%0 Journal Article
%T The Successful Use of Inhaled Nitric Oxide in the Management of Severe Hepatopulmonary Syndrome after Orthotopic Liver Transplantation
%A Joshua Santos
%A Philip Young
%A Igor Barjaktarevic
%A Catherine Lazar
%A Irawan Susanto
%A Tisha Wang
%J Case Reports in Hepatology
%D 2014
%I Hindawi Publishing Corporation
%R 10.1155/2014/415109
%X Hepatopulmonary syndrome (HPS) is characterized by pulmonary vasodilation and subsequent hypoxemia in the setting of hepatic dysfunction. There is currently no pharmacologic intervention that has been shown to significantly affect outcomes and liver transplantation remains the mainstay of therapy. Unfortunately, patients undergoing liver transplantation are at high risk of significant hypoxemia and mortality in the early postoperative period. In the following case series, we present two cases of patients with severe HPS who underwent liver transplantation and experienced marked hypoxemia in the early postoperative period. In both cases, we successfully treated the patients with inhaled nitric oxide for their severe refractory life-threatening hypoxemia which led to immediate and dramatic improvements in their oxygenation. Although the use of inhaled nitric oxide in patients with HPS has been sporadically reported in pediatric literature and in animal studies, to our knowledge, our cases are the first recorded in adult patients. 1. Introduction Hepatopulmonary syndrome (HPS) represents one of the major causes of hypoxia and dyspnea among patients with end-stage liver disease. It is characterized by the triad of hypoxemia with an abnormal alveolar-arterial (A-a) gradient, presence of intrapulmonary shunting, and chronic liver disease [1]. The frequency of this disorder among patients with cirrhosis varies from 4% to 47% and may be overall underdiagnosed due to other comorbidities and pulmonary complications of liver disease that mask its presence [1]. HPS significantly influences both functional status and survival among this population of patients [1, 2]. Multiple medications have been tested for HPS with no significant improvement in oxygenation, and supportive therapy with supplemental oxygen and liver transplantation remain the only two therapies with proven benefit [3]. Unfortunately, liver transplantation itself carries a high risk of morbidity and mortality in the perioperative and postoperative period. Patients with HPS often suffer from persistent hypoxemia and are at increased risk for prolonged mechanical ventilation with a longer ICU length of stay compared to other liver transplant recipients [4, 5]. In the following case series, we present two cases of patients with severe HPS who underwent liver transplantation and experienced marked hypoxemia in the early postoperative period. In both cases, we were successful in bridging these patients with severe refractory life-threatening hypoxemia with inhaled nitric oxide which led to immediate and
%U http://www.hindawi.com/journals/crihep/2014/415109/