Zhu Yifan, Jiang Qi, Zhang Wen, Hu Renjie, Dong Wei, Zhang Hao, Zhang Haibo
Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai, China.
Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Front Cardiovasc Med. 2022 Sep 27;9:999032. doi: 10.3389/fcvm.2022.999032. eCollection 2022.
Truncus arteriosus (TA) is a rare congenital heart disease with a high rate of early mortality. The occurrence of post-operative pulmonary hypertension crisis (PHC), known to be a common and life-threatening complication, increases due to the irreversible development of pulmonary vascular resistance with age. We sought to figure out the risk factors for PHC and describe the surgical outcomes of TA patients with late referral (repair <1 month excluded).
We retrospectively reviewed patients after TA repair between 2009 and 2021 at Shanghai Children's Medical Center. The occurrence of PHC was defined according to post-operative Pp/Ps ≥ 1 and clinical manifestations. Risk factors for PHC and mortality were conducted by multivariable analysis.
A total of 98 patients were treated, including 55 males and 43 females. The median age at repair was 121 (69, 245) days. Post-operative PHC occurred in 22 (22.4%) patients with a median age of 186 (122, 293) days. By multivariable analysis, patients with the sum of Z-score of pre-operative bilateral pulmonary artery (PA) diameters (OR: 1.6, 95% CI: 1.2-2.3, = 0.01) was more likely to experience PHC. Longer CPB duration contributed to early death (OR: 1.0, 95% CI: 1.0-1.0, = 0.01). Total survival at 10 years was 81.4%. In 4.5 (2.9, 7.5) years of follow-up, twenty-six patients received 30 reinterventions. Valved reconstruction of RVOT most predicted reinterventions (OR: 4.2, 95% CI: 1.4-13.0, = 0.01).
Surgical repair of TA patients with late referral has resulted in comparatively favorable early and mid-term outcomes. PHC occurred more commonly in patients with overextended bilateral PA pre-operatively. Meanwhile, valved reconstruction of RVOT would more likely lead to early reintervention.
动脉干（TA）是一种罕见的先天性心脏病，早期死亡率很高。术后肺动脉高压危象（PHC）是一种常见且危及生命的并发症，随着年龄增长，肺血管阻力不可逆地发展，其发生率会增加。我们试图找出PHC的危险因素，并描述晚期转诊（排除<1个月时进行修复）的TA患者的手术结果。
我们回顾性分析了2009年至2021年在上海儿童医学中心接受TA修复术后的患者。PHC的发生根据术后Pp/Ps≥1及临床表现来定义。通过多变量分析确定PHC和死亡率的危险因素。
共治疗98例患者，其中男性55例，女性43例。修复时的中位年龄为121（69，245）天。22例（22.4%）患者术后发生PHC，中位年龄为186（122，293）天。通过多变量分析，术前双侧肺动脉（PA）直径Z评分总和较高的患者（比值比：1.6，95%置信区间：1.2 - 2.3，P = 0.01）更易发生PHC。体外循环时间延长会导致早期死亡（比值比：1.0，95%置信区间：1.0 - 1.0，P = 0.01）。10年总生存率为81.4%。在4.5（2.9，7.5）年的随访中，26例患者接受了30次再次干预。右心室流出道带瓣重建最易导致再次干预（比值比：4.2，95%置信区间：1.4 - 13.0，P = 0.01）。
晚期转诊的TA患者进行手术修复已取得了相对良好的早期和中期结果。术前双侧PA过度扩张的患者更易发生PHC。同时，右心室流出道带瓣重建更易导致早期再次干预。
