Contents

Search

hepatopulmonary syndrome

Etiology: - arterial hypoxemia in patients with cirrhosis - most common in patients with portal hypertension Pathology: 1) hypoxemia 2) low pulmonary vascular tone 3) poor or absent hypoxic pressor response 4) marked dilation of pulmonary vasculature 5) arteriovenous connections leading to intrapulmonary shunting & VQ mismatch 6) portal hypertension Clinical manifestations: - dyspnea on exertion or at rest - some patients have platypnea &/or orthodeoxia - ascites, pedal edema, splenomegaly, spider nevi, clubbing (case report) [6] Laboratory: - pulse oximetry - arterial blood gas a) pO2 < 70 mm Hg b) Aa oxygen gradient > 20 mmg Hg Special laboratory: - echocardiography bubble study (diagnostic) [2] - bubbles of intravenous agitated saline that appear in the left side of the heart within 3-6 beats suggests an abnormally dilated pulmonary vascular bed [2] * video [6] Management: - liver transplantation may be curative

Related

cirrhosis portopulmonary hypertension

General

lung disease vascular disease (vasculopathy) liver disease syndrome

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 792
  2. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2021.
  3. Rodriguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome-a liver-induced lung vascular disorder. N Engl J Med. 2008 May 29;358(22):2378-87 PMID: 18509123
  4. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004 May 1;363(9419):1461-8. PMID: 15121411
  5. 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
  6. Kumar P, Rao PN Images in Clinical Medicine. Hepatopulmonary Syndrome. N Engl J Med 2020; 382:e14. March 5 PMID: 32130817 https://www.nejm.org/doi/full/10.1056/NEJMicm1901205
  7. Grace JA, Angus PW. Hepatopulmonary syndrome: update on recent advances in pathophysiology, investigation, and treatment. J Gastroenterol Hepatol. 2013 Feb;28(2):213-9. PMID: 23190201 Review.
  8. Krowka MJ, Fallon MB, Kawut SM et ak International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation. 2016 Jul;100(7):1440-52. PMID: 27326810