Yamaguchi Akira, Iwamoto Shintaro
Division of Cardiovascular Surgery, National Center for Child Health and Development, Tokyo, Japan.
Biostatistics Unit, Department of Data Science, National Center for Child Health and Development, Tokyo, Japan.
Ann Thorac Surg Short Rep. 2024 Feb 28;2(3):385-389. doi: 10.1016/j.atssr.2024.01.016. eCollection 2024 Sep.
The primary treatment for hypoplastic left heart syndrome (HLHS) is the Fontan pathway, which entails performing the Glenn procedure. We hypothesized that the superior vena cava in patients with HLHS was short. As the length of the superior vena cava influences the Glenn procedure, we compared its length between patients with HLHS and those with other congenital heart diseases.
Patients with HLHS or its variant, patients with ventricular septal defects (VSD), and patients with pulmonary atresia with intact ventricular septum (PA/IVS)-including critical pulmonary stenosis-were enrolled in this study. The effective superior vena cava ratio (ESCVR), which is defined as the inferior border of the left brachiocephalic vein to the superior surface of the right pulmonary artery/height, was measured.
The median ESVCR of the HLHS, VSD, and PA/IVS patients was 12.54 mm/m, 17.96 mm/m, and 18.46 mm/m, respectively. ESVCR of the HLHS group was significantly smaller than that of the other groups ( = .0013 vs VSD group, = .0002 vs PA/IVS group).
Patients with HLHS have a relatively short superior vena cava, which may complicate the Glenn procedure.
左心发育不全综合征（HLHS）的主要治疗方法是采用Fontan路径，这需要进行格林手术。我们推测HLHS患者的上腔静脉较短。由于上腔静脉的长度会影响格林手术，我们比较了HLHS患者与其他先天性心脏病患者上腔静脉的长度。
本研究纳入了HLHS患者或其变异型患者、室间隔缺损（VSD）患者以及室间隔完整的肺动脉闭锁（PA/IVS）患者（包括重度肺动脉狭窄患者）。测量有效上腔静脉比率（ESCVR），其定义为左头臂静脉下缘至右肺动脉上表面的距离/身高。
HLHS、VSD和PA/IVS患者的ESCVR中位数分别为12.54mm/m、17.96mm/m和18.46mm/m。HLHS组的ESCVR显著小于其他组（与VSD组相比P = 0.0013，与PA/IVS组相比P = 0.0002）。
HLHS患者的上腔静脉相对较短，这可能会使格林手术复杂化。
