Abstract
Long-term prospective data on hepatopulmonary syndrome (HPS) from a large number of patients, especially in Asian patients, are lacking. We evaluated the long-term prognosis of HPS and the development of acute-on-chronic liver failure (ACLF), and related factors. A total of 142 patients with cirrhosis who underwent saline-agitated contrast echocardiography for the diagnosis of HPS were enrolled and observed prospectively from 2014 to 2019. A total of 59 patients (41%) were diagnosed with HPS (24 grade 1, 23 grade 2, 12 grade 3). Thirty-eight and 37 patients died in the HPS and non-HPS groups, respectively (p < 0.01). The 5-year survival rate was 47% in the HPS group and 62% in the non-HPS group. In the Cox proportional hazards model, HPS and Model for End-stage Liver Disease (MELD) score ≥ 18, and Child-Turcotte-Pugh (CTP) class B/C were significant risk factors for mortality after adjusting for other risk factors (HPS hazard ratio [HR] = 1.9, p = 0.01; MELD score ≥ 18 HR = 2.3, p < 0.01; CTP class B/C HR = 2.9, p < 0.01). Compared to that in non-HPS group, the HPS group had a significantly higher incidence of ACLF during follow-up (p < 0.01) and more frequently presented with lung involvement of ACLF (p = 0.03). In the long-term follow-up cohort, patients with HPS showed poorer prognosis than that of patients without HPS. HPS was a risk factor for ACLF development independent of hepatic dysfunction, and lung involvement was significantly common than without ACLF.
Highlights
Patients with cirrhosis diagnosed with hepatopulmonary syndrome had a poor prognosis and higher probability of Acute-on-Chronic failure than those who did not
While the prevalence of hepatopulmonary syndrome (HPS) varies, it is estimated to occur in approximately 4–32% of cirrhosis patients who are waiting for liver transplantation.[2,4,8−15] In our previous study of Korean patients with cirrhosis, the prevalence of HPS was relatively high, at 41.5%
In Model 1, HPS, albumin, and prothrombin time were identified as risk factors for mortality (HPS hazard ratio [HR] = 1.9, 95% confidence interval [CI] = 1.13–3.12, p = 0.01; albumin HR = 0.59, 95% CI = 0.35–1.00, p = 0.05; international normalized ratio (INR) HR = 1.8, 95% CI = 1.03–3.26, p = 0.04)
Summary
Hepatopulmonary syndrome (HPS) is characterized as a defect in arterial oxygenation caused by pulmonary vascular dilatation in the setting of chronic liver disease.[1,2,3,4,5,6] It is defined by positive echocardiography findings and a more than 15 mmHg increase in differentiation of oxygen pressure, inducing hypoxia and dyspnea, in patients with cirrhosis.[1,4,7] While the prevalence of HPS varies, it is estimated to occur in approximately 4–32% of cirrhosis patients who are waiting for liver transplantation.[2,4,8−15] In our previous study of Korean patients with cirrhosis, the prevalence of HPS was relatively high, at 41.5%.16There are no specific symptoms of HPS, and the exact pathogenic mechanism of HPS is not fully understood. The poor prognosis is generally known.[2,3,13,17] long-term prospective data from a large number of patients are lacking, especially in Asian countries. Longitudinal data of HPS and risk factors related to HPS remain insufficient. This study evaluated the long-term prognosis and the development of acute-on-chronic liver failure (ACLF) in HPS and its related factors based on a previously reported HPS study cohort at our institution.[16]. Long-term prospective data on hepatopulmonary syndrome (HPS) from a large number of patients, especially in Asian patients, are lacking. We evaluated the long-term prognosis of HPS and the development of acute-on-chronic liver failure (ACLF), and related factors
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