Portopulmonary Hypertension (Literature Review)
Potii VV1*, Kiriienko VT2, Potii DA1, Glukhova EI3 and Kunickaya OS3
1Kiev Medical University, Kiev, Ukraine
2National Medical University Named After A.A. Bogomolets, Kiev, Ukraine
3Donetsk National Medical University, Liman, Ukraine
*Corresponding Author: Potii VV, Kiev Medical University, Kiev, Ukraine.
October 21, 2021; Published: December 31, 2021
Portopulmonary hypertension is a form of pulmonary arterial hypertension that develops as a complication of portal hypertension. The specific gravity of liver cirrhosis as a cause of portal hypertension is about 75%, which means that a significant proportion of patients with portopulmonary hypertension have liver cirrhosis, therefore, these patients are potentially candidates for liver transplantation. Although this complication is not common, it is extremely important to distinguish portopulmonary hypertension from other causes of pulmonary arterial hypertension, since an increase in mean pulmonary arterial pressure > 35 mmHg is associated with a 50-100% mortality rate during waiting and after liver transplantation. There are no clear recommendations for the treatment of this complication in patients with portal hypertension, since there are not enough clinical trials in this group of patients. A significant number of obstacles can limit the adequate treatment of patients with portopulmonary hypertension and explain the lower survival rate of this group of patients compared with other types of pulmonary arterial hypertension. Until recently, only one randomized controlled trial included patients with portopulmonary hypertension, and most of the treatment data came from relatively small observational studies. Currently, the treatment of portopulmonary hypertension includes therapy specific for pulmonary arterial hypertension regardless of its cause, and in some cases such therapy is necessary to facilitate successful liver transplantation.
Keywords: Portopulmonary Hypertension; Pulmonary Arterial Hypertension; Liver Cirrhosis; Portal Hypertension; Mean Pulmonary Arterial Pressure; Transthoracic Echocardiography; Liver Transplantation
- Humbert M., et al. “Pathology and pathobiology of pulmonary hypertension: state of the art and research perspectives”. European Respiratory Journal 53 (2019): 1801887.
- Badesch DB., et al. “Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry”. Chest2 (2010): 376-387.
- Kawut SM., et al. “Clinical risk factors for portopulmonary hypertension”. Hepatology 1 (2008): 196-203.
- Sithamparanathan S., et al. “National Pulmonary Hypertension Service Research Collaboration of the United Kingdom and Ireland”. Journal of Heart Lung Transplant7 (2017): 770.
- Krowka MJ., et al. “Portopulmonary hypertension: Results from a 10-year screening algorithm”. Hepatology6 (2006): 1502.
- Krowka MJ., et al. “Portopulmonary hypertension: a report from the US-based REVEAL Registry”. Chest4 (2012): 906.
- Le Pavec J., et al. “Portopulmonary hypertension: survival and prognostic factors”. American Journal of Respiratory and Critical Care Medicine 6 (2008): 637.
- Sithamparanathan S., et al. “Survival in portopulmonary hypertension: Outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry”. The Journal of Heart and Lung Transplantation 7 (2016): 770.
- The venot T., et al. “What is the true relationship between spontaneous portosystemic shunts and portopulmonary hypertension in cirrhotic patients?” Gastroenterology5 (2018): 1647-1648.
- Rodríguez-Roisin R., et al. “Pulmonary-Hepatic vascular Disorders (PHD)”. European Respiratory Journal5 (2014): 861.
- Bolognesi M., et al. “Splanchnic vasodilation and hyperdynamic circulatory syndrome in cirrhosis”. World Journal of Gastroenterology 20 (2014): 2555-2563.
- Baeyens N., et al. “Endothelial fluid shear stress sensing in vascular health and disease”. Journal of Clinical Investigation 126 (2016): 821-828.
- Pascall E and Tulloh RM. “Pulmonary hypertension in congenital heart disease”. Future Cardiology 14 (2018): 343-353.
- Furuta M., et al. “An autopsy case of portopulmonary hypertension associated with idiopathic portal hypertension”. European Respiratory Journal 42 (2013): P2649.
- Thomson JR., et al. “Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family”. Journal of Medical Genetics 37 (2000): 741-745.
- Desroches-Castan A., et al. “Bone morphogenetic protein 9 is a paracrine factor controlling liver sinusoidal endothelial cell fenestration and protecting against hepatic fibrosis”. Hepatology 70 (2019): 1392-1408.
- Rochon ER., et al. “Pulmonary vascular complications of liver disease 2 study, bmp 9/10 in pulmonary vascular complications of liver disease”. American Journal of Respiratory and Critical Care Medicine 201 (2020): 1575-1578.
- Nikolic I., et al. “Bone morphogenetic protein 9 is a mechanistic biomarker of portopulmonary hypertension”. American Journal of Respiratory and Critical Care Medicine 199 (2019): 891-902.
- Long L., et al. “Selective enhancement of endothelial BMPR-II with BMP9 reverses pulmonary arterial hypertension”. Nature Medicine 21 (2015): 777-785.
- Matsubara O., et al. “Histometrical investigation of the pulmonary artery in severe hepatic disease”. Journal of Pathology1 (1984): 31.
- Pietra GG. “Histopathology of primary pulmonary hypertension”. Chest2 (1994): 2S.
- Hoeper MM., et al. “Portopulmonary hypertension and hepatopulmonary syndrome”. Lancet 9419 (2004): 1461-1468.
- Abragamovich MO and Abragamovich OO. “Porto Pul’monal’na gípertenzíya: osoblivostí patogenezu, díagnostiki, klíníchnogo perebígu ta líkuvannya [Portopulmonary hypertension: features of patogenesis, diagnostics, clinical course and treatment] Ukrayinskyy Medychnyy Almanakh 13.4 (2010): 15-19.
- Ramsay M. “Portopulmonary hypertension and right heart failure in patients with cirrhosis”. Current Opinion in Anesthesiology 2 (2010): 145-150.
- Krowka MJ., et al. “International liver transplant society practice guidelines: Diagnosis and management of hepatopulmonary syndrome and portopulmonary hypertension”. Transplantation 7 (2016): 1440-1452.
- Martin Paul., et al. “Evaluation for Liver Transplantation in Adults: 2013 Practice Guideline by the AASLD and the American Society of Transplantation”. Hepatology (2014): 1144-1465.
- Krowka MJ., et al. “Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation”. Liver Transplant 6 (2000): 443-450.
- Sitbon O., et al. “Portopulmonary hypertension: light at the end of the tunnel?” Chest 141 (2012): 840-842.
- Savale L., et al. “Portopulmonary hypertension in the current era of pulmonary hypertension management”. Journal of Hepatology 73 (2020): 130-139.
- Preston IR., et al. “Ambrisentan in portopulmonary hypertension: A multicenter, open-label trial”. The Journal of Heart and Lung Transplantation 39 (2020): 464-472.
- Ghofrani HA., et al. “Riociguat for the treatment of pulmonary arterial hypertension”. The New England Journal of Medicine 369 (2013): 330-340.
- Rubin LJ., et al. “Riociguat for the treatment of pulmonary arterial hypertension: a long-term extension study (PATENT-2)”. European Respiratory Journal 45 (2015): 1303-1313.
- Cartin-Ceba R., et al. “Riociguat treatment for portopulmonary hypertension: a subgroup analysis from the PATENT-1/-2 studies”. Pulmonary Circulation 8 (2018): 2045894018769305.
- Sitbon O., et al. “Macitentan for the treatment of portopulmonary hypertension (PORTICO): a multicentre, randomised, double-blind, placebo-controlled, phase 4 trial”. The Lancet Respiratory Medicine 7 (2019): 594-604.