Hayes Don, Black Sylvester M, Tobias Joseph D, Kirkby Stephen, Mansour Heidi M, Whitson Bryan A
Department of Pediatrics, Ohio State University College of Medicine, Columbus, Ohio; Department of Internal Medicine, Ohio State University College of Medicine, Columbus, Ohio; Section of Pulmonary Medicine, Nationwide Children's Hospital, Columbus, Ohio.
Department of Surgery, Ohio State University College of Medicine, Columbus, Ohio.
Ann Thorac Surg. 2016 Jan;101(1):246-52. doi: 10.1016/j.athoracsur.2015.06.024. Epub 2015 Aug 25.
The influence of varying levels of pulmonary hypertension (PH) on survival in idiopathic pulmonary fibrosis is not well defined.
The United Network for Organ Sharing database was queried from 2005 to 2013 to identify first-time lung transplant candidates listed for lung transplantation who were tracked from waitlist entry date until death or censoring to determine the influence of PH on patients with advanced lung disease. Using data for right heart catheterization measurements, mild PH was defined as mean pulmonary artery pressure of 25 mm Hg or more, and severe as 35 mm Hg or more.
Of 6,657 idiopathic pulmonary fibrosis patients, 6,651 were used for univariate analysis, 6,126 for Kaplan-Meier survival function, 6,013 for multivariate Cox models, and 5,186 (mild PH) and 2,014 (severe PH) for propensity score matching, respectively. Univariate Cox proportional hazards analysis found significant differences in survival for mild PH (hazard ratio [HR] 1.689, 95% confidence interval [CI]: 1.434 to 1.988, p < 0.001) and severe PH (HR 2.068, 95% CI: 1.715 to 2.493, p < 0.001). Further assessment by multivariate Cox models identified significant risk for death for mild PH (HR 1.433, 95% CI: 1.203 to 1.706, p < 0.001) and severe PH (HR 1.597, 95% CI: 1.308 to 1.949, p < 0.001). Propensity score matching confirmed the risk for death for mild PH (HR 1.530, 95% CI: 1.189 to 1.969, p = 0.001) and severe PH (HR 2.103, 95% CI: 1.436 to 3.078, p < 0.001).
The manifestation of PH, even with mild severity, is associated with significantly increased risk for death among patients with idiopathic pulmonary fibrosis awaiting lung transplantation, so referral should be considered early in the disease course.
不同程度的肺动脉高压（PH）对特发性肺纤维化患者生存的影响尚不明确。
查询器官共享联合网络数据库2005年至2013年的数据，以确定首次被列入肺移植等待名单的肺移植候选者，从等待名单登记日期开始跟踪直至死亡或失访，以确定PH对晚期肺部疾病患者的影响。利用右心导管测量数据，轻度PH定义为平均肺动脉压25毫米汞柱或更高，重度PH定义为35毫米汞柱或更高。
6657例特发性肺纤维化患者中，6651例用于单因素分析，6126例用于Kaplan-Meier生存函数分析，6013例用于多因素Cox模型分析，5186例（轻度PH）和2014例（重度PH）用于倾向得分匹配。单因素Cox比例风险分析发现，轻度PH（风险比[HR]1.689，95%置信区间[CI]：1.434至1.988，p<0.001）和重度PH（HR 2.068，95%CI：1.715至2.493，p<0.001）患者的生存存在显著差异。多因素Cox模型进一步评估发现，轻度PH（HR 1.433，95%CI：1.203至1.706，p<0.001）和重度PH（HR 1.597，95%CI：1.308至1.949，p<0.001）患者有显著的死亡风险。倾向得分匹配证实了轻度PH（HR 1.530，95%CI：1.189至1.969，p = 0.001）和重度PH（HR 2.103，95%CI：1.436至3.078，p<0.001）患者的死亡风险。
即使是轻度的PH表现，也与等待肺移植的特发性肺纤维化患者的死亡风险显著增加相关，因此在疾病进程早期就应考虑转诊。
