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肺动脉高压患者肺动脉瘤的频率、预测因素及其预后影响。

Frequency, Predictors, and Prognostic Impact of Pulmonary Artery Aneurysms in Patients With Pulmonary Arterial Hypertension.

机构信息

Department of Cardiology, Hospital Universitario 12 de Octubre, Madrid, Spain.

Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands.

出版信息

Am J Cardiol. 2019 Feb 1;123(3):474-481. doi: 10.1016/j.amjcard.2018.10.028. Epub 2018 Nov 6.

Abstract

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.

摘要

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。

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