Carotti Adriano, Albanese Sonia B, Di Donato Roberto M
Department of Medical and Surgical Paediatric Cardiology, Ospedale Pediatrico Bambino Gesù, Scientific Institute (IRCCS), Rome, Italy.
Acta Paediatr Suppl. 2006 Jul;95(452):22-6. doi: 10.1111/j.1651-2227.2006.tb02410.x.
To correlate anatomic and genetic features of paediatric patients with pulmonary atresia, ventricular septal defect (VSD) and multiple aortopulmonary collateral arteries with surgical outcome.
44 consecutive patients aged 33 +/- 40 mo underwent either primary one-stage unifocalization (n = 32) or palliative right ventricular outflow tract reconstruction (n = 12) followed by secondary unifocalization and repair (n = 10) based on preoperative morphometric and functional evaluation of pulmonary blood sources. Chromosome 22q11.2 microdeletion occurred in 41% of cases. Combined VSD closure during one-stage procedures was guided by an intraoperative pulmonary flow study. Complete repair was accomplished in 35 cases (83%, 95% CI 72-95%). Variables examined included occurrence of confluent intrapericardial pulmonary arteries, central pulmonary arteries, confluent intraparenchymal pulmonary arteries, dominant collateral or pulmonary arteries, and chromosome 22q11.2 microdeletion. The sensitivity and specificity of the pulmonary flow study in predicting postoperative pulmonary haemodynamics were also tested.
Eight-year actuarial survival and freedom from reoperation were 85% and 63%, respectively. Sensitivity and specificity of the pulmonary flow study were 94% and 100%, respectively. None of the anatomical variables examined was significantly related to the outcome of treatment. The only statistically relevant association was detected between survival and occurrence of 22q11.2 microdeletion (p < 0.003). Logistic analysis showed an increased likelihood of positive outcome in relation to first- (p < 0.02) or second-stage (p < 0.04) complete correction.
Morphology of pulmonary blood supply has no major impact on surgical outcome. Pulmonary flow study is a highly specific and sensitive intraoperative test. Chromosome 22q11.2 microdeletion remains the only variable significantly affecting survival.
将患有肺动脉闭锁、室间隔缺损（VSD）及多条主肺动脉侧支血管的儿科患者的解剖学和遗传学特征与手术结果相关联。
44例年龄为33±40个月的连续患者，根据术前对肺血来源的形态学和功能评估，接受了一期单灶化手术（n = 32）或姑息性右心室流出道重建术（n = 12），随后进行二期单灶化和修复术（n = 10）。41%的病例发生了22q11.2染色体微缺失。一期手术中联合室间隔缺损封堵术由术中肺血流研究指导。35例（83%，95%可信区间72 - 95%）完成了完全修复。检查的变量包括心包内汇合的肺动脉、中央肺动脉、实质内汇合的肺动脉、主要侧支或肺动脉的情况，以及22q11.2染色体微缺失。还测试了肺血流研究在预测术后肺血流动力学方面的敏感性和特异性。
八年精算生存率和免于再次手术率分别为85%和63%。肺血流研究的敏感性和特异性分别为94%和100%。所检查的解剖学变量均与治疗结果无显著相关性。唯一具有统计学意义的关联是在生存与22q11.2微缺失的发生之间检测到的（p < 0.003）。逻辑分析显示，与一期（p < 0.02）或二期（p < 0.04）完全矫正相关的阳性结果可能性增加。
肺血供应形态对手术结果无重大影响。肺血流研究是一种高度特异和敏感的术中检查。22q11.2染色体微缺失仍然是唯一显著影响生存的变量。
