Department of Catheterization Lab, Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
Department of Radiology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong, China.
Eur J Cardiothorac Surg. 2017 Aug 1;52(2):225-232. doi: 10.1093/ejcts/ezx064.
The prognosis of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA-VSD-MAPCAs) after surgery shows substantial clinical heterogeneity and predictors for outcomes are lacking. This study aimed to assess the predictive value of preoperative cardiac computed tomography angiography (CTA) for survival in patients with PA-VSD-MAPCAs.
We retrospectively analysed PA-VSD-MAPCA patients with preoperative CTA who underwent both right ventricular outflow tract reconstruction and MAPCA unifocalization ( n = 24) or pulmonary artery rehabilitation ( n = 28). The end-point was overall survival. Prognostic values of CTA were assessed using Cox univariate and multivariate analyses. The significant threshold of independent parameters was calculated using receiver-operating characteristic (ROC) curves.
During a median follow-up of 1145 days, a total of 13 deaths were observed. Multivariate analysis identified a high pulmonary vein index (PVI) [hazard ratio (HR) = 0.03; 95% confidence interval (CI): 0.03, 0.28; P < 0.01] and the presence of native pulmonary artery (HR = 0.06; 95% CI: 0.10, 0.35; P < 0.01) as independent positive predictors of better survival. The area under the ROC curve for PVI was 0.79 ( P < 0.01), and a cut-off point of 438 mm 2 /m 2 was deemed the significant threshold for survival (sensitivity 92%, specificity 72%).
Preoperational high PVI and native pulmonary artery presence were significant morphologic predictors of a positive survival advantage in PA-VSD-MAPCA patients. A PVI ≥438 mm 2 /m 2 may be a reliable positive prognosticator that could improve the decision-making strategy for PA-VSD-MAPCA patients.
肺动脉闭锁伴室间隔缺损合并主-肺动脉侧支（PA-VSD-MAPCA）患者术后的预后存在显著的临床异质性，且缺乏预后预测因素。本研究旨在评估术前心脏 CT 血管造影（CTA）对 PA-VSD-MAPCA 患者生存的预测价值。
我们回顾性分析了 24 例行右心室流出道重建和 MAPCA 归并（n=24）或肺动脉重建（n=28）的 PA-VSD-MAPCA 患者的术前 CTA。终点为总生存率。使用 Cox 单因素和多因素分析评估 CTA 的预后价值。使用受试者工作特征（ROC）曲线计算独立参数的显著阈值。
在中位随访 1145 天期间，共观察到 13 例死亡。多因素分析发现高肺静脉指数（PVI）[风险比（HR）=0.03；95%置信区间（CI）：0.03，0.28；P<0.01]和存在固有肺动脉（HR=0.06；95%CI：0.10，0.35；P<0.01）是生存的独立正预测因子。PVI 的 ROC 曲线下面积为 0.79（P<0.01），PVI≥438mm²/m² 被认为是生存的显著阈值（敏感性 92%，特异性 72%）。
术前高 PVI 和固有肺动脉的存在是 PA-VSD-MAPCA 患者生存优势的显著形态学预测因子。PVI≥438mm²/m² 可能是一个可靠的阳性预后预测因子，可以改善 PA-VSD-MAPCA 患者的决策策略。
