Koth Andrew, Sidell Doug, Bauser-Heaton Holly, Wise-Faberowski Lisa, Hanley Frank L, McElhinney Doff B, Asija Ritu
1Division of Pediatric Cardiology,Stanford Hospital and Clinics,Stanford,CA,USA.
2Department of Anesthesia, Stanford University,Stanford,CA,USA.
Cardiol Young. 2019 Jan;29(1):19-22. doi: 10.1017/S1047951118001427. Epub 2018 Aug 30.
A 22q11 chromosome deletion is common in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals. We sought to determine whether 22q11 chromosome deletion is associated with increased postoperative morbidity after unifocalisation surgery.
We included all patients with this diagnosis undergoing primary or revision unifocalisation ± ventricular septal defect closure at our institution from 2008 to 2016, and we excluded patients with unknown 22q11 status. Demographic and surgical data were collected. We compared outcomes between those with 22q11 chromosome deletion and those without using non-parametric analysis.
We included 180 patients, 41% of whom were documented to have a chromosome 22q11 deletion. Complete unifocalisation was performed in all patients, and intracardiac repair was performed with similar frequency regardless of 22q11 chromosome status. Duration of mechanical ventilation was longer in 22q11 deletion patients. This difference remained significant after adjustment for delayed sternal closure and/or intracardiac repair. Duration of ICU stay was longer in patients with 22q11 deletion, although no longer significant when adjusted for delayed sternal closure and intracardiac repair. Finally, length of hospital stay was longer in 22q11-deleted patients, but this difference was not significant on unadjusted or adjusted analysis.
Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals and 22q11 deletion are at risk for greater prolonged mechanical ventilation after unifocalisation surgery. Careful attention should be given to the co-morbidities of this population in the perioperative period to mitigate risks that may complicate the postoperative course.
22q11染色体缺失在法洛四联症、肺动脉闭锁和主-肺动脉侧支血管粗大的患者中很常见。我们试图确定22q11染色体缺失是否与单灶化手术后术后发病率增加有关。
我们纳入了2008年至2016年在我们机构接受初次或翻修单灶化手术±室间隔缺损修补术的所有此类诊断患者，并排除了22q11状态不明的患者。收集了人口统计学和手术数据。我们使用非参数分析比较了有22q11染色体缺失和没有22q11染色体缺失的患者的结局。
我们纳入了180例患者，其中41%被记录有22q11染色体缺失。所有患者均进行了完全单灶化手术，无论22q11染色体状态如何，心脏内修复的频率相似。22q11缺失患者的机械通气时间更长。在调整了延迟胸骨闭合和/或心脏内修复后，这种差异仍然显著。22q11缺失患者的重症监护病房住院时间更长，尽管在调整了延迟胸骨闭合和心脏内修复后不再显著。最后，22q11缺失患者的住院时间更长，但在未调整或调整分析中，这种差异均不显著。
患有法洛四联症、肺动脉闭锁和主-肺动脉侧支血管粗大且有22q11缺失的儿童在单灶化手术后有更长时间机械通气延长的风险。在围手术期应仔细关注该人群的合并症，以降低可能使术后病程复杂化的风险。
