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目的探讨儿童混合型完全性肺静脉异位连接(TAPVC)的诊治要点及影响预后的主要因素。方法近5 a来,在广东省心血管研究所确诊为混合型TAPVC的患儿共17例,收集该17例患儿的临床资料和随访结果,进行回顾性分析。结果 17例混合型TAPVC患儿中心上型+心内型14例,心上型+心下型3例,并肺静脉回流梗阻6例。17例术前全部行超声心动图检查,其中13例行多排螺旋CT扫描,14例行手术治疗。行CT扫描者,10例经手术探查证实此10例患儿CT能够准确显示所有肺静脉回流径路。行超声检查者,12例超声报告了所有4条肺静脉回流径路,5例超声未能报告肺静脉全部回流径路。超声报告所有肺静脉回流径路者,经CT或手术探查,证实其中7例超声显示的所有肺静脉回流径路均准确,4例超声准确显示了3条肺静脉回流径路,1例超声显示的肺静脉回流径路完全错误。并肺静脉回流梗阻的6例患儿,超声心动图发现6例,而CT仅发现1例。14例行手术治疗者,急诊手术6例,择期手术8例。围术期死亡2例,均为急诊手术者。12例康复出院者,随访时间2～53(28.8±17.4)个月,中位随访时间30个月。术后3例发生吻合口狭窄,其中1例术后2个月死于严重吻合口狭窄导致的急性肺水肿。结论混合型TAPVC以心上型+心内型最多见。结合超声心动图和多排螺旋CT扫描是术前评估的最佳策略。对肺静脉回流径路的显示多排螺旋CT优于超声心动图,对肺静脉回流梗阻的显示超声心动图优于多排螺旋CT。急诊手术可能是围术期死亡的危险因素。术后随访主要并发症为吻合口狭窄。
Objective To investigate the main points of diagnosis and treatment of children with mixed complete pulmonary venous connection (TAPVC) and prognosis. Methods In the past 5 years, a total of 17 children with mixed type TAPVC diagnosed by Guangdong Provincial Cardiovascular Institute were collected. The clinical data and follow-up results of the 17 children were collected and analyzed retrospectively. Results In 17 cases of mixed type TAPVC, there were 14 cases of supraocardiography and cardioembolism, 3 cases of cardia and hypocardial type, and 6 cases of pulmonary vein reflux obstruction. Seventeen patients underwent echocardiography preoperatively. Thirteen patients underwent multislice CT scan and 14 patients underwent surgical treatment. Line CT scan, 10 cases confirmed by surgical exploration of these 10 cases of children with CT can accurately show all pulmonary venous return path. In the ultrasound examination, all four pulmonary venous return routes were reported by ultrasound in 12 cases, and all of the pulmonary venous return paths were not reported in 5 cases. Ultrasound report of all pulmonary venous return pathways, by CT or surgical exploration, confirmed that 7 cases showed all the pulmonary vein pulmonary vein ultrasonography pathways are accurate, 4 cases of ultrasound shows exactly 3 pulmonary venous reflux path, 1 case of pulmonary venous drainage pathways showed completely wrong . 6 cases of obstructive pulmonary venous return obstruction, 6 cases were found by echocardiography, and 1 case was found by CT. 14 cases of surgical treatment, emergency surgery in 6 cases, elective surgery in 8 cases. Perioperative death in 2 cases, both emergency surgery. Twelve cases of discharged patients were followed up for 2 ~ 53 months (28.8 ± 17.4 months) with a median follow - up time of 30 months. Anastomotic stenosis occurred in 3 cases, of which 1 died of acute pulmonary edema caused by severe anastomotic stricture 2 months after operation. Conclusion Mixed TAPVC is the most common form of heart-type + intracardiac. Combination of echocardiography and multi-slice spiral CT scan is the best strategy for preoperative evaluation. Multi-slice spiral CT is superior to echocardiography in displaying pulmonary venous return pathways and superior to multislice spiral CT in demonstrating pulmonary venous return obstruction. Emergency surgery may be a risk factor for perioperative death. Postoperative follow-up of the main complications of anastomotic stenosis.