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Corresponding author at: Cardiology Department, St Vincent’s Hospital, Victoria St, Darlinghurst 2010, Australia
Affiliations
Heart Transplant Unit, St Vincent’s Hospital, Sydney, NSW, AustraliaSchool of Medicine, University of Notre Dame, Sydney, NSW, AustraliaSchool of Medicine, University of New South Wales, Sydney, NSW, AustraliaVictor Chang Cardiac Research Institute, Sydney, NSW, Australia
]. It highlights the close and dynamic relationship between the liver, right ventricle
(RV), and pulmonary arteries (Figure 1). However, it is not as simple as the pressure transmission across a compliant, low-pressure
right ventricle and associated remodelling. Peppas et al. [
] provide a comprehensive review of PPHTN, but also show that there are still significant
gaps in our knowledge and clinical practice with regards to this type of PH.
Figure 1Mechanisms of portopulmonary hypertension.
National Pulmonary Hypertension Service Research Collaboration of the United Kingdom and Ireland. Survival in portopulmonary hypertension: outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry.
Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation.
Temporary treatment interruptions with oral selexipag in pulmonary arterial hypertension: Insights from the Prostacyclin (PGI2) Receptor Agonist in Pulmonary Arterial Hypertension (GRIPHON) study.
Portopulmonary hypertension is defined as the development of pulmonary arterial hypertension in the setting of portal hypertension with or without liver cirrhosis. Portal hypertension-associated haemodynamic changes, including hyperdynamic state, portosystemic shunts and splanchnic vasodilation, induce significant alterations in pulmonary vascular bed and play a pivotal role in the pathogenesis of the disease. If left untreated, portopulmonary hypertension results in progressive right heart failure, with a poor prognosis.
