心下型完全性肺静脉异位引流的急诊手术和早中期随访结果

Infracardiac total anomalous pulmonary venous connection: Urgent operation and early to mid-term follow-up results

目的回顾完全性肺静脉异位引流(TAPVC)心下型的急诊手术经验和早中期随访结果.方法2000年9月至2003年6月,手术证实的4例心下型TAPVC,平均年龄27 d.肺静脉回流的部位包括:门静脉2例,下腔静脉1例,另1例不详.3例临床上有肺静脉回流梗阻表现,所有患儿均行急诊手术.结果无手术死亡.1例术后合并肺炎,长时间呼吸机支持,术后19 d自动出院.其余3例均痊愈出院.3例患儿得到随访,随访时间14个月～3年.2例恢复良好,心功能Ⅰ级;1例术后4个月起出现肺静脉梗阻,仅作保守治疗但效果不理想,术后3年失访.结论心下型TAPVC病情危重,急症手术可挽救生命,手术早期效果良好.术后肺静脉梗阻一般内科治疗无效,应积极手术干预.

Objective To report our surgical experience and early to mid-term follow-up results of infracardiac total anomalous pulmonary venous connection (TAPVC).Methods Four patients with infracardiac TAPVC undergoing surgical repair in this institute between Sept. 2000 and June 2003 were reviewed. Postoperative follow-up was made in three patients.Results Mean age at operation was 27 days (range 9-35 days). Mean body weight was 3.7 kg (range 2.9- 4.7kg). Sites of drainage to the infra-diaphragm veins included portal vein in 2 cases and inferior vena cava in 1. The other one was unclear. Clinical pulmonary venous obstruction (PVO) was present in 3 cases. All patients underwent urgent operation. Mean bypass time was 134 minutes (range 76-169 minutes) and mean aortic cross-clamp time was 54 minutes (range 36-74 minutes). There was no early operative mortality. Delayed sternum closure was made in 2 cases. Inhalation of nitric oxide was given to 2 cases for the significant postoperative pulmonary hypertension, while peritoneal dialysis was performed in 2 cases with temporary renal failure. The mean ventilation time was 4.5 days (range 2-18 days). Mean time of ICU monitoring and hospital stay was 7 days (range 4-18 days) and 11.5 days (range 8-18 days), respectively. The postoperative follow-up was available in three patients ranging from 14 months to 3 years. Two patients recovered well and were in NYHA functional class Ⅰ. One patient presented with PVO since four months after the primary repair, who made no improvement in spite of medical treatment and was lost follow-up three years after operation.Conclusions Corrective operation for infracardiac TAPVC on urgent basis is life saving and has gratifying early results. Mid-term postoperative course can be complicated by recurrent PVO. Early, aggressive reintervention for recurrent PVO should be considered.