Review
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World J Gastroenterol. Jul 7, 2014; 20(25): 8072-8081
Published online Jul 7, 2014. doi: 10.3748/wjg.v20.i25.8072
Portopulmonary hypertension and hepatopulmonary syndrome
Florence Aldenkortt, Marc Aldenkortt, Laurence Caviezel, Jean Luc Waeber, Anne Weber, Eduardo Schiffer
Florence Aldenkortt, Marc Aldenkortt, Laurence Caviezel, Jean Luc Waeber, Anne Weber, Eduardo Schiffer, Division of Anesthesiology, Department of Anesthesiology, Clinical Pharmacology and Intensive Care, Geneva University Hospitals, 1211 Geneva, Switzerland
Author contributions: All authors contributed equally to this work.
Correspondence to: Florence Aldenkortt, MD, Staff Anesthesiologist, Division of Anesthesiology, Department of Anesthesiology, Clinical Pharmacology and Intensive Care, Geneva University Hospitals, Rue Gabrielle-Perret-Gentil 4, 1211 Geneva, Switzerland. florence.aldenkortt@hcuge.ch
Telephone: +41-79-5532173 Fax: +41-22-3727690
Received: November 28, 2013
Revised: March 18, 2014
Accepted: April 5, 2014
Published online: July 7, 2014
Processing time: 216 Days and 16.9 Hours
Abstract

Portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS) are two frequent complications of liver disease, with prevalence among liver transplant candidates of 6% and 10%, respectively. Both conditions result from a lack of hepatic clearance of vasoactive substances produced in the splanchnic territory. Subsequently, these substances cause mainly pulmonary vascular remodeling and some degree of vasoconstriction in POPH with resulting elevated pulmonary pressure and right ventricular dysfunction. In HPS the vasoactive mediators cause intrapulmonary shunts with hypoxemia. Medical treatment is disappointing overall. Whereas liver transplantation (LT) results in the disappearance of HPS within six to twelve months, its effect on POPH is highly unpredictable. Modern strategies in managing HPS and POPH rely on a thorough screening and grading of the disease’s severity, in order to tailor the appropriate therapy and select only the patients who will benefit from LT. The anesthesiologist plays a central role in managing these high-risk patients. Indeed, the important hemodynamic and respiratory modifications of the perioperative period must be avoided through continuation of the preoperatively initiated drugs, appropriate intraoperative monitoring and proper hemodynamic and respiratory therapies.

Keywords: End stage liver disease; Hepatopulmonary syndrome; Portopulmonary Hypertension; Anesthesia

Core tip: Portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS) are frequent complications of liver disease. Both conditions result from diminished hepatic clearance of splanchnic vasoactive substances. They cause pulmonary vasoconstriction in POPH resulting in elevated pulmonary pressure, right ventricular dysfunction and intrapulmonary shunts with hypoxemia in HPS. The only lasting treatment is liver transplantation (LT). Whereas LT results in the disappearance of HPS, its effect on POPH is unpredictable. The anesthesiologist plays a central role in managing HPS and POPH during LT as preoperative screening and grading of the disease allows the selection of appropriate therapies.