hepatopulmonary syndrome
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Etiology
- arterial hypoxemia in patients with cirrhosis
- most common in patients with portal hypertension
Pathology
- hypoxemia
- low pulmonary vascular tone
- poor or absent hypoxic pressor response
- marked dilation of pulmonary vasculature
- arteriovenous connections leading to intrapulmonary shunting & VQ mismatch
- 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
- pO2 < 70 mm Hg
- Aa oxygen gradient > 20 mmg Hg
Diagnostic procedures
- 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
More general terms
Additional terms
References
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 792
- ↑ 2.0 2.1 2.2 Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2021.
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/18509123
- ↑ Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet. 2004 May 1;363(9419):1461-8. PMID: https://www.ncbi.nlm.nih.gov/pubmed/15121411
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/23155142 Free PMC Article
- ↑ 6.0 6.1 6.2 Kumar P, Rao PN Images in Clinical Medicine. Hepatopulmonary Syndrome. N Engl J Med 2020; 382:e14. March 5 PMID: https://www.ncbi.nlm.nih.gov/pubmed/32130817 https://www.nejm.org/doi/full/10.1056/NEJMicm1901205
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/23190201 Review.
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/27326810