Michielon Guido, Di Donato Roberto M, Pasquini Luciano, Giannico Salvatore, Brancaccio Gianluca, Mazzera Ennio, Squitieri Cosimo, Catena Glauco
Dipartimento Medico Chirurgico di Cardiologia Pediatrica, DMCCP, Ospedale Pediatrico Bambino Gesù, P.zza S.Onofrio 4, 00165 Rome, Italy.
Eur J Cardiothorac Surg. 2002 Aug;22(2):184-91. doi: 10.1016/s1010-7940(02)00247-6.
To evaluate late outcome of non-isomeric total anomalous pulmonary venous connection (TAPVC) repair, controlling for anatomic subtypes and surgical technique.
Between 1983 and 2001, 89 patients (median age 54 days) underwent repair for supracardiac (38), cardiac (26), infracardiac (16) or mixed (nine) TAPVC. Ten patients (11.2%) presented associated anomalies other than PDA. Twenty-eight patients (31.5%) were emergencies, due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included the double-patch technique with left atrial enlargement in 29 patients and side-to-side anastomosis between the pulmonary venous (PV) confluence and the left atrium in 29 patients. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Total follow-up was 727.16 patient-years (mean 8.55 years, 98.8% complete).
Early mortality was 7.86% (7/89). Ten patients (11.2%) underwent reintervention, including reoperation (eight), balloon dilation (one) and intraoperative stents placement (one), for anastomotic (four) or diffuse PV stenosis (six), with four late deaths. Kaplan-Meier survival is 87.3+/-0.036 SE% at 18.07 years with no difference according to anatomic type or surgical technique. Freedom from PV reintervention for operative survivors is 86.7+/-0.052 SE% at 18.07 years. Cox proportional hazard indicates associated anomalies (P=0.008) and reoperation for intrinsic PV stenosis (P=0.034) as independent predictors of mortality. According to logistic analysis, preoperative obstruction predicts higher risk of reintervention for intrinsic PV stenosis (P=0.022), while the double-patch technique increased the risk of late arrhythmias (P=0.005).
Side-to-side anastomosis provides excellent results for TAPVC repair while left atrial enlargement procedures appear to be associated with higher risk of late arrhythmias. Although early and aggressive reintervention for recurrent PV obstruction is mandatory, intrinsic PV stenosis remains a predictor of adverse outcome.
评估非异构型完全性肺静脉异位连接（TAPVC）修复术的远期疗效，同时考虑解剖亚型和手术技术的影响。
1983年至2001年间，89例患者（中位年龄54天）接受了心上型（38例）、心内型（26例）、心下型（16例）或混合型（9例）TAPVC修复术。10例患者（11.2%）存在除动脉导管未闭（PDA）以外的相关畸形。28例患者（31.5%）因引流受阻为急诊手术。心上型和心下型TAPVC修复术，29例采用双补片技术并扩大左心房，29例采用肺静脉（PV）汇合处与左心房侧侧吻合术。心内型TAPVC修复术首选冠状静脉窦开窗术。总随访时间为727.16患者年（平均8.55年，完成率98.8%）。
早期死亡率为7.86%（7/89）。10例患者（11.2%）接受了再次干预，包括再次手术（8例）、球囊扩张（1例）和术中置入支架（1例），原因是吻合口狭窄（4例）或弥漫性PV狭窄（6例），其中4例晚期死亡。18.07年时，Kaplan-Meier生存率为87.3±0.036 SE%，不同解剖类型或手术技术之间无差异。手术存活者在18.07年时无PV再次干预的自由度为86.7±0.052 SE%。Cox比例风险模型显示，相关畸形（P = 0.008）和因PV固有狭窄进行再次手术（P = 0.034）是死亡率的独立预测因素。根据逻辑分析，术前梗阻预示着PV固有狭窄再次干预的风险更高（P = 0.022），而双补片技术增加了晚期心律失常的风险（P = 0.005）。
侧侧吻合术在TAPVC修复术中效果良好，而扩大左心房的手术似乎与较高的晚期心律失常风险相关。尽管对于复发性PV梗阻必须尽早积极进行再次干预，但PV固有狭窄仍是不良预后的预测因素。
