Boger A J, Baak R, Lee P C, Boersma E, Meijboom F J, Witsenburg M
Department of Cardiothoracic Surgery, Thoraxcenter, University Hospital, Erasmus Medical Center, Rotterdam, The Netherlands.
Eur J Cardiothorac Surg. 1999 Sep;16(3):296-9. doi: 10.1016/s1010-7940(99)00236-5.
An ever increasing number of patients with an operated congenital cardiac anomaly is reaching adulthood. Insight in the long-term characteristics of these patients is steadily growing for the more common anomalies, but is still scarce for the less frequent entities. In this regard we report our results and long-term follow-up of surgically treated total anomalous pulmonary venous return (TAPVR).
Since 1973 44 patients with TAPVR were treated with surgery. There were 23 girls and 21 boys. Hospital mortality concerned six patients and additionally one patient died 2.5 months after surgery. Follow-up data could be collected from all the surviving patients at last outpatient visit or by phone contact. The mean duration of the follow-up was 12 years (range 1 month to 24 years).
The Kaplan-Meier estimate of survival at 15 years was 84% (95% CI 73-95%). Reoperations were performed for five patients at 7 days to 4.5 years after surgery. In one patient this involved obstruction of pulmonary venous return, in four complications of the primary repair. The reoperation-free survival at 15 years was 74% (95% CI 61-87%). At the end of follow-up all survivors were in NYHA functional class I and, at appropriate age, normally attended school or were employed. None of the patients was using cardiac medication.
We conclude that, despite a possible hectic initial postoperative course of surgery for TAPVR, only a limited number of reoperations is necessary relatively short after initial surgery. The long-term perspective for those surviving surgical treatment is good.
越来越多接受先天性心脏畸形手术的患者已步入成年期。对于较常见的心脏畸形，我们对这些患者长期特征的认识在不断增加，但对于较少见的类型仍知之甚少。在此，我们报告手术治疗完全性肺静脉异位引流（TAPVR）的结果及长期随访情况。
自1973年以来，44例TAPVR患者接受了手术治疗。其中女孩23例，男孩21例。住院期间死亡6例，另有1例患者在术后2.5个月死亡。通过最后一次门诊就诊或电话联系收集所有存活患者的随访数据。随访的平均时长为12年（范围1个月至24年）。
15年时的Kaplan-Meier生存率估计为84%（95%可信区间73%-95%）。5例患者在术后7天至4.5年进行了再次手术。其中1例是肺静脉回流梗阻，4例是初次修复的并发症。15年时无再次手术的生存率为74%（95%可信区间为61%-87%）。随访结束时，所有存活患者的心功能均为纽约心脏协会（NYHA）I级，且在适龄时正常上学或就业。无一例患者使用心脏药物。
我们得出结论，尽管TAPVR手术后初期病程可能较为复杂，但初次手术后相对较短时间内仅需进行有限次数的再次手术。手术治疗存活者的长期前景良好。
