Background and aims

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.

Methods

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.

Results

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).

Conclusions

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.

中文翻译：

---

肝肺综合征与肝硬化患者发生急转慢性肝功能衰竭和预后不良有关

背景和目标

缺乏来自大量患者，尤其是亚洲患者的肝肺综合征 (HPS) 的长期前瞻性数据。我们评估了 HPS 的长期预后和急性慢性肝衰竭 (ACLF) 的发展以及相关因素。

方法

从 2014 年到 2019 年，共有 142 名接受盐水搅拌对比超声心动图诊断 HPS 的肝硬化患者入组并进行前瞻性观察。

结果

共有 59 名患者 (41%) 被诊断为 HPS（24 名 1 级、23 名 2 级、12 名 3 级）。HPS 组和非 HPS 组分别有 38 和 37 名患者死亡（p  < 0.01）。HPS 组的 5 年生存率为 47%，非 HPS 组为 62%。在 Cox 比例风险模型中，HPS 和终末期肝病模型 (MELD) 评分 ≥ 18 和 Child-Turcotte-Pugh (CTP) 等级 B/C 是调整其他风险因素 (HPS) 后死亡率的重要危险因素风险比 [HR] = 1.9，p  = 0.01；MELD 评分 ≥ 18 HR = 2.3，p  < 0.01；CTP B/C 类 HR = 2.9，p  < 0.01）。与非 HPS 组相比，HPS 组随访期间 ACLF 发生率显着升高（p < 0.01) 并且更常表现为 ACLF 的肺部受累 ( p  = 0.03)。

结论

在长期随访队列中，HPS 患者的预后比非 HPS 患者差。HPS 是 ACLF 发展的危险因素，与肝功能障碍无关，与没有 ACLF 的情况相比，肺部受累更为常见。