目的:左心室流出道梗阻(LVOTO)是主动脉弓中断(IAA)婴儿发病和死亡的主要原因。LVOT发育可能是流动介导的,因此,IAA形态可能会影响LVOT直径和随后的再干预。我们研究了IAA形态(主动脉弓异常的类型和存在)与LVOT直径和再干预的关系。
方法:在单一机构对所有IAA(2001-2022)手术患者进行了回顾。我们比较了IAA-A与IAA-B;IAA与主动脉弓异常(AAb)与无;IAA-B与异常锁骨下动脉(AAbS)与其他。主要结果包括LVOT直径(mm),排放时的LVOTO(≥50mmHg),以及LVOT的再干预。
结果:77名婴儿(平均年龄10±19天)随访7.6(5.5-9.7)年。围手术期死亡率为3.9%(3/77),长期死亡率为5.2%(4/77)。在51例IAA-B(66%)和22例IAA-A(31%)患者中,30%(n=22)具有AAb。较小的LVOT直径与IAA-B相关[IAA-A:5.40(4.68-5.80),IAA-B:4.60(3.92-5.50),p=0.007],AAb[AAb:4.00(3.70-5.04)vs无:5.15(4.30-5.68)p=0.006],合并IAA-B+AAbS[IAA-B+AAbS:4.00(3.70-5.02)vs其他:5.00(4.30-5.68),p=0.002]。在AAb中,LVOTO的可能性更高(N=6(25%)vsN=1(2%),p=0.004)和IAA-B+AAbS(N=1(2%)vsN=6(30%),p=0.002)。事件发生时间分析显示IAA-BAAbS中LVOT再干预增加的信号(p=0.11)。
结论:IAA-B和AAb与小的LVOT直径和早期的LVOTO有关,尤其是组合。这可能反映了发育过程中近端牙弓的流量较低。大多数再干预发生在IAA-B+AAbS,因此,这些患者应在初次修复时仔细考虑进行LVOT干预.
OBJECTIVE: Left ventricular outflow tract obstruction (LVOTO) is a major cause of morbidity and mortality in infants with interrupted aortic arch (IAA). Left Ventricular Outflow Tract (LVOT) development may be flow-mediated, thus IAA morphology may influence LVOT diameter and subsequent reintervention. We investigated the association of IAA morphology [type and presence of aortic arch aberrancy (AAb)] with LVOT diameter and reintervention.
METHODS: All surgical patients with IAA (2001-2022) were reviewed at a single institution. We compared IAA-A versus IAA-B; IAA with aortic AAb versus none; IAA-B with aberrant subclavian (AAbS) artery versus others. Primary outcomes included LVOT diameter (mm), LVOTO at discharge (≥50 mmHg), and LVOT reintervention.
RESULTS: Seventy-seven infants (mean age 10 ± 19 days) were followed for 7.6 (5.5-9.7) years. Perioperative mortality was 3.9% (3/77) and long-term mortality was 5.2% (4/77). Out of 51 IAA-B (66%) and 22 IAA-A (31%) patients, 30% (n = 22) had AAb. Smaller LVOT diameter was associated with IAA-B [IAA-A: 5.40 (4.68-5.80), IAA-B: 4.60 (3.92-5.50), P = 0.007], AAb [AAb: 4.00 (3.70-5.04) versus none: 5.15 (4.30-5.68), P = 0.006], and combined IAA-B + AAbS [IAA-B + AAbS: 4.00 (3.70-5.02) versus other: 5.00 (4.30-5.68), P = 0.002]. The likelihood of LVOTO was higher among AAb [N = 6 (25%) vs N = 1 (2%), P = 0.004] and IAA-B + AAbS [N = 1 (2%) vs N = 6 (30%), P = 0.002]. Time-to-event analysis showed a signal towards increased LVOT reintervention in IAA-B + AAbS (P = 0.11).
CONCLUSIONS: IAA-B and AAb are associated with small LVOT diameter and early LVOTO, especially in combination. This may reflect lower flow in the proximal arch during development. Most reinterventions occur in IAA-B + AAbS, hence these patients should be carefully considered for LVOT intervention at the time of initial repair.