Department of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Academy of Medical Science/Guangdong Provincial People's Hospital, Guangzhou, Guangdong 510080, China.
Chin Med J (Engl). 2019 Apr 5;132(7):811-818. doi: 10.1097/CM9.0000000000000145.
Pulmonary artery hypertension associated with congenital heart disease (PAH-CHD) occurs predominantly among patients with uncorrected CHD. Treatment of severe pediatric PAH-CHD remains a major intractability. This study evaluated the predictors and prognoses of children with PAH-CHD who underwent surgical correction.
The data for 59 children with severe PAH-CHD who underwent surgical correction, with or without postoperative medication, between May 2011 and June 2015 at the Guangdong Provincial People's Hospital were analyzed retrospectively. A regression analysis, receiver-operating characteristic (ROC) curves, and Kaplan-Meier curves were used for survival analysis.
Fifty-nine children with severe PAH-CHD underwent heart catheterization and correction, with or without specific anti-PAH drugs postoperatively, were included in this study. The pulmonary pressure, heart function, and ending events were observed and median observation period was 49 ± 20 months. Twenty-eight patients (50%) received at least one additional anti-PAH drug after correction. The survival rate after 2 years was 91.5% (54/59); two patients were in a critical condition, and three were lost to follow-up. Twelve patients (29%) still received over one additional PAH-specific therapy at follow-up, whereas 42 (75%) had successfully stopped drug treatment. Two patients (3.5%) died and one underwent a second thoracotomy to remove the ventricular septal defect patch. Acute vasoreactivity test (AVT) criteria had limited efficacy in predicting pediatric PAH-CHD, whereas pulmonary vascular resistance (PVR) ≤ 6.65 Wood units (WU)/m or PVR/systemic vascular resistance (SVR) ≤ 0.39 during AVT indicated a good prognosis after surgical correction with an AUC of 98.3% (95% confidence interval [CI]: 96.0-100%), 98.4% (95% CI: 96.0-100%) sensitivity of 100%, 100% and specificity of 82.1%, 92.9%, respectively.
Although the criteria for positive AVT currently used are unsuitable for pediatric patients with PAH-CHD, PVR and PVR/SVR during AVT are excellent predictors of outcome in pediatric PAH-CHD. Surgery aided by anti-PAH drugs is an effective strategy and should be recommended for severe pediatric PAH-CHD with PVR ≤ 6.65 WU/m and PVR/SVR ≤ 0.39 after iloprost aerosol inhalation.
与先天性心脏病（CHD）相关的肺动脉高压（PAH-CHD）主要发生在未经矫正的 CHD 患者中。严重儿科 PAH-CHD 的治疗仍然是一个主要的难题。本研究评估了接受手术矫正的 PAH-CHD 患儿的预测因素和预后。
回顾性分析 2011 年 5 月至 2015 年 6 月在广东省人民医院接受手术矫正（伴或不伴术后药物治疗）的 59 例严重 PAH-CHD 患儿的资料。采用回归分析、受试者工作特征（ROC）曲线和 Kaplan-Meier 曲线进行生存分析。
本研究纳入了 59 例严重 PAH-CHD 患儿，这些患儿均接受了心脏导管检查和矫正，并在术后接受了特定的抗-PAH 药物治疗。观察了肺动脉压、心功能和终点事件，中位观察期为 49±20 个月。28 例（50%）患儿在矫正后至少接受了一种额外的抗-PAH 药物治疗。2 年后的生存率为 91.5%（54/59）；2 例患儿病情危急，3 例患儿失访。12 例（29%）患儿在随访时仍接受一种以上的额外 PAH 特异性治疗，而 42 例（75%）患儿成功停止了药物治疗。2 例患儿（3.5%）死亡，1 例患儿因室间隔缺损修补片切除而行二次开胸手术。急性血管反应试验（AVT）标准对预测儿科 PAH-CHD 的疗效有限，而 AVT 时的肺血管阻力（PVR）≤6.65 伍德单位（WU）/m 或 PVR/体循环阻力（SVR）≤0.39 则提示手术矫正后预后良好，其 AUC 为 98.3%（95%CI：96.0-100%），敏感性为 100%（95%CI：96.0-100%），特异性为 82.1%（95%CI：96.0-100%）和 92.9%（95%CI：96.0-100%）。
尽管目前使用的 AVT 阳性标准不适合儿科 PAH-CHD 患者，但 AVT 时的 PVR 和 PVR/SVR 是儿科 PAH-CHD 患者结局的良好预测指标。伊洛前列素气雾剂吸入后，PVR≤6.65 WU/m 和 PVR/SVR≤0.39 的严重儿科 PAH-CHD 患儿，在抗-PAH 药物辅助下进行手术是一种有效的治疗策略，应予以推荐。
