Yan Xinjian, Cen Jianzheng, Luo Xiaokang, Chen Jimei, Wen Shusheng, Wu Jinlin, Zhuang Jian
Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China.
Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China.
Transl Pediatr. 2022 Nov;11(11):1813-1822. doi: 10.21037/tp-22-491.
The early diagnosis of unilateral absence of pulmonary artery (UAPA) in children offers an opportunity for effective intervention. Due to the lack of clinical evidence, a consensus regarding surgical treatment has yet to be reported. The aim of this study is to evaluate the effectiveness and safety of pulmonary artery (PA) reconstruction with a "two-segment" technique to repair UAPA in patients with pulmonary hypertension.
Intraoperatively, the ligamentum arteriosum connecting the innominate artery and distal PA was dissected and occluded. A conduit created by fresh autologous pericardium formed the first "segment" of the neo-PA. The second "segment" was a Gore vascular graft with integrated rings anastomosed between the proximal end of the pericardial conduit and the main pulmonary artery (MPA).
A total of five consecutive patients were included, and the absent PA was successfully reconstructed using the "two-segment" technique in all patients. Following revascularization, the direct measurement of the pressure in MPA during the operation showed that the average mean pulmonary artery pressure (mPAP) decreased from 31.3±16.0 to 16.8±4.2 mmHg (P=0.047). The average mPAP/radial mean arterial pressure (rMAP) ratio decreased from 0.59±0.27 preoperatively to 0.30±0.10 postoperatively (P=0.028). The mean follow-up period was 18.85±4.67 months. The median diameter of the reconstructed PA (pericardial segment) measured by transthoracic echocardiography (TTE) was 6.1 mm. One patient safely underwent a redo operation to repair relative stenosis in the neo-PA.
Early PA reconstruction may effectively alleviate pulmonary hypertension in children with UAPA. The "two-segment" technique is safe and can facilitate potential redo pulmonary arterioplasty. Anticoagulation and antiplatelet therapy, as well as frequent follow-up, is required after the operation.
儿童单侧肺动脉缺如（UAPA）的早期诊断为有效干预提供了机会。由于缺乏临床证据，尚未有关于手术治疗的共识报道。本研究的目的是评估采用“两段式”技术进行肺动脉（PA）重建修复肺动脉高压患者UAPA的有效性和安全性。
术中，解剖并结扎连接无名动脉和远端PA的动脉导管。由新鲜自体心包制成的管道构成新PA的第一“段”。第二“段”是带一体化环的戈尔血管移植物，吻合于心包管道近端与主肺动脉（MPA）之间。
共纳入5例连续患者，所有患者均成功采用“两段式”技术重建缺如的PA。血运重建后，术中直接测量MPA压力显示，平均肺动脉压（mPAP）从31.3±16.0降至16.8±4.2 mmHg（P = 0.047）。平均mPAP/桡动脉平均压（rMAP）比值从术前的0.59±0.27降至术后的0.30±0.10（P = 0.028）。平均随访期为18.85±4.67个月。经胸超声心动图（TTE）测量重建PA（心包段）的中位直径为6.1 mm。1例患者安全地接受了再次手术以修复新PA中的相对狭窄。
早期PA重建可有效缓解UAPA患儿的肺动脉高压。“两段式”技术安全，可便于潜在的再次肺动脉成形术。术后需要抗凝和抗血小板治疗以及频繁随访。
