Department of Cardiology, Hospital Universitario 12 de Octubre, Madrid, Spain.
Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands.
Am J Cardiol. 2019 Feb 1;123(3):474-481. doi: 10.1016/j.amjcard.2018.10.028. Epub 2018 Nov 6.
Detection of pulmonary artery aneurysms (PAA) in pulmonary arterial hypertension (PAH) is increasing. We sought to determine the frequency of PAA in a PAH cohort, variables related to its development and its prognostic impact. We conducted a retrospective analysis of PAH patients who underwent a computed tomography or magnetic resonance. PAA was defined as a pulmonary artery >40 mm. Baseline, echocardiographic, and hemodynamic findings at PAH diagnosis were compared. Freedom from death or lung transplant was estimated by Kaplan-Meier method and compared by log-rank test. Predictors of PAA development were analyzed with multivariate models. Two-hundred patients underwent a computed tomography and/or magnetic resonance. In 77 (38%), a PAA (48.3 ± 7.2 mm) was detected. Time-course (months) of PAH was an independent risk factor for PAA (hazard ratio 1.01; 95% confidence interval 1.002 to 1.019; p = 0.016) whilst connective tissue disease was associated with a lower risk (hazard ratio 0.236; 95% confidence interval 0.060 to 0.920; p = 0.037). PAA patients showed lower rates of death and lung transplant from PAH diagnosis (p = 0.005), but no differences appeared when survival analysis was performed from first imaging test (p = 0.269). PAA patients presented a nonsignificant higher rate of sudden death (5% PAA vs 1% no-PAA; p = 0.073). In conclusion, the frequency of PAA was 38%. PAH time-course was an independent risk factor for PAA development whereas connective tissue disease -related PAH patients showed a lower risk. PAA patients showed lower rates of death or lung transplant from PAH diagnosis but no differences were found from imaging test. PAA patients had a nonsignificant higher rate of sudden death.
肺动脉瘤(PAA)在肺动脉高压(PAH)中的检出率正在增加。我们旨在确定 PAH 患者队列中 PAA 的发生率、与其发生相关的变量以及其预后影响。我们对接受计算机断层扫描或磁共振检查的 PAH 患者进行了回顾性分析。将肺动脉直径>40mm 定义为 PAA。比较 PAH 诊断时的基线、超声心动图和血流动力学发现。采用 Kaplan-Meier 法估计无死亡或肺移植的生存率,并采用对数秩检验进行比较。采用多变量模型分析 PAA 发生的预测因素。200 例患者接受了计算机断层扫描和/或磁共振检查。其中 77 例(38%)检测到 PAA(48.3±7.2mm)。PAH 的时间进程(月)是 PAA 的独立危险因素(风险比 1.01;95%置信区间 1.002 至 1.019;p=0.016),而结缔组织疾病与较低的风险相关(风险比 0.236;95%置信区间 0.060 至 0.920;p=0.037)。PAA 患者从 PAH 诊断起的死亡率和肺移植率较低(p=0.005),但首次影像学检查时的生存分析未见差异(p=0.269)。PAA 患者的猝死率较高(5% PAA 与 1% 无 PAA;p=0.073)。总之,PAA 的发生率为 38%。PAH 的时间进程是 PAA 发生的独立危险因素,而结缔组织疾病相关的 PAH 患者的风险较低。PAA 患者从 PAH 诊断起的死亡率和肺移植率较低,但影像学检查未见差异。PAA 患者的猝死率较高,但无统计学意义。