Hepatopulmonary syndrom

   Hepatopulmonary syndrome is defined as a clinical disorder (associated with advanced liver disease) due to disturbed pulmonary gas exchange leading to hypoxemia and widespread intrapulmonary vasodilation and shunting of blood in the absence of detectable primary cardiopulmonary disease.

Hepatopulmonary syndrom

  In chronic liver disease, pulmonary affection is common and may result in hypoxia and cyanosis. The pulmonary changes that may complicate chronic liver diseases are: 

•• Hypoxia. 

•• Intrapulmonary shunting of blood via microscopic AV fistula (from pulmonary arteriole to vein). 

•• Reduce transfer factor—due to deposition of collagen in the vessel wall causing thickening of vessel wall resulted in reduced gas exchange. 

•• Pleural effusion.

 •• Raised diaphragm. 

•• Basal atelectasis. 

•• Primary pulmonary hypertension. 

The syndrome is characterized by: 

•• Platypnea (dyspnea in sitting posture). 

•• Orthodeoxia (reduced O2 saturation in sitting and standing posture than supine posture).

 •• Cyanosis. 

•• Spider nevi and clubbing (inconstant finding). 

The disease is mostly found with autoimmune hepatitis or long-standing cirrhosis.

  PATHOLOGY/PATHOPHYSIOLOGY

  The Aa-PaO2 gradient exceeds 15 mm Hg. The intrapulmonary shunting is via microscopic arteriovenous (AV) fistula. The vasoactive substances that could involve in pulmonary vasodilatation are unknown probably NO, endothelin and other arachidonic acid metabolites are responsible for pulmonary vasodilation and shunting.

  DIAGNOSIS

  •• Increase Aa-Pa gradient O2 > 15 mm Hg. 

•• Transthoracic contrast enhanced echocardiography. 

•• 99mmTc macroaggregated albumin lung scan. 

•• Pulmonary angiography—Spongy appearance of basal pulmonary vessels correspond to the infiltrate in lung field.

MANAGEMENT

  •• No pharmacological treatment is effective. 

•• Hepatic transplantation is the best mode of therapy. 

•• Reversal is not always guaranteed where AV shunts are large. Then they require coil embolic therapy which should precede transplant.

 •• TIPS—It can be useful for palliation in a patient awaiting for hepatic transplantation.

References

1: Koch DG, Fallon MB. Hepatopulmonary syndrome. Clin Liver Dis. 2014

May;18(2):407-20. doi: 10.1016/j.cld.2014.01.003. Epub 2014 Feb 25. PMID:

24679503.

2: Rodríguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome--a liver-induced lung

vascular disorder. N Engl J Med. 2008 May 29;358(22):2378-87. doi:

10.1056/NEJMra0707185. PMID: 18509123.

3: Gupta S, Krowka MJ. Hepatopulmonary syndrome. CMAJ. 2018 Feb 26;190(8):E223.

doi: 10.1503/cmaj.170253. PMID: 29483331; PMCID: PMC5826708.

4: Fuhrmann V, Krowka M. Hepatopulmonary syndrome. J Hepatol. 2018

Sep;69(3):744-745. doi: 10.1016/j.jhep.2018.01.002. Epub 2018 Jun 28. PMID:

29960651.

5: Kumar P, Rao PN. Hepatopulmonary Syndrome. N Engl J Med. 2020 Mar

5;382(10):e14. doi: 10.1056/NEJMicm1901205. PMID: 32130817.

6: Grilo-Bensusan I, Pascasio-Acevedo JM. Hepatopulmonary syndrome: What we know

and what we would like to know. World J Gastroenterol. 2016 Jul

7;22(25):5728-41. doi: 10.3748/wjg.v22.i25.5728. PMID: 27433086; PMCID:

PMC4932208.

7: Soulaidopoulos S, Cholongitas E, Giannakoulas G, Vlachou M, Goulis I. Review

article: Update on current and emergent data on hepatopulmonary syndrome. World

J Gastroenterol. 2018 Mar 28;24(12):1285-1298. doi: 10.3748/wjg.v24.i12.1285.

PMID: 29599604; PMCID: PMC5871824.

8: Iqbal S, Smith KA, Khungar V. Hepatopulmonary Syndrome and Portopulmonary

Hypertension: Implications for Liver Transplantation. Clin Chest Med. 2017

Dec;38(4):785-795. doi: 10.1016/j.ccm.2017.08.002. Epub 2017 Sep 23. PMID:

29128026.

9: Cartin-Ceba R, Krowka MJ. Pulmonary Complications of Portal Hypertension.

Clin Liver Dis. 2019 Nov;23(4):683-711. doi: 10.1016/j.cld.2019.06.003. Epub

2019 Aug 9. PMID: 31563218.

10: Lv Y, Fan D. Hepatopulmonary Syndrome. Dig Dis Sci. 2015 Jul;60(7):1914-23.

doi: 10.1007/s10620-015-3593-0. Epub 2015 Mar 3. PMID: 25732713.

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