rojo A. Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview. Eur Respir Rev 2012; 21(125): 223-33.
[http://dx.doi.org/10.1183/09059180.00007211] [PMID: 22941887]
[4]
Ramsay MA, Simpson BR, Nguyen AT, Ramsay KJ, East C, Klintmalm GB. Severe pulmonary hypertension in liver transplant candidates. Liver Transpl Surg 1997; 3(5): 494-500.
[http://dx.doi.org/10.1002/lt.500030503] [PMID: 9346791]
[5]
Kawut SM, Krowka MJ, Trotter JF, et al. Clinical risk factors for portopulmonary hypertension. Hepatology 2008; 48(1): 196-203.
[http://dx.doi.org/10.1002/hep.22275] [PMID: 18537192]
[6]
Savale L, Guimas M, Ebstein N, et al. Portopulmonary hypertension in the current era of pulmonary hypertension management. J Hepatol 2020; 73: 130-9.
[7]
Schouten JNL, Garcia-Pagan JC, Valla DC, Janssen HLA. Idiopathic noncirrhotic portal hypertension. Hepatology 2011; 54(3): 1071-81.
[http://dx.doi.org/10.1002/hep.24422] [PMID: 21574171]
[8]
Thomas C, Glinskii V, de Jesus Perez V, Sahay S. Portopulmonary hypertension: From bench to bedside. Front Med (Lausanne) 2020; 7: 569413.
[http://dx.doi.org/10.3389/fmed.2020.569413] [PMID: 33224960]
[9]
Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 2019; 53(1): 1801913.
[http://dx.doi.org/10.1183/13993003.01913-2018]
[10]
Porres-Aguilar M, Zuckerman MJ, Figueroa-Casas JB, Krowka MJ. Portopulmonary hypertension: State of the art. Ann Hepatol 2008; 7(4): 321-30.
[http://dx.doi.org/10.1016/S1665-2681(19)31832-0] [PMID: 19034231]
[11]
Rodríguez-Roisin R, Krowka MJ, Hervé P, Fallon MB. Pulmonary-Hepatic vascular Disorders (PHD). Eur Respir J 2004; 24(5): 861-80.
[http://dx.doi.org/10.1183/09031936.04.00010904] [PMID: 15516683]
[12]
Hervé P, Lebrec D, Brenot F, et al. Pulmonary vascular disorders in portal hypertension. Eur Respir J 1998; 11(5): 1153-66.
[http://dx.doi.org/10.1183/09031936.98.11051153] [PMID: 9648972]
[13]
Benjaminov FS, Prentice M, Sniderman KW, Siu S, Liu P, Wong F. Portopulmonary hypertension in decompensated cirrhosis with refractory ascites. Gut 2003; 52(9): 1355-62.
[http://dx.doi.org/10.1136/gut.52.9.1355] [PMID: 12912870]
[14]
Neuhofer W, Gülberg V, Gerbes AL. Endothelin and endothelin receptor antagonism in portopulmonary hypertension. Eur J Clin Invest 2006; 36(s3) (Suppl. 3): 54-61.
[http://dx.doi.org/10.1111/j.1365-2362.2006.01690.x] [PMID: 16919012]
[15]
Kéreveur A, Callebert J, Humbert M, et al. High plasma serotonin levels in primary pulmonary hypertension. Effect of long-term epoprostenol (prostacyclin) therapy. Arterioscler Thromb Vasc Biol 2000; 20(10): 2233-9.
[http://dx.doi.org/10.1161/01.ATV.20.10.2233] [PMID: 11031209]
[16]
Hervé P, Launay JM, Scrobohaci ML, et al. Increased plasma serotonin in primary pulmonary hypertension. Am J Med 1995; 99(3): 249-54.
[http://dx.doi.org/10.1016/S0002-9343(99)80156-9] [PMID: 7653484]
[17]
Roberts KE, Fallon MB, Krowka MJ, et al. Serotonin transporter polymorphisms in patients with portopulmonary hypertension. Chest 2009; 135(6): 1470-5.
[http://dx.doi.org/10.1378/chest.08-1909] [PMID: 19141529]
[18]
Tuder RM, Cool CD, Geraci MW, et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med 1999; 159(6): 1925-32.
[http://dx.doi.org/10.1164/ajrccm.159.6.9804054] [PMID: 10351941]
[19]
Le Pavec J, Souza R, Herve P, et al. Portopulmonary hypertension. Am J Respir Crit Care Med 2008; 178(6): 637-43.
[http://dx.doi.org/10.1164/rccm.200804-613OC] [PMID: 18617641]
[20]
Swanson KL, Wiesner RH, Nyberg SL, Rosen CB, Krowka MJ. Survival in portopulmonary hypertension: Mayo Clinic experience categorized by treatment subgroups. Am J Transplant 2008; 8(11): 2445-53.
[http://dx.doi.org/10.1111/j.1600-6143.2008.02384.x] [PMID: 18782292]
[21]
Krowka MJ, Fallon MB, Mulligan DC, Gish RG. Model for end-stage liver disease (MELD) exception for portopulmonary hypertension. Liver Transpl 2006; 12(S3) (Suppl. 3): S114-6.
[http://dx.doi.org/10.1002/lt.20975] [PMID: 17123283]
[22]
Ashfaq M, Chinnakotla S, Rogers L, et al. The impact of treatment of portopulmonary hypertension on survival following liver transplantation. Am J Transplant 2007; 7: 1258-1264.eHeal.
[http://dx.doi.org/10.1111/j.1600-6143.2006.01701.x]
[23]
Krowka MJ, Miller DP, Barst RJ, et al. Portopulmonary hypertension. Chest 2012; 141(4): 906-15.
[http://dx.doi.org/10.1378/chest.11-0160] [PMID: 21778257]
[24]
Lazaro Salvador M, Quezada Loaiza CA, Rodríguez Padial L, et al. Portopulmonary hypertension: prognosis and management in the current treatment era – results from the REHAP registry. Intern Med J 2021; 51(3): 355-65.
[http://dx.doi.org/10.1111/imj.14751] [PMID: 31943676]
[25]
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 Transplant 2017; 36(7): 770-9.
[http://dx.doi.org/10.1016/j.healun.2016.12.014] [PMID: 28190786]
[26]
Martin P, DiMartini A, Feng S, Brown R Jr, Fallon M. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology 2014; 59(3): 1144-65.
[http://dx.doi.org/10.1002/hep.26972] [PMID: 24716201]
[27]
Savale L, Sattler C, Coilly A, et al. Long‐term outcome in liver transplantation candidates with portopulmonary hypertension. Hepatology 2017; 65(5): 1683-92.
[http://dx.doi.org/10.1002/hep.28990] [PMID: 27997987]
[28]
Preston IR, Burger CD, Bartolome S, et al. Ambrisentan in portopulmonary hypertension: A multicenter, open-label trial. J Heart Lung Transplant 2020; 39(5): 464-72.
[http://dx.doi.org/10.1016/j.healun.2019.12.008] [PMID: 32008947]
[29]
Cartin-Ceba R, Halank M, Ghofrani HA, et al. Riociguat treatment for portopulmonary hypertension: A subgroup analysis from the PATENT‐1/‐2 studies. Pulm Circ 2018; 8(2): 1-4.
[http://dx.doi.org/10.1177/2045894018769305] [PMID: 29565224]
[30]
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: 594-604.
[http://dx.doi.org/10.1016/S2213-2600(19)30091-8]
[31]
Krowka M, Cottreel E, Hoeper MM, et al. Macitentan improves risk categorization for liver transplant mortality in patients with portopulmonary hypertension: A portico study post hoc analysis. Liver Transpl 2020; 26(7): 935-40.
[http://dx.doi.org/10.1002/lt.25747] [PMID: 32150762]

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