17例心下型完全性肺静脉异位引流的外科治疗

Surgical treatment for infra-cardiac total anomalous pulmonary venous drainage and early to mid follow up results In 17 cases

目的 评估新生儿、小婴儿心下型完全性肺静脉异位引流的纠治方法及效果.方法 2000年7月至2009年6月,共纠治心下型完全性肺静脉异位引流17例.病婴出生15天～3.7月龄,其中1月龄以下9例,占52.9%;体重（4.10±0.95）kg,其中5 kg以下15例,占全组88.2%.4例低温低流量灌注下,经右心房切口,切开房间隔,左房与肺静脉吻合;13例深低温停循环灌注,采用心脏上翻法,左心房斜形切口与肺静脉无张力缝合,其中吻合口宽度保证在2.5～4.0 cm.结果 全组手术顺利,无手术死亡.6例术后延迟关胸,5例发生肺高压危象,2例出现暂时性Ⅲ度房室传导阻滞,2例急性肾功能衰竭,4例肺水肿,所有病婴均治愈出院.1例术后反复出现肺部感染,心脏超声发现吻合口流速明显增快（3.05 m/s）、严重肺动脉高压、三尖瓣重度反流,1年后失随访.16例随访1个月～9年,平均（51.8±35.0）个月.随访期均行心脏超声、X线胸片、心电图检查.多数病婴心功能良好,心影较术前明显缩小,肺充血消失,心电图示窦性节律,吻合口血流速度正常.结论 新生儿及婴幼儿心下型完全性肺静脉异位引流病情危重,早期诊断、早期手术效果良好,手术治疗的关键在于左心房与肺静脉吻合口应足够大,避免狭窄的发生.

Objective To evaluate the effectiveness of urgent surgical correction for infra-cardiac total anomalous pulmonary venous drainage （TAPVD） in infants and children. Methods From July 2000 to April 2009, seventeen patients with infra-cardiac type of total anomalous pulmonary venous connection received surgical correction in our department. The age of patients ranged from 15 days to 3.7 months at admission, 9 patients （ 52.9 % ） were younger than 1 month of age. The mean body weight was （4.10 ±0.95） kg and was less than 5 kg in 15 patients （88.2%）. Deep hypothermic circulatory arrest （DHCA） without cerebral perfusion was used in 13 patients （76%）, and continuous hypothermic low-flow perfusion was used in4 （24%）. Incisions on the left atrium and pulmonary veins were sutured without tension, and the width of the anastomosis was 2.5 to 4.0 cm. Results Urgent operations were performed in all patients and no early death occurred during surgery.Mean bypass time was 121.9 minutes （ ranging from 70 to 277 minutes） and mean aortic cross-clamp time was 44 minutes （ ranging from 30 to 74 minutes）. 6 neonates had delayed wound closure after surgery and 4 had obvious pulmonary edema. Inhalation of nitric oxide was given to 5 cases for the management of significant postoperative pulmonary hypertension, while peritoneal dialysis was performed in 2 cases with temporary renal failure. Transient atrioventricular block of grade 3 occurred in 2patients and acute renal failure occurred in 2 cases. All patients were uneventfully discharged. The mean ventilation time was 45 h （ranging from 12 to 430 h）. Mean duration in ICUs and hospitals were 15 days （ ranging from 4 to 40 days） and 22. 5days （ ranging from 10 to 42 days）, respectively. During the period of follow up, one patient presented with pulmonary venous obstruction and recurrent pulmonary infection with increased flow speed at the site of anastomosis, pulmonary hypertension and tricuspid regurgitation on echocardiogram. No improvement was observed in the patient spite of medical treatment and he was lost during follow up one year after operation. 16 patients received postoperative follow up for 1 month to 9 years, mean （51.8 ± 35.0） months. Echocardiography, chest radiography, and ECG were performed during this period. As a result, most children had good cardiac function, with sinus rhythm on ECG and apparently reduced pulmonary congestion on radiography.No obstructive pulmonary venous return was observed on echocardiography. Most patients had good cardiac functions. Conclusion Corrective operation for infra-cardiac TAPVC on urgent basis may provide favorite outcomes. The prognosis is associated with the size of anastomosis between the pulmonary vein and left atrium, as large anastomosis may prevent the obstruction of pulmonary venous retum.