经导管同期治疗复合型先天性心脏病的疗效观察

Simultaneous transcatheter therapy of combined congenital heart disease

目的研究经导管同期治疗复合型先天性心脏病的策略并评价其疗效。方法34例患者,男12例,女22例。复合类型为:房间隔缺损(ASD)合并肺动脉瓣狭窄(PS)、室间隔缺损(VSD)、动脉导管未闭(PDA)分别是16例、7例、3例,合并二尖瓣狭窄(Lutembacher综合征)2例;VSD合并PDA3例、Taussig-Bing畸形合并PDA1例;2例并发3种畸形(并发ASD、VSD和PDA1例,并发ASD、PS和PDA1例)。经导管介入治疗的原则:先行瓣膜球囊扩张术纠正瓣膜狭窄,其次行VSD封堵术,再次行PDA封堵术,最后行ASD封堵术。术后3d、1个月、6个月分别行经胸超声心动图(TTE)、X线检查评价治疗效果。结果34例复合型先天性心脏病患者均一次性治疗成功。术中未发生任何重要并发症。术后即刻TTE和造影示ASD,VSD,PDA所有封堵器位置良好,无残余分流。16例ASD合并PS者,跨肺动脉瓣平均压差由术前52.6±20.5mmHg下降到术后14.3±9.5mmHg(1mmHg=0.133kPa),差异有统计学意义(P<0.05)。2例Lutembacher综合征患者,超声心动图测量二尖瓣瓣口面积分别由二尖瓣球囊扩张术前1.0和1.2cm2增加到术后1.9和2.0cm2,平均左房压分别由29和26mmHg降至8和7mmHg。对于1例Taussig-Bing畸形合并PDA者,用18mmASD伞成功封堵16mmPDA,降低肺动脉压,改善患者症状,为二次手术创造条件。术后72h、1个月、6个月TTE示所有患者各水平分流均消失,左心房、左心室进行性地缩小,所有封堵器位置固定良好,无移位及脱落;同时行X线检查,肺血均明显减少,房室内径均明显恢复。结论复合型先天性心脏病经导管同期介入治疗虽较单纯型复杂,技术要求相对较高,但如指征掌握恰当,治疗策略合理,操作规范,同样可获得良好的治疗效果。

Objective To investigate the algorithm and efficacy of interventional treatment for combined congenital heart diseases. Methods Thirty-four patients (12 males and 22 females) with combined congenital heart disease underwent simultaneous transcatheter therapy. The types of the congenital heart defect combination were as follow: ASD and PS (16 cases); ASD and VSD (7 cases); ASD and PDA (3 cases); ASD and MS (2 cases); VSD and PDA (3 cases); Taussig-Bing and PDA(1 case); PDA, ASD and PS (1 case); ASD, VSD and PDA (1 case). The algorithm of transcatheter intervention was: PBPV or PBMV at first followed by the occlusion of VSD, PDA and ASD successively. Transthoracic echocardiography (TTE) and X-ray examination were done 3d, 1 m and 3 m after the procedure to evaluate the outcome of the procedure. Results All patients were treated successfully at one time. No complications occurred during and immediately after the procedure. There was no residual shunt and all the occlusion devices were found in the suitable site evaluated by means of TTE and X-ray right after the procedure. In 16 patients with ASD and PS, the systolic pressure across the pulmonary valve decreased from (53±20) mm?Hg to (14±10) mm?Hg (P<0.05). For 2 cases of Lutembacher syndromes, mitral valve area increased from 1.0, 1.2 to 1.9, 2.0 cm^2 and the mean left atrial prissure decreased from 29, 26 mm?Hg to 8, 7 mm?Hg. Successful occlusion of the patient with Tassig-Bing and PDA with 18 mm ASD device diminished the pulmonary pressure, improved the patient's symptoms and provided a desiable condition for the following operation. During the 72 h～6 months follow-up, no residual shunt and dislocation of the devices were found by TTE. At the same time X-ray examination showed that pulmonary vascularity and the enlargement of heart were diminished significantly. Conclusion Compared with simple congenital heart disease, transcatheter intervention for combined defects is more difficult. However, a satisfactory result can be obtained from suitable indications, proper algorithm of procedures and standardized manipulations.