Pulmonary vascular complications of liver disease

Am J Respir Crit Care Med. 2013 Jan 15;187(2):133-43. doi: 10.1164/rccm.201209-1583CI. Epub 2012 Nov 15.


Hepatopulmonary syndrome and portopulmonary hypertension are two pulmonary vascular complications of liver disease. The pathophysiology underlying each disorder is distinct, but patients with either condition may be limited by dyspnea. A careful evaluation of concomitant symptoms, the physical examination, pulmonary function testing and arterial blood gas analysis, and echocardiographic, imaging, and hemodynamic studies is crucial to establishing (and distinguishing) these diagnoses. Our understanding of the pathobiology, natural history, and treatment of these disorders has advanced considerably over the past decade; however, the presence of either still increases the risk of morbidity and mortality in patients with underlying liver disease. There is no effective medical treatment for hepatopulmonary syndrome. Although liver transplantation can resolve hepatopulmonary syndrome, there appears to be worse survival even with transplantation. Liver transplantation poses a very high risk of death in those with significant portopulmonary hypertension, where targeted medical therapies may improve functional status and allow successful transplantation in a small number of select patients.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Hepatopulmonary Syndrome / diagnosis
  • Hepatopulmonary Syndrome / etiology*
  • Hepatopulmonary Syndrome / pathology
  • Hepatopulmonary Syndrome / physiopathology
  • Hepatopulmonary Syndrome / therapy
  • Humans
  • Hypertension, Portal / complications
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / pathology
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy
  • Liver Diseases / complications*
  • Liver Transplantation
  • Lung / pathology
  • Lung / physiopathology
  • Prognosis