Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing.
Department of Rheumatology, The Affiliated Wuxi No.2 People's Hospital of Nanjing Medical University, Wuxi.
Rheumatology (Oxford). 2020 Nov 1;59(11):3221-3228. doi: 10.1093/rheumatology/keaa100.
Pulmonary artery enlargement is a common manifestation of chest CT in patients with pulmonary arterial hypertension (PAH). The exact clinical significance of this phenomenon has not been clarified in connective tissue disease (CTD)-associated PAH (CTD-PAH). We aimed to explore the association between the dilatation of pulmonary artery and prognosis of CTD-PAH patients.
We retrospectively investigated 140 CTD-PAH patients diagnosed by echocardiography from 2009 to 2018. A chest multi-slice CT was performed on all the patients. Main pulmonary artery (MPA), right pulmonary artery (RPA), left pulmonary artery (LPA), ascending aorta (AAo) and descending aorta (DAo) diameters were measured. The ratios MPA/AAo and MPA/DAo were also calculated. The primary end point was all-cause mortality.
During the observational period of 3.44 (0.23) years, 36 patients were followed to death. Cox univariate proportional hazard analysis showed that age, gender, MPA diameter, LPA diameter and RPA diameter were related to the risk of 5-year all-cause mortality in patients with CTD-PAH. In Cox multivariate proportional hazard analysis, MPA diameter and gender were predictors of all-cause mortality in CTD-PAH patients. An all-cause mortality risk prediction model revealed that baseline MPA diameter has the ability to predict 5-year all-cause mortality in CTD-PAH patients. Kaplan-Meier analysis showed that the 5-year survival rate was significantly lower in patients with MPA ≥37.70 mm (P ≤ 0.00012) compared with MPA ≤ 37.70 mm.
MPA diameter ≥37.70 mm measured by chest multi-slice CT was a potential independent risk factor of the poor long-term prognosis in Chinese CTD-PAH patients.
肺动脉扩张是肺动脉高压（PAH）患者胸部 CT 的常见表现。在结缔组织病（CTD）相关 PAH（CTD-PAH）中，这种现象的确切临床意义尚未阐明。我们旨在探讨肺动脉扩张与 CTD-PAH 患者预后的关系。
我们回顾性调查了 2009 年至 2018 年间通过超声心动图诊断的 140 例 CTD-PAH 患者。所有患者均行胸部多层螺旋 CT 检查。测量主肺动脉（MPA）、右肺动脉（RPA）、左肺动脉（LPA）、升主动脉（AAo）和降主动脉（DAo）直径。还计算了 MPA/AAo 和 MPA/DAo 比值。主要终点是全因死亡率。
在 3.44±0.23 年的观察期内，36 例患者死亡。Cox 单因素比例风险分析显示，年龄、性别、MPA 直径、LPA 直径和 RPA 直径与 CTD-PAH 患者 5 年全因死亡率风险相关。在 Cox 多因素比例风险分析中，MPA 直径和性别是 CTD-PAH 患者全因死亡率的预测因素。全因死亡率风险预测模型显示，基线 MPA 直径能够预测 CTD-PAH 患者 5 年全因死亡率。Kaplan-Meier 分析显示，MPA≥37.70mm 的患者 5 年生存率明显低于 MPA≤37.70mm 的患者（P≤0.00012）。
胸部多层螺旋 CT 测量的 MPA 直径≥37.70mm 是中国 CTD-PAH 患者预后不良的潜在独立危险因素。
