Januszewska Katarzyna, Nawrocki Pawel, Lehner Anja, Stegger Julia, Kleinerueschkamp Felix, Malec Edward
Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, Westphalian Wilhelm University of Muenster, University Hospital Muenster, Muenster, Germany.
Department of Pediatric Cardiology and Pediatric Intensive Care, Ludwig-Maximilian-University, Klinikum Großhadern, Munich, Germany.
Eur J Cardiothorac Surg. 2020 Jun 1;57(6):1098-1104. doi: 10.1093/ejcts/ezz376.
The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation.
A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3-9.8) years and median weight 12.7 (range 7.6-26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n = 129) with the shunt on the left and right-sided RV-PA (n = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively.
Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P = 0.001 and P = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P = 0.001), but there is no evidence of the impact on the development of the left PAs (P = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P = 0.14).
Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs.
右心室至肺动脉（RV-PA）分流术在诺伍德手术后可提供稳定的血流动力学，但会影响中央肺动脉（PA）的发育。本研究的目的是分析在进行Fontan手术前，左心发育不全综合征患儿中央PA的几何形态与RV-PA分流部位及二期手术类型的关系。
共有161例左心发育不全综合征患儿，中位年龄2.7岁（范围1.3 - 9.8岁），中位体重12.7 kg（范围7.6 - 26.1 kg），在接受诺伍德手术并进行RV-PA分流术后接受Fontan手术。患者分为两组：左侧RV-PA（L-RV-PA）组（n = 129），分流位于左侧；右侧RV-PA组（n = 32），分流位于新主动脉右侧。对Fontan手术前及所有心导管介入治疗前获得的血管造影数据进行回顾性分析。
在第二阶段和第三阶段之间，以及在Fontan手术即将进行之前，L-RV-PA组需要更多的PA导管介入治疗（P = 0.001和P = 0.03）。在该组中，左PA最小直径小于R-RV-PA组（P = 0.021）。在L-RV-PA组中，将分流开放至Fontan手术会增加PA介入治疗的发生率（P = 0.001），但没有证据表明对左PA的发育有影响（P = 0.075）。也没有证据表明二期手术类型会影响Fontan手术前的介入治疗率（P = 0.14）。
进行L-RV-PA分流的患儿需要更多的PA导管介入治疗。右侧RV-PA分流以及随后在分流部位进行的格林吻合术与中央PA无扭曲且更对称的发育相关。
