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portopulmonary hypertension

Pathology: - development of pulmonary artery hypertension in patients with portal hypertension [1] - hepatic cirrhosis generally cause of portal hypertension Laboratory: - arterial blood gas - hypoxemia is rare Special laboratory: - pulmonary artery catheterization - necessary for diagnosis [1] - pulmonary hypertension - echocardiogram suggestive of pulmonary hypertension Differential diagnosis: - distinguished from hepatopulmonary syndrome which is a right to left shunt without pulmonary hypertension Management: - macitentan (Opsumit) may be of benefit [4] - liver transplantation [1]

Related

hepatopulmonary syndrome

General

portal hypertension pulmonary hypertension

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 16, 17. American College of Physicians, Philadelphia 2012, 2015 - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  2. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004 May 1;363(9419):1461-8. PMID: 15121411
  3. Fritz JS, Fallon MB, Kawut SM. Pulmonary vascular complications of liver disease. Am J Respir Crit Care Med. 2013 Jan 15;187(2):133-43. Review. PMID: 23155142 Free PMC Article
  4. Ingram I Opsumit Shows Promise in Portopulmonary Hypertension. Positive results in first randomized trial in the disease. MedPage Today. September 20, 2018 https://www.medpagetoday.com/meetingcoverage/ers/75206 - Sitbon O, et al Efficacy and safety of macitentan in portopulmonary hypertension: The PORTICO trial. European Respiratory Society (ERS) 2018; Abstract OA267.