Retrospective analysis of the results of liver transplantation for adults with severe hepatopulmonary syndrome

Abstract

The hepatopulmonary syndrome (HPS), consisting of elevated alveolar-arterial oxygen gradient and intrapulmonary vascular abnormalities in the presence of advanced liver disease, is associated with high mortality. Liver transplantation (LT) has been used for the treatment of HPS; however, the success of LT for the treatment of HPS is not uniformly documented. We reviewed our experience over a 5-year period and identified eight adult patients with incapacitating respiratory symptoms compatible with HPS. Inclusion criteria included hypoxemia, normal lung volumes, reduced oxygen diffusing capacity (D L CO), and the presence of intrapulmonary shunting. Underlying liver disease was caused by hepatitis C (2 patients), primary biliary cirrhosis (1 patient), cryptogenic cirrhosis (1 patient), alcohol (2 patients), and hepatitis C with alcohol P atients with chronic advanced liver disease frequently show abnormalities in numerous organs. For example, renal insufficiency (hepatorenal syndrome) and peripheral arteriovenous shunting are widely recognized sequelae of cirrhosis. Less often recognized are the defects in pulmonary gas exchange observed with liver disease. A hepatopulmonary syndrome (HPS) has been described and encompasses a specific group of patients with severe liver disease and without cardiorespiratory disease who develop impaired gas exchange because of intrapulmonary vasodilatation (IPVD).In the past, hypoxemia was considered an absolute contraindication to liver transplantation (LT) 1 because of the operative and perioperative complications that arise in patients with gas exchange abnormalities. As the recognition of and experience with HPS has increased, concepts regarding indications LT for transplantation have come full circle, and it has been suggested that, in the face of otherwise stable liver disease, symptomatic HPS in and of itself may constitute an indication for LT. The role for transplantation in HPS is supported by the observation that the metabolic defects responsible for the pulmonary manifestations of HPS clearly are hepatic in origin, progressive, and, at present, refractory to medical management. However, the success of LT for the treatment of HPS is not uniformly documented.In the current study, we reviewed our single-center experience of eight patients with diagnosed HPS evaluated for LT, and report their long-term outcome and complications. Experimental Procedures Preoperative EvaluationAll patients were referred to UCLA for liver transplantation (LT) routinely undergo pulmonary function

Ocorrência de síndrome hepatopulmonar em pacientes cirróticos candidatos a transplante de fígado

“…Underscoring the role of schistosomiasis-related PH (without associated cirrhosis) in the etiology studies. (20) According to the 2004 arterial blood gas standards for the diagnosis of HPS, (1) the frequency of the syndrome in the present study was 45.5%.…”

Hepatopulmonary syndrome: an update

“…18,22 However, each HPS case that is considered severe should be analyzed individually because of the high morbidity and mor-tality rates associated with this condition, both during surgery and during the postoperative period. 5,14,47 Without liver transplantation, the long-term mortality rate is higher among cirrhotic patients with HPS than among those without HPS. 13,90 Prospective analysis among candidates for liver transplantation has demonstrated significantly lower mean survival among patients with HPS than among those without HPS (4.8 months and 35.3 months respectively; P = 0.005) when they do not receive a transplant, even compared with the same classification of Child-Pugh score (0.26 months and 3.82 months respectively; P = 0.01).…”

“…13 This is likely to have been due to the rapid progression of hypoxemia in the patients with HPS and the mean annual decline in PaO 2 of approximately 5 mmHg. 13 It has been shown that, following transplantation, the oxygen saturation improves, the oxygen dependence of HPS patients is resolved 47 and there are similar mortality rates among patients with and without HPS. 13 These findings confirm the changes to the course of HPS and consequent prognosis for such patients that transplantation causes.…”

“…5 Symptoms such as dyspnea and platypnea are common in HPS, but are not pathognomonic and can be found in the early phase, whereas spider angioma, peripheral cyanosis and finger clubbing are found in more advanced stages. 5,46 The combination of non-specific clinical criteria, associated with the lack of standardization in the diagnostic criteria for defining HPS and the lack of a gold standard confirmation test 47 may lead to diagnostic errors, 7 This also explains the wide range of prevalence. 9,11 There are reports of occurrences of HPS in patients with cirrhosis, with rates ranging from 4% 48 to 32%.…”

Therapy Insight: hepatopulmonary syndrome and orthotopic liver transplantation

“…27 Hypoxemia can be corrected as early as 6-12 months after OLT, 9,30 although the time taken for hypoxemia to reverse can be longer in patients who have severe HPS. An increased recovery time is observed when the patient has a preoperative PaO 2 ≤52 mmHg, a preoperative AaPO 2 ≥66 mmHg, is >48 years of age, or if the liver disease is a result of alcohol abuse.…”