Michielon Guido, Di Carlo Duccio, Brancaccio Gianluca, Guccione Paolo, Mazzera Ennio, Toscano Alessandra, Di Donato Roberto Michele
Department of Medico-Chirurgico di Cardiochirurgia e, DMCCP, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
Ann Thorac Surg. 2003 Aug;76(2):581-8; discussion 588. doi: 10.1016/s0003-4975(03)00344-8.
This study investigates the correlation between surgical timing and 15-year longitudinal left ventricular and mitral valve function, after repair of anomalous coronary artery origin from the pulmonary artery.
Between 1987 and 2002, 31 patients (median age, 7.1 months) underwent repair for anomalous origin of the left (n = 28), right (n = 2), or both (n = 1) coronary arteries from the pulmonary artery. Repair was accomplished by subclavian interposition in 5 patients, intrapulmonary tunnel in 12, and direct aortic reimplantation in 14. Primary mitral valve repair was never associated with coronary revascularization. Total follow-up was 186.4 patient-years (mean, 77.2 months).
Fifteen-year actuarial survival was 92.9% +/- 4.9% for coronary transfer, 40.0% +/- 21.9% for subclavian interposition, and 89.9% +/- 7.5% for intrapulmonary tunnel (p = 0.019). Five patients required further intervention for supravalvular pulmonary stenosis (n = 3), baffle leak (n = 1), and mitral valve replacement (n = 1). Coronary transfer allowed best freedom from long-term reoperation (92.3% +/- 7.4%). Left ventricular shortening fraction increased from 17.3% +/- 6.3% before operation to 34.1% +/- 4.6% at last follow-up (p < 0.01). Regression analysis demonstrated a linear relationship between age at repair and shortening fraction recovery (r(2) = 0.573, p < 0.01). Patients younger than 6 months of age showed worse preoperative shortening fraction (15.9% +/- 5.2%) and best longitudinal shortening fraction recovery (36.4% +/- 5.1%; p < 0.001). Major improvement in mitral valve function was observed within 1 year from surgery in 90.4% of survivors.
Repair of anomalous coronary artery origin from the pulmonary artery in younger symptomatic infants offers the best potential for recovery of left ventricular function, despite a worse initial presentation. Coronary transfer is associated with superior long-term survival and freedom from reoperation. Most patients with patent two-coronary repair will recover normal mitral valve function; therefore, simultaneous mitral valve surgery seems unwarranted.
本研究调查了肺动脉起源异常的冠状动脉修复术后手术时机与15年纵向左心室和二尖瓣功能之间的相关性。
1987年至2002年间，31例患者（中位年龄7.1个月）接受了左冠状动脉（n = 28）、右冠状动脉（n = 2）或双侧冠状动脉（n = 1）起源于肺动脉的修复手术。5例患者采用锁骨下动脉移植术进行修复，12例采用肺内隧道术，14例采用直接主动脉再植入术。初次二尖瓣修复从未与冠状动脉血运重建相关。总随访时间为186.4患者年（平均77.2个月）。
冠状动脉移植术的15年精算生存率为92.9%±4.9%，锁骨下动脉移植术为40.0%±21.9%，肺内隧道术为89.9%±7.5%（p = 0.019）。5例患者因瓣上肺动脉狭窄（n = 3）、挡板漏血（n = 1）和二尖瓣置换（n = 1）需要进一步干预。冠状动脉移植术使长期再次手术的自由度最高（92.3%±7.4%）。左心室缩短分数从术前的17.3%±6.3%增加到最后随访时的34.1%±4.6%（p < 0.01）。回归分析显示修复时年龄与缩短分数恢复之间存在线性关系（r² = 0.573，p < 0.01）。6个月以下的患者术前缩短分数较差（15.9%±5.2%），纵向缩短分数恢复最佳（36.4%±5.1%；p < 0.001）。90.4%的幸存者在术后1年内二尖瓣功能有显著改善。
对于有症状的小婴儿，肺动脉起源异常的冠状动脉修复术为左心室功能恢复提供了最佳潜力，尽管初始表现较差。冠状动脉移植术与较高的长期生存率和再次手术自由度相关。大多数双冠状动脉修复通畅的患者将恢复正常的二尖瓣功能；因此，同期二尖瓣手术似乎没有必要。
