Chávez Mariana, Zubair M Mujeeb, Staffa Steven J, Emani Sitaram M, Quinonez Luis G, Kaza Aditya, Hoganson David M, Baird Christopher W
Cardiac Surgery Department, Boston Children's Hospital, Harvard Medical School, Boston, Mass.
Cardiac Surgery Department, Cleveland Clinic, Cleveland, Ohio.
J Thorac Cardiovasc Surg. 2025 Jul;170(1):25-33. doi: 10.1016/j.jtcvs.2025.02.025. Epub 2025 Mar 5.
The impact of early age on outcomes for repair of complete atrioventricular canal defects (CAVCs) remains poorly defined. We evaluated young infants with CAVC, comparing those who underwent primary repair versus primary pulmonary artery banding (PAB) and results related to left atrioventricular valve (AVV) reintervention and survival.
Patients (age <60 days) with CAVC were evaluated (January 2005 to April 2022) at a single institution. Patients were categorized as having primary CAVC repair or PAB. Patients with complex unbalanced CAVC and severely hypoplastic ventricles and those not undergoing CAVC repair after PAB were excluded. Outcome measures included total number of operations, reoperation on the left AVV, hospital length of stay, and mortality.
CAVC was identified in 135 patients, mean age 33 ± 19 days and weight 3.4 ± 0.7 kg at primary operation. Additional diagnosis included transposition of the great arteries (n = 4), tetralogy of Fallot (n = 9), transposition of the great arteries (n = 13), and total and partial anomalous pulmonary venous return (n = 7). Thirty-three patients required preoperative respiratory support. Primary CAVC repair was performed in 101 patients at 38 ± 16.6 days and 3.5 ± 0.7 kg, and primary PAB was performed in 34 patients at 16 ± 15 days and 3.2 ± 0.7 kg, of whom 62% (n = 21) underwent subsequent CAVC repair at 6.9 ± 4.7 months and 6.6 ± 2.3 kg. When we compared patients undergoing primary CAVC versus PAB; 55% versus 48% had preoperative mild and 39% versus 29% mild-moderate or greater atrioventricular valve regurgitation (AVVR). In patients who underwent CAVC repair, a 2-patch repair was used in 66% of cases and posterior left AVV annuloplasty in 34%. Predischarge reoperation for left AVVR was required in 13% (n = 14/101) patients whereas in patients who underwent PAB, it was required in 14% (n = 3/21). Hospital length of stay was shorter for primary CAVC (25 vs 41 days). Overall, median follow-up was 4.5 years. Patients undergoing primary CAVC had fewer total number of operations (1.3 vs 2.5, P < .001) and fewer reoperations on the left AVV (18% vs 24%, P = .56). Overall, freedom from reoperation in primary CAVC for left AVVR at 1 and 5 years was 85% and 82% compared with patients who underwent PAB (89% and 69%). At follow-up, 88% of patients undergoing primary CAVC repair had mild or less left AVVR, whereas 82% undergoing initial PAB had mild or less left AVVR. There were 10 deaths; overall mortality was 6% in patients who underwent primary CAVC and 19% in patients who underwent PAB. Similarly, follow-up rates of significant AVVR and mortality did not differ significantly between groups (P > .05).
Definitive CAVC repair at ≤60 days can be performed with acceptable midterm survival. Primary CAVC repair versus primary PAB for young patients undergoing CAVC has a trend toward fewer total operations, fewer reoperations for AVVR, decreased hospital LOS, and less mortality. However, reoperation rates for AVVR and mortality were not statistically different, and pacemaker implantation occurred in 10% of patients who underwent primary repair. These results underscore the need for cautious interpretation, given the limitations of statistical power. Reoperation for left AVVR remains a challenge and occurs early after repair. Evolving surgical techniques to avoid postoperative left AVV dysfunction should further reduce early postoperative morbidity and hospital resource use.
早期年龄对完全性房室通道缺损(CAVC)修复结果的影响仍不明确。我们评估了患有CAVC的幼儿,比较了接受一期修复与一期肺动脉环扎术(PAB)的患儿,以及与左房室瓣(AVV)再次干预和生存相关的结果。
对一家机构(2005年1月至2022年4月)收治的年龄<60天的CAVC患者进行评估。患者被分为接受一期CAVC修复或PAB。排除患有复杂不平衡CAVC和严重发育不良心室的患者以及PAB后未接受CAVC修复的患者。观察指标包括手术总数、左AVV再次手术、住院时间和死亡率。
共识别出135例CAVC患者,初次手术时平均年龄33±19天,体重3.4±0.7kg。其他诊断包括大动脉转位(n = 4)、法洛四联症(n = 9)、大动脉转位(n = 13)以及完全性和部分性肺静脉异位引流(n = 7)。33例患者术前需要呼吸支持。101例患者在38±16.6天、体重3.5±0.7kg时接受了一期CAVC修复,34例患者在16±15天、体重3.2±0.7kg时接受了一期PAB,其中62%(n = 21)的患者在6.9±4.7个月、体重6.6±2.3kg时接受了后续CAVC修复。比较接受一期CAVC与PAB的患者;55%对48%术前有轻度房室瓣反流(AVVR),39%对29%有轻度至中度或更严重的AVVR。在接受CAVC修复的患者中,66%的病例采用了双补片修复,34%采用了左AVV后瓣环成形术。13%(n = 14/101)的CAVC修复患者出院前因左AVVR需要再次手术,而接受PAB的患者中,这一比例为14%(n = 3/21)。一期CAVC修复患者的住院时间较短(25天对41天)。总体而言,中位随访时间为4.5年。接受一期CAVC修复的患者手术总数较少(1.3次对2.5次,P <.001),左AVV再次手术较少(18%对24%,P =.56)。总体而言,一期CAVC修复患者1年和5年时左AVVR无需再次手术的比例分别为85%和82%,而接受PAB的患者为89%和69%。随访时,接受一期CAVC修复的患者中88%有轻度或更轻的左AVVR,而接受初始PAB的患者中82%有轻度或更轻的左AVVR。共有10例死亡;接受一期CAVC修复的患者总体死亡率为6%,接受PAB的患者为19%。同样,两组之间显著AVVR的随访率和死亡率无显著差异(P >.05)。
在≤60天时进行确定性CAVC修复可获得可接受的中期生存率。对于接受CAVC修复的年轻患者,一期CAVC修复与一期PAB相比,手术总数有减少趋势,AVVR再次手术减少,住院时间缩短,死亡率降低。然而,AVVR再次手术率和死亡率无统计学差异,10%接受一期修复的患者需要植入起搏器。鉴于统计效能的局限性,这些结果强调需要谨慎解读。左AVVR再次手术仍然是一个挑战,且在修复后早期发生。不断发展手术技术以避免术后左AVV功能障碍应进一步降低术后早期发病率和医院资源使用。