Division of Cardiology, Department of Pediatrics, Columbia University Vagelos College of Physicians and Surgeons, NewYork-Presbyterian Morgan Stanley Children's Hospital, New York, New York, USA; Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
Division of Pediatric Cardiology, Perelman School of Medicine at the University of Pennsylvania, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA; Division of Pediatric Cardiology, Washington University School of Medicine and St Louis Children's Hospital, St Louis, Missouri, USA.
J Am Coll Cardiol. 2023 Aug 15;82(7):615-627. doi: 10.1016/j.jacc.2023.05.051.
Neonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy.
The objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population.
Neonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <-2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth.
We included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were -2.19 (25th-75th percentile: -2.55 to -1.94) and -2.23 (25th-75th percentile: -2.64 to -1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001).
Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF.
患有肺动脉发育不良（hPA）的症状性法洛四联症（sTOF）新生儿被认为是高危人群。需要数据来告知 hPA 对结局的影响，以及该人群中肺动脉（PA）生长的理想管理策略。
本研究的目的是量化 hPA 对 sTOF 新生儿结局的影响，并衡量该人群中策略对 PA 生长的影响。
从先天性心脏病研究协作组回顾了 2005 年至 2017 年患有 sTOF 的新生儿。hPA 的标准包括单侧 PA z 评分<-2.0 和对侧 PA z 评分<0。主要结局是死亡率。次要结局包括再干预和 PA 生长。
我们纳入了 542 例患有 sTOF 的新生儿，其中 188 例（35%）有 hPA，354 例（65%）有正常 PA，中位随访时间为 4.1 年。中位右 hPA 和左 hPA z 评分分别为-2.19（25 至 75 百分位数：-2.55 至-1.94）和-2.23（25 至 75 百分位数：-2.64 至-1.91）。分期修复（与初次 TOF 修复相比）在 hPA 组中较不常见（36%比 44%；P=0.07）。两组的生存率相似（未调整的 P=0.16；调整后的 P=0.25）。hPA 组的再干预更常见（HR：1.28；95%CI：1.01-1.63；P=0.044）；在明确修复后没有差异（HR：1.21；95%CI：0.93-1.58；P=0.16）。hPA 组的 PA 在 1 年内生长更大，特别是右 PA（P<0.001）。
尽管有这种看法，但 sTOF 新生儿中存在 hPA 并不会增加死亡率或明确修复后再干预的整体危险。hPA 组的 PA 生长更好。这些发现表明，hPA 的存在不会对 sTOF 的结局产生不利影响。
