Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic, Rochester, MN.
Division of Pulmonary and Critical Care Medicine, Department of Medicine, Mayo Clinic, Rochester, MN.
Chest. 2018 Feb;153(2):414-426. doi: 10.1016/j.chest.2017.09.035. Epub 2017 Oct 5.
Intrapulmonary vascular dilatations (IPVD) frequently are detected in patients with liver disease by the delayed appearance of microbubbles at contrast-enhanced echocardiography. IPVD with an elevated alveolar-arterial (A-a) gradient define hepatopulmonary syndrome (HPS); however, the importance of IPVD in the absence of abnormal gas exchange is unknown. We aimed to determine the clinical impact of IPVD in patients with liver disease.
We performed a cross-sectional study within the Pulmonary Vascular Complications of Liver Disease 2 Study, a multicenter, prospective cohort study of patients being evaluated for liver transplant. We excluded patients with obstructive or restrictive lung disease, HPS, or intracardiac shunting. We compared patients with and those without IPVD.
Forty-six patients with IPVD and 81 patients without IPVD were included. Patients with IPVD were more likely to have autoimmune hepatitis and less likely to have cryptogenic cirrhosis and hepatocellular carcinoma. Patients with IPVD had higher Child-Pugh scores (6 [interquartile range (IQR), 5-7] vs 5 [IQR, 4-7]; P = .04), possibly higher Model for End-Stage Liver Disease scores (14.5 [IQR, 11.6-15.8] vs 12.2 [IQR, 9.4-15.5]; P = .06), higher PaO levels (97.9 [IQR, 92.0-103.0] vs 89.0 [IQR, 82.0-96.9] mm Hg; P < .001), and lower A-a gradients (9.9 [IQR, 6.2-13.5] vs 14.9 [IQR, 9.0-21.8] mm Hg; P < .001). Symptoms and quality of life were similar between the groups.
Autoimmune hepatitis and increased liver disease severity were associated with the presence of IPVD, which was characterized by higher PaO levels. Future studies to better characterize IPVD pathogenesis and the relationship of IPVD to HPS are warranted.
在接受对比增强超声心动图检查的肝病患者中，常可发现肺内血管扩张（IPVD）。存在 IPVD 并伴有肺泡-动脉（A-a）梯度升高定义为肝肺综合征（HPS）；然而，在无异常气体交换的情况下，IPVD 的重要性尚不清楚。本研究旨在确定肝病患者中 IPVD 的临床意义。
我们在多中心前瞻性队列研究——肺血管并发症肝病 2 研究（Pulmonary Vascular Complications of Liver Disease 2 Study）中进行了一项横断面研究，该研究纳入了正在接受肝移植评估的患者。我们排除了存在阻塞性或限制性肺疾病、HPS 或心内分流的患者。我们比较了有和无 IPVD 的患者。
共纳入 46 例有 IPVD 和 81 例无 IPVD 的患者。有 IPVD 的患者更可能患有自身免疫性肝炎，而不太可能患有隐源性肝硬化和肝细胞癌。有 IPVD 的患者的 Child-Pugh 评分更高（6[四分位距（IQR），5-7]比 5[IQR，4-7]；P=0.04），可能有更高的终末期肝病模型评分（14.5[IQR，11.6-15.8]比 12.2[IQR，9.4-15.5]；P=0.06），更高的 PaO2 水平（97.9[IQR，92.0-103.0]比 89.0[IQR，82.0-96.9]mmHg；P<0.001），以及更低的 A-a 梯度（9.9[IQR，6.2-13.5]比 14.9[IQR，9.0-21.8]mmHg；P<0.001）。两组的症状和生活质量相似。
自身免疫性肝炎和肝病严重程度增加与 IPVD 有关，IPVD 的特征是 PaO2 水平升高。未来有必要进行进一步的研究以更好地阐明 IPVD 的发病机制以及 IPVD 与 HPS 的关系。
