经导管封堵全腔静脉肺动脉连接术外管道开窗的初步临床应用

Effects of transcatheter closure of extracardiac Fontan fenestration by Amplatzer duct or septal ocduder in three patients

目的探讨经导管封堵全腔静脉肺动脉连接术外管道开窗的方法及疗效。方法2007年3至7月共行3例外管道开窗封堵术，男2例，女1例，年龄分别为7、14及8岁，术前诊断均为发绀，属复杂先天性心脏病，不能行双心室矫治，而行全腔静脉肺动脉连接术，术中均加行外管道开窗。术后患者持续存在口唇发绀，血氧饱和度降低。3例患者行心导管及造影检查后证实上、下腔静脉与肺动脉吻合口通畅，无狭窄改变，肺动脉平均压均小于15mmHg（1mmHg=0．133kPa），分别采用AGA公司Amplatzer动脉导管未闭封堵器及房间隔缺损封堵器对外管道与右房之问的交通（开窗）进行封堵。结果所有患者均封堵成功，封堵后即刻、15min、30min及1h分别测肺动脉及腔静脉压，与封堵前比较无明显升高，且患者无明确不适症状，血氧饱和度较封堵术前均有提高，发绀减轻，超声心动图及造影证实封堵器形态及位置良好。无术中及术后并发症，术后随访3至6个月，经超声心动图证实封堵器形态及位置良好，无残余分流。结论经导管外管道开窗封堵术技术成功率高、疗效确切，但远期疗效尚需密切随访。

Objective The purpose of this study was to report our experiences from the transcatheter closure of patent fenestration after total eavopulmonary connection（TCPC） with an extra cardiac conduit. Methods Three patients （7,14 and 8 years old） with various forms of functionally univentricular heart lesions received a total cavopulmonary connection with an extra cardiac conduit as a final reconstructive procedure. Transcatheter occlusion of the fenestration was accomplished using a 8/6 mm Amplatzer duct occluder in one patient, and 5 mm or 10 mm Amplatzer septal occluder in the other two patients. Residual shunting following occlusion was assessed using angiography and echocardiography. Results Post total cavopulmonary connection with an extra cardiac conduit, diagnostic catheterization revealed normal pressures in the superior vena cava and pulmonary artery without obstruction at the site of the anastomosis. Angiography of the extra cardiac conduit confirmed the communication between the conduit and the atrium in all three patients and patients still suffered from cyanosis and low oxygen saturation. Immediate full occlusion of fenestration was obtained in all patients. Post closure, mean central venous pressure returned to normal accompanied with significantly increased oxygen saturation. Cyanosis was also significantly attenuated. There were no procedural complications or device failures at intra-hospital and during the 3 to 6 months follow up period. Conclusions The Amplatzer septal or duct occluder device is a safe and effective strategy for the Fontan fenestration occlusion.