背景:目前的观察性研究可能没有大量样本来研究右室流出道(RVOT)切口法洛四联症(TOF)完全修复后肺动脉瓣(PV)形态差异与结果之间的关系。本研究旨在评估肺静脉形态差异对TOF完全修复后结果的影响。方法:本研究为回顾性队列研究。纳入2012年1月至2017年12月在阜外医院行RVOT切口TOF修补术的连续患者,并根据PV形态差异进行比较(形态异常为单尖和双尖,而三尖瓣形态正常)。主要结局定义为死亡的复合,或重新干预,或显著的环形峰梯度(APG),或显著的肺反流(PR),以先发生者为准。多变量Cox模型分析用于评估PV形态差异与结局之间的关系。进行亚组分析和倾向评分分析作为敏感性分析,以评估我们结果的稳健性。结果:该队列共包括1,861例初步诊断为TOF的患者,1,688例接受RVOT切口CR-TOF。中位年龄为318天[四分位距(IQR):223-534天],男性体重中位数为8.9kg(IQR:7.6-10.5kg),占60.0%(1,011)。1,673例CR-TOF患者的完整随访数据,中位随访时间为49个月。随访时肺静脉形态正常患者的主要结局和显著APG的调整后风险较低[调整后风险比(HR):0.68;95%CI:0.46-0.98;调整后HR:0.22;95%CI:0.07-0.71]。随访期间主要结果的趋势保持不变,即使在亚组和倾向得分匹配分析中也是如此。结论:在对大型TOF队列数据的分析中,三尖瓣肺静脉正常的患者与主要结局的风险降低和显著APG的风险较低相关,与异常单叶或二叶PVs患者相比。
Background: Current observational studies may not have large samples to investigate the relationship between pulmonary valve (PV) morphology differences and outcomes after complete repair for tetralogy of Fallot (TOF) by right ventricular outflow tract (RVOT) incision. This study aimed to assess the impact of PV morphology differences on outcomes after complete repair for TOF. Methods: This is a retrospective cohort study. Consecutive patients who underwent TOF repair with RVOT incision at Fuwai Hospital from January 2012 to December 2017 were included and compared according to PV morphology differences (unicuspid or bicuspid was abnormal morphology, while the tricuspid valve was normal morphology). The primary outcome was defined as a composite of death, or reintervention, or significant annular peak gradient (APG), or significant pulmonary regurgitation (PR), whichever occurred first. Multivariable Cox model analysis was used to assess the relationships between PV morphology differences and outcomes. Subgroup analysis and Propensity-score analysis were performed as sensitivity analyses to assess the robustness of our results. Results: The cohort included a total of 1,861 patients with primary diagnosis of TOF, with 1,688 undergoing CR-TOF with RVOT incision. The median age was 318 days [interquartile range (IQR): 223-534 days], a median weight of 8.9 kg (IQR: 7.6-10.5 kg) and 60.0% (1,011) were male. Complete follow-up data were available for 1,673 CR-TOF patients with a median follow-up duration of 49 months. Adjusted risks for the primary outcome and significant APG were lower for patients with normal PV morphology at follow up [adjusted hazard ratio (HR): 0.68; 95% CI: 0.46-0.98; adjusted HR: 0.22; 95% CI: 0.07-0.71, respectively]. The trend for the primary outcome during follow-up remained unchanged, even in subgroups and propensity score matching analyses. Conclusions: In this analysis of data from a large TOF cohort, patients with normal tricuspid PVs were associated with a decreased risk of the primary outcome and a lower risk of significant APG, as compared with patients with abnormal unicuspid or bicuspid PVs.