Griselli Massimo, McGuirk Simon P, Winlaw David S, Stümper Oliver, de Giovanni Joseph V, Miller Paul, Dhillon Rami, Wright John G, Barron David J, Brawn William J
Department of Cardiac Surgery, Diana, Princess of Wales Children's Hospital, Birmingham, United Kingdom.
J Thorac Cardiovasc Surg. 2004 Jan;127(1):251-8. doi: 10.1016/j.jtcvs.2003.08.052.
Congenital heart defects with major aortopulmonary collateral arteries show marked variability in the size and distribution of native pulmonary arteries. We sought to classify the size and distribution of native pulmonary arteries and to determine their influence on surgical outcome.
Between 1989 and 2002, 164 patients underwent surgical intervention for congenital heart defects with major aortopulmonary collateral arteries (median age, 10 months). Three patterns of native pulmonary arteries were identified: intrapericardial native pulmonary arteries present (group I); confluent intrapulmonary native pulmonary arteries without intrapericardial native pulmonary arteries (group II); and nonconfluent intrapulmonary native pulmonary arteries (group III). Thirty-seven (23%) patients had single-stage and 76 (47%) patients had multistage complete repair. Thirty (18%) patients await septation, and 8 (5.0%) patients are not septatable. Follow-up is 98% complete (median follow-up, 5.8 years).
In the 164 patients there were 15 (9.1%) early and 12 (7.3%) late deaths. Early mortality after complete repair was 4.4% (n = 5). Actuarial survival was 90% +/- 3% and 85% +/- 4% at 1 and 10 years, respectively. Actuarial freedom from surgical or catheter reintervention in septated patients was 77% +/- 4% and 45% +/- 8% at 1 and 10 years, respectively. On multivariate analysis, the morphology of the native pulmonary arteries was the only factor that influenced actuarial survival after complete repair (P =.04). Group III had the highest risk of death after septation (P =.008). Group II fared better than group III after the initial operation (P <.05).
Current classifications of congenital heart defects with major aortopulmonary collateral arteries are based on the presence or absence of intrapericardial pulmonary arteries. We have identified a subgroup without intrapericardial native pulmonary arteries but with confluent intrapulmonary native pulmonary arteries. This group has a better outcome than those with nonconfluent intrapulmonary native pulmonary arteries.
伴有主要主-肺动脉侧支动脉的先天性心脏缺陷在天然肺动脉的大小和分布上表现出显著差异。我们试图对天然肺动脉的大小和分布进行分类，并确定它们对手术结果的影响。
1989年至2002年间，164例患有伴有主要主-肺动脉侧支动脉的先天性心脏缺陷患者接受了手术干预（中位年龄10个月）。确定了三种天然肺动脉模式：存在心包内天然肺动脉（I组）；肺内汇合的天然肺动脉但无心包内天然肺动脉（II组）；以及肺内不汇合的天然肺动脉（III组）。37例（23%）患者接受了一期手术，76例（47%）患者接受了多期完全修复。30例（18%）患者等待分隔，8例（5.0%）患者无法进行分隔。随访完成率为98%（中位随访时间5.8年）。
在164例患者中，有15例（9.1%）早期死亡和12例（7.3%）晚期死亡。完全修复后的早期死亡率为4.4%（n = 5）。1年和10年时的精算生存率分别为90%±3%和85%±4%。分隔患者1年和10年时免于手术或导管再次干预的精算自由度分别为77%±4%和45%±8%。多因素分析显示，天然肺动脉的形态是影响完全修复后精算生存率的唯一因素（P = 0.04）。III组在分隔后死亡风险最高（P = 0.008）。II组在初次手术后的情况优于III组（P < 0.05）。
目前伴有主要主-肺动脉侧支动脉的先天性心脏缺陷的分类是基于心包内肺动脉的有无。我们确定了一个无心包内天然肺动脉但肺内天然肺动脉汇合的亚组。该组的结果优于肺内天然肺动脉不汇合的组。
