van de Woestijne Pieter C, Romeo Jamie L R, van Beynum Ingrid, Witsenburg Maarten, Mokhles M Mostafa, Bogers Ad J J C
Department of Cardiothoracic Surgery, Erasmus University Medical Centre, Rotterdam, The Netherlands.
Department of Pediatric Cardiology, Erasmus University Medical Centre, Rotterdam, The Netherlands.
JTCVS Open. 2021 Sep 24;8:546-555. doi: 10.1016/j.xjon.2021.09.025. eCollection 2021 Dec.
Pulmonary atresia and ventricular septal defect (PA-VSD), with or without systemic pulmonary collateral arteries (SPCAs), represents a complex anatomic and surgical spectrum of congenital heart disease. Currently, there is limited evidence on homograft durability after complete correction, which potentially could be affected by anatomic differences in pulmonary vasculature.
This retrospective single-center study included all 69 consecutive PA-VSD patients (46 with SPCAs, 23 without SPCAs) operated on between 1978 and 2018. The primary interest was in homograft durability after complete repair. Longitudinal echocardiographic homograft function and right ventricular systolic pressure were analyzed with linear mixed-effects models.
The median duration of follow-up was 20 years. Of the 46 patients with SPCAs, 37 (80.4%) underwent biventricular correction at a median age of 2.7 years (interquartile range [IQR], 1.8-6.3 years). Two patients are currently awaiting unifocalization and correction. All 23 patients without SPCAs underwent successful complete correction at a median age of 1.6 years (IQR, 1.1-3.6 years). Freedom from any reintervention after 20 years was 15%. When a homograft was used during correction, freedom from homograft replacement after 20 years was comparable in the 2 groups ( = .925), at 32 ± 11% in the SPCA group and 32 ± 13% in the non-SPCA group. Indications for homograft replacement were isolated stenosis (n = 7; 46.7%), isolated regurgitation (n = 3; 20.0%), and mixed stenosis and regurgitation (n = 5; 33.3%) in the SPCA group and isolated stenosis (n = 8; 88.9%) and stenosis and regurgitation (n = 1; 11.1%) in the non-SPCA group. Peak homograft gradient was significantly ( = .0003) higher in patients without SPCA, with a comparable rate of progression in the 2 groups. However, the prevalence of severe pulmonary regurgitation (PR) was higher in patients with SPCAs, estimated at 35% at 10 years, compared with 15% in patients without SPCAs.
Homografts used for right ventricular outflow tract reconstruction in patients with PA-VSD, either with or without SPCAs, have similar limited durability. Repeated reintervention is common, and careful follow-up with attention to severe PR is warranted.
肺动脉闭锁合并室间隔缺损（PA-VSD），无论有无体肺侧支动脉（SPCAs），均代表先天性心脏病复杂的解剖和手术范畴。目前，关于完全矫正术后同种异体移植物耐久性的证据有限，这可能受肺血管解剖差异的影响。
这项回顾性单中心研究纳入了1978年至2018年间连续接受手术的69例PA-VSD患者（46例有SPCAs，23例无SPCAs）。主要关注完全修复后同种异体移植物的耐久性。采用线性混合效应模型分析纵向超声心动图同种异体移植物功能和右心室收缩压。
随访时间中位数为20年。46例有SPCAs的患者中，37例（80.4%）在中位年龄2.7岁（四分位间距[IQR]，1.8 - 6.3岁）时接受了双心室矫正。2例患者目前正在等待进行单灶化和矫正。所有23例无SPCAs的患者在中位年龄1.6岁（IQR，1.1 - 3.6岁）时成功完成了完全矫正。20年后无任何再次干预的比例为15%。矫正期间使用同种异体移植物时，2组患者20年后免于同种异体移植物置换的比例相当（P = 0.925），SPCA组为32 ± 11%，非SPCA组为32 ± 13%。SPCA组同种异体移植物置换的指征为单纯狭窄（n = 7；46.7%）、单纯反流（n = 3；20.0%）和混合性狭窄与反流（n = 5；33.3%），非SPCA组为单纯狭窄（n = 8；88.9%）和狭窄与反流（n = 1；11.1%）。无SPCA患者的同种异体移植物峰值梯度显著更高（P = 0.0003），2组进展速率相当。然而，有SPCAs患者的重度肺反流（PR）患病率更高，估计10年时为35%，而无SPCAs患者为15%。
用于PA-VSD患者右心室流出道重建的同种异体移植物，无论有无SPCAs，耐久性均相似且有限。重复再次干预很常见，因此有必要进行仔细的随访并关注重度PR。
