Heart, Lung and Circulation

Portopulmonary Hypertension: The Interplay Between the Liver and Pulmonary Arteries

      Portopulmonary hypertension (PPHTN) is a cause of pre-capillary pulmonary hypertension (PH) that occurs in the setting of advanced liver disease [
      • Galie N.
      • McLaughlin V.V.
      • Rubin L.J.
      • Simonneau G.
      An overview of the 6th World Symposium on Pulmonary Hypertension.
      ]. 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. [
      • Peppas S.
      • Nagraj S.
      • Koutsias G.
      • Kladas M.
      • Archontakis-Barakakis P.
      • Schizas D.
      • et al.
      Portopulmonary hypertension: a review of the current literature.
      ] 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 thumbnail gr1
      Figure 1Mechanisms of portopulmonary hypertension.


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      • Portopulmonary Hypertension: A Review of the Current Literature
        Heart, Lung and CirculationVol. 31Issue 9
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          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.
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