Gastroenterology

Gastroenterology

Volume 125, Issue 4, October 2003, Pages 1042-1052
Gastroenterology

Clinical-liver, pancreas, and biliary tract
Prognostic significance of the hepatopulmonary syndrome in patients with cirrhosis

https://doi.org/10.1016/S0016-5085(03)01207-1Get rights and content

Abstract

Background & aims: The hepatopulmonary syndrome (HPS) has been defined by chronic liver disease, arterial deoxygenation, and widespread intrapulmonary vasodilation. Mortality of patients with HPS is considered to be high, but the effect of HPS on survival in patients with cirrhosis remains unclear. Methods: A total of 111 patients with cirrhosis were studied prospectively by using transthoracic contrast echocardiography for detection of pulmonary vasodilation, blood gas analysis, and pulmonary function test. Twenty different clinical characteristics and survival times were noted. Results: Twenty-seven patients (24%) had HPS. Their mortality was significantly higher (median survival, 10.6 months) compared with patients without HPS (40.8 mo, P < 0.05), even after adjusting for liver disease severity (2.9 vs. 14.7 months in Child-Pugh class C with [n = 15] and without HPS [n = 35, P < 0.05]; 35.3 vs. 44.5 months in Child-Pugh class B with [n = 7] and without HPS [n = 23, P = NS]), and exclusion of patients who underwent liver transplantation during follow-up (median survival 4.8 vs. 35.2 months, P = 0.005). Causes of death were mainly nonpulmonary and liver-related in the 19 patients with and the 35 patients without HPS who died. In multivariate analysis, HPS was an independent predictor of survival besides age, Child-Pugh class, and blood urea nitrogen. Mortality correlates with severity of HPS. Conclusions: The presence of HPS independently worsens prognosis of patients with cirrhosis. This should influence patient management and scoring systems and accelerate the evaluation process for liver transplantation.

Section snippets

Patients

A total of 111 adult patients with biopsy examination-proven cirrhosis consecutively admitted to our department irrespective of the presence of HPS between November 1996 and September 1998 for further management with special regard on the evaluation for orthotopic liver transplantation (OLT) were recruited for this study. Indication for OLT was made independently of the presence of HPS according to standard criteria.20 All patients underwent transthoracic contrast echocardiography, arterial

Study population

Twenty-seven patients (24%) fulfilled the criteria for diagnosis of HPS. Their clinical characteristics in comparison with those patients without HPS are shown in Table 1. Mean age, sex, and cause of cirrhosis was comparable in both groups. Patients with HPS had a higher mean Child-Pugh score (10.8 vs. 9.4, P < 0.05) and Model of End-Stage Liver Disease score (20.6 vs. 16, P < 0.01). In concordance, mean total bilirubin level was significantly higher, and mean values of serum albumin and

Discussion

This prospective clinical study showed that the presence of HPS has a major influence on survival in patients with cirrhosis. Patients with HPS showed a 3.8-times lower median survival time compared with those without this syndrome. But even subgroup analysis according to liver disease severity showed a clear survival disadvantage of HPS patients in Child-Pugh class C (5-times lower median survival). In cirrhotic patients with Child-Pugh class B, the difference in survival was less substantial

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      Two single-centre studies and one prospective multicentre study have evaluated the natural history and prognosis of patients with HPS. The first single-centre study reported a median survival of 4.8 months for patients with HPS (n = 27), compared to 35.2 months for patients without HPS (n = 84).54 In the second study, patients with HPS (n = 37) had a median survival of 24 months and a 5-year survival rate of 23%, compared to a median survival of 87 months and a 5-year survival rate of 63% in patients without HPS (n = 47).8

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