Topic Highlight
Copyright ©2014 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. Mar 14, 2014; 20(10): 2586-2594
Published online Mar 14, 2014. doi: 10.3748/wjg.v20.i10.2586
Cirrhosis and hepatopulmonary syndrome
Gokhan Tumgor
Gokhan Tumgor, Department of Pediatric Gastroenterology, Cukurova University Medical Faculty, 01380 Sarıcam, Adana, Turkey
Author contributions: Tumgor G solely contributed to this paper.
Correspondence to: Gokhan Tumgor, MD, Associate Professor, Department of Pediatric Gastroenterology, Cukurova University Medical Faculty, 01380 Sarıcam, Adana, Turkey. gtumgor74@yahoo.com
Telephone: +90-506-5443280 Fax: +90-322-3386060-3116
Received: October 27, 2013
Revised: January 5, 2014
Accepted: January 20, 2014
Published online: March 14, 2014
Processing time: 136 Days and 10.3 Hours
Abstract

Hepatopulmonary syndrome (HPS) is characterized as a triad: liver disease, intrapulmonary vascular dilatation and arterial hypoxemia. HPS is reported to be present in 4% to 32% of adult patients with end-stage liver disease and in 9%-20% of children. The pathogenesis of HPS has not been clearly identified. Portal hypertension causes impairment in the perfusion of the bowel and increases the enteral translocation of Gram (-) bacteria and endotoxins. This stimulates the release of vasoactive mediators, such as tumor necrosis factor-alpha, heme oxygenase-derived carbon monoxide and nitric oxide. Genetic alterations have not been associated with this syndrome yet; however, cytokines and chemokines have been suggested to play a role. Recently, it was reported that cumulated monocytes lead to the activation of vascular endothelial growth factor-dependent signaling pathways and pulmonary angiogenesis, which plays an important role in HPS pathogenesis. At present, the most effective and only radical treatment is a liver transplant (LT). Cirrhotic patients who are on the waiting list for an LT have a shorter survival period if they develop HPS. Therefore, it is suggested that all cirrhotic cases should be followed closely for HPS and they should have priority in the waiting list.

Keywords: Cirrhosis; Hepatopulmonary syndrome; Pathophysiology; Liver transplantation

Core tip: Hepatopulmonary syndrome (HPS) is an important complication of cirrhosis. HPS is a significant factor in dyspnea and cyanosis in cirrhotic cases. At present, the most effective and only radical treatment is a liver transplant. Cirrhotic patients who are on the waiting list for a liver transplant have a shorter survival period if they develop HPS. Therefore, it is suggested that all cirrhotic cases should be followed closely for HPS and they should have priority in the waiting list. This review aims to reevaluate the recent progress in the diagnosis, pathophysiology and treatment of HPS.