Spearman Andrew D, Ginde Salil, Goot Benjamin H, Schaal Amy M, Feng Mingen, Pan Amy Y, Frommelt Michele A, Frommelt Peter C
Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Children's Wisconsin, Herma Heart Institute, 9000 West Wisconsin Avenue, Milwaukee, WI, 53226, USA.
Cardiovascular Center, Medical College of Wisconsin, 8701 West Watertown Plank Road, Milwaukee, WI, 53226, USA.
Pediatr Cardiol. 2020 Dec;41(8):1632-1638. doi: 10.1007/s00246-020-02421-z. Epub 2020 Jul 24.
Individuals with single ventricle congenital heart disease (CHD) undergo multiple staged surgical palliations. Staged single ventricle palliation with a superior cavopulmonary connection (SCPC) in infancy followed by a Fontan in early childhood relies on passive, unobstructed pulmonary blood flow and normal pulmonary vasculature. We hypothesized that patients with echocardiographic identification of retrograde flow in a branch pulmonary artery (PA) after SCPC or Fontan are at increased risk for adverse outcomes. We conducted a retrospective chart review of patients seen at Children's Wisconsin from 1999 to 2019. Inclusion criteria included a history of single ventricle congenital heart disease and surgical palliation with a superior cavopulmonary connection (SCPC). We created two cohorts based on transthoracic echocardiographic identification of branch PA flow patterns: those with color Doppler-defined pulmonary artery flow reversal (PA reversal cohort) and those with normal anterograde flow (Non-reversal cohort). We identified 21 patients in the PA reversal cohort and 539 patients in the Non-reversal cohort. The PA reversal cohort had increased hospital length of stay after SCPC palliation (p < 0.001) and decreased transplant-free survival (p = 0.032), but there was no difference in overall survival (p = 0.099). There was no difference in hospital length of stay after Fontan (p = 0.17); however, the PA reversal cohort was significantly less likely to progress to Fontan palliation during early childhood (p = 0.005). Echocardiographic color Doppler identification of branch PA flow reversal in patients with single ventricle physiology is a high-risk indicator for adverse short- and long-term outcomes.
患有单心室先天性心脏病（CHD）的个体需要接受多次分期手术姑息治疗。婴儿期进行上腔静脉-肺动脉连接（SCPC）的分期单心室姑息治疗，随后在儿童早期进行Fontan手术，依赖于被动、无阻碍的肺血流和正常的肺血管系统。我们假设，在SCPC或Fontan术后经超声心动图识别出分支肺动脉（PA）存在逆向血流的患者发生不良结局的风险增加。我们对1999年至2019年在威斯康星儿童医院就诊的患者进行了回顾性病历审查。纳入标准包括单心室先天性心脏病病史和上腔静脉-肺动脉连接（SCPC）手术姑息治疗。我们根据经胸超声心动图识别的分支PA血流模式创建了两个队列：那些经彩色多普勒定义为肺动脉血流逆转的患者（PA逆转队列）和那些具有正常顺行血流的患者（非逆转队列）。我们在PA逆转队列中识别出21例患者，在非逆转队列中识别出539例患者。PA逆转队列在SCPC姑息治疗后住院时间延长（p < 0.001），无移植生存率降低（p = 0.032），但总体生存率无差异（p = 0.099）。Fontan术后住院时间无差异（p = 0.17）；然而，PA逆转队列在儿童早期进展为Fontan姑息治疗的可能性显著降低（p = 0.005）。超声心动图彩色多普勒识别单心室生理患者的分支PA血流逆转是不良短期和长期结局的高风险指标。
