Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia.
Royal Children's Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
J Thorac Cardiovasc Surg. 2013 Apr;145(4):1033-1039. doi: 10.1016/j.jtcvs.2012.05.017. Epub 2012 Jun 13.
Pulmonary artery (PA) sling is a rare vascular anomaly associated with congenital tracheal stenosis. The natural history is poor and these patients often require early surgical intervention. We describe our experience with repair of this condition.
From 1984 to 2011, 21 patients with PA sling underwent repair at the Royal Children's Hospital (median age, 5.9 months). PA sling was associated with compression of the trachea in all patients. Tracheal surgery was required in 12 (57.1%) patients. All patients had an echocardiogram, and concomitant repair of coexisting cardiac anomalies was performed in 6 (28.6%, 6/21) patients.
Operative mortality was 14.3% (3/21), occurring at 19 days, 4.4 months, and 5 months after surgery. Operative mortality for the first 10 years was 22.2% (1984-1993; 2/9), the next 10 years was 14.3% (1994-2003; 1/7), and 0% for the most recent 7 years (2004-2011; 0/5). All deaths occurred in patients requiring tracheal repair (25%, 3/12). No deaths have occurred since 2004 with introduction of the slide tracheoplasty technique. One (5.6%, 1/18) late death occurred at 8 months after repair. After tracheal repair, intervention for excessive granulations and tracheomalacia was necessary in 6 (50%, 6/12) patients. Median follow-up was 8 years (mean, 8.6 ± 6.4 years; range, 5 months to 20.6 years), and all survivors (100%, 17/17) remain asymptomatic.
Children with PA sling who do not require tracheal surgery have excellent outcomes. Mortality is determined by the need for tracheal surgery. However, with the advent of the slide tracheoplasty technique, mortality can be reduced. Survival beyond 1 year after surgery offers excellent prognosis.
肺动脉吊带是一种罕见的血管异常，与先天性气管狭窄有关。其自然病史较差，这些患者通常需要早期手术干预。我们描述了我们在修复这种情况方面的经验。
1984 年至 2011 年，21 例肺动脉吊带患者在皇家儿童医院接受修复（中位年龄 5.9 个月）。所有患者的肺动脉吊带均伴有气管受压。12 例（57.1%）患者需要行气管手术。所有患者均行超声心动图检查，6 例（28.6%，6/21）患者同时行并存心脏畸形修复。
手术死亡率为 14.3%（3/21），分别发生在术后 19 天、4.4 个月和 5 个月。第 1 个 10 年的手术死亡率为 22.2%（1984-1993 年；2/9），第 2 个 10 年为 14.3%（1994-2003 年；1/7），最近 7 年为 0%（2004-2011 年；0/5）。所有死亡均发生在需要气管修复的患者（25%，3/12）。自 2004 年采用滑动气管成形术技术以来，未再发生死亡。修复后 8 个月，1 例（5.6%，1/18）患者死亡。气管修复后，6 例（50%，6/12）患者需要干预治疗过度肉芽组织和气管软化。中位随访时间为 8 年（平均 8.6±6.4 年；范围 5 个月至 20.6 年），所有存活者（100%，17/17）均无症状。
无需气管手术的肺动脉吊带患儿预后良好。死亡率取决于气管手术的需要。然而，随着滑动气管成形术技术的出现，死亡率可以降低。手术后 1 年以上存活者预后极佳。
