Division of Pulmonary, Allergy, and Critical Care Medicine, University of Alabama at Birmingham (UAB), Birmingham, AL, United States of America.
Gregory Fleming James Cystic Fibrosis Research Center, Birmingham, AL, United States of America.
PLoS One. 2020 Feb 20;15(2):e0229173. doi: 10.1371/journal.pone.0229173. eCollection 2020.
Pulmonary artery (PA) enlargement, defined as pulmonary artery to ascending aorta diameter ratio (PA:A)>1 on computed tomography (CT), is a marker of pulmonary vascular disease in chronic lung diseases. PA enlargement is prevalent in cystic fibrosis (CF), but its relationship to hemodynamics and prognostic utility in severe CF are unknown. We hypothesized that the PA:A would have utility in identifying pulmonary hypertension (PH) in severe CF and that PA enlargement would be associated with reduced transplant-free survival.
We conducted a retrospective study of adults with CF undergoing lung transplant evaluation at a single center between 2000 and 2015. CT, right heart catheterization (RHC), and clinical data were collected. The PA:A was measured from a single CT slice. We measured associations between PA:A and invasive hemodynamic parameters including PH defined as a mPAP ≥25mmHg using adjusted linear and logistic regression models. Kaplan-Meier and adjusted Cox regression models were used to measure associations between PA:A>1, RHC-defined PH, and transplant-free survival in severe CF.
We analyzed 78 adults with CF that had CT scans available for review, including 44 that also had RHC. RHC-defined PH defined as a mPAP ≥25mmHg was present in 36% of patients with CF undergoing transplant evaluation. The PA:A correlated with mPAP (r = 0.73; 95% CI 3.87-7.80; p<0.001) and PVR (r = 0.42, p = 0.005) and the PA:A>1 was an independent predictor of PH (aOR 4.50; 95% CI 1.05-19.2; p = 0.042). PA:A>1 was independently associated with increased hazards for death or transplant (aHR 2.69; 95% CI 1.41-5.14; P = 0.003). The presence of mPAP ≥25mmHg was independently associated with decreased survival in this cohort.
PA enlargement is associated with pulmonary hemodynamics and PH in severe CF. PA enlargement is an independent prognostic indicator of PH and decreased survival in this population.
肺动脉（PA）扩张，定义为 CT 上肺动脉与升主动脉直径比（PA:A）>1，是慢性肺部疾病中肺血管疾病的标志物。PA 扩张在囊性纤维化（CF）中很常见，但它与血液动力学的关系及其在严重 CF 中的预后应用尚不清楚。我们假设 PA:A 将有助于识别严重 CF 中的肺动脉高压（PH），并且 PA 扩张与无移植生存率降低相关。
我们对 2000 年至 2015 年期间在一家中心接受肺移植评估的成年 CF 患者进行了回顾性研究。收集 CT、右心导管检查（RHC）和临床数据。从单个 CT 切片测量 PA:A。我们使用调整后的线性和逻辑回归模型测量了 PA:A 与包括 PH 定义为 mPAP≥25mmHg 的有创血液动力学参数之间的关联。Kaplan-Meier 和调整后的 Cox 回归模型用于测量 PA:A>1、RHC 定义的 PH 与严重 CF 无移植生存率之间的关联。
我们分析了 78 名有 CT 扫描可供复查的 CF 患者，其中 44 名患者还进行了 RHC。接受移植评估的 CF 患者中有 36%存在 RHC 定义的 PH，定义为 mPAP≥25mmHg。PA:A 与 mPAP（r = 0.73；95%CI 3.87-7.80；p<0.001）和 PVR（r = 0.42，p = 0.005）相关，PA:A>1 是 PH 的独立预测因子（aOR 4.50；95%CI 1.05-19.2；p = 0.042）。PA:A>1 与死亡或移植的风险增加独立相关（aHR 2.69；95%CI 1.41-5.14；P = 0.003）。在该队列中，mPAP≥25mmHg 的存在与生存率降低独立相关。
PA 扩张与严重 CF 中的肺血液动力学和 PH 相关。PA 扩张是该人群 PH 和生存率降低的独立预后指标。
