Konstantinov Igor E, Karamlou Tara, Williams William G, Quaegebeur Jan M, del Nido Pedro J, Spray Thomas L, Caldarone Christopher A, Blackstone Eugene H, McCrindle Brian W
Hospital for Sick Children, Toronto, Canada.
J Thorac Cardiovasc Surg. 2006 May;131(5):1136-1141.e2. doi: 10.1016/j.jtcvs.2005.03.051.
The objective was to determine outcomes and risk factors of surgical management of patients with aortopulmonary window associated with interrupted aortic arch.
From 1987 to 1997, 472 neonates with interrupted aortic arch were enrolled prospectively from 33 institutions. Associated aortopulmonary window was present in 20 patients. Competing risk methodology determined the prevalence of reintervention for postrepair pulmonary artery and aortic arch obstruction.
Interrupted aortic arch was type A in 17 patients and type B in 3 patients. Aortopulmonary window morphology was type I (n = 10), type II (n = 5), and type III (n = 5). Associated cardiovascular anomalies were common, including atrial septal defect (n = 13) and systemic venous anomalies (n = 3). Overall survival after initial admission was 91%, 86%, and 84% at 1, 5, and 10 years, respectively. Fifteen patients underwent single-stage repair, and 4 patients underwent staged repair. There was an increased prevalence of patch augmentation of the interrupted aortic arch anastomosis in lower-weight infants (2.3 kg vs 3.1 kg, P = .07). Competing risk analysis estimated that 5 years after repair, 51% had initial arch reintervention, 6% had initial pulmonary artery reintervention, and 43% were alive without reintervention. Reintervention for arch obstruction was more likely for those with interrupted aortic arch type B (P = .08) and for those with higher weight at initial repair (P = .003).
Complete correction of aortopulmonary window in the setting of interrupted aortic arch can be performed with low mortality in the neonatal period. Reinterventions for aortic arch obstruction are the most frequent complication after repair, but pulmonary artery stenosis also occurs. Use of patch augmentation may reduce the need for subsequent arch reintervention.
本研究旨在确定合并主动脉弓中断的主肺动脉窗患者手术治疗的结果及危险因素。
1987年至1997年，前瞻性纳入了来自33家机构的472例主动脉弓中断的新生儿。其中20例患者合并主肺动脉窗。采用竞争风险方法确定修复后肺动脉和主动脉弓梗阻再次干预的发生率。
17例患者的主动脉弓中断为A型，3例为B型。主肺动脉窗形态为I型（n = 10）、II型（n = 5）和III型（n = 5）。常见合并心血管畸形，包括房间隔缺损（n = 13）和体静脉畸形（n = 3）。初次入院后的总体生存率在1年、5年和10年分别为91%、86%和84%。15例患者接受了一期修复，4例患者接受了分期修复。体重较轻的婴儿（2.3 kg vs 3.1 kg，P = 0.07）主动脉弓中断吻合口补片扩大的发生率较高。竞争风险分析估计，修复后5年，51%的患者接受了初次主动脉弓再次干预，6%的患者接受了初次肺动脉再次干预，43%的患者存活且未接受再次干预。B型主动脉弓中断患者（P = 0.08）和初次修复时体重较高的患者（P = 0.003）更有可能因主动脉弓梗阻接受再次干预。
在新生儿期，合并主动脉弓中断的主肺动脉窗可实现低死亡率的完全矫正。主动脉弓梗阻再次干预是修复后最常见的并发症，但也会发生肺动脉狭窄。使用补片扩大可能会减少后续主动脉弓再次干预的需求。
