三尖瓣下移畸形二次手术治疗(附17例报告)

Reoperations on Ebstein's anomaly:Report for 17 Cases

目的总结三尖瓣下移畸形再次手术的治疗经验。方法回顾性分析17例三尖瓣下移畸形二次手术患者的临床资料,其中男6例,女11例,年龄3～54岁,平均23岁,所有患者均有劳累后气促、心悸等症状,其中活动后口唇青紫2例。第一次手术行三尖瓣悬吊并房化心室横向折叠14例,三尖瓣环缩同时修补房间隔缺损2例,三尖瓣机械瓣置换1例。再次手术时间距第一次手术时间1～20年,平均8年,术前心功能Ⅱ级6例,Ⅲ级11例。再次手术行解剖矫治8例,三尖瓣机械瓣替换7例,三尖瓣成形2例(其中交界环缩1例,人工腱索+三尖瓣环环缩1例),所有手术均在体外循环直视下进行,体外循环时间70～287(89.3±11.1)min,主动脉阻断时间24～111(64±8.6)min。术前及术后常规行食管超声检查。结果无住院死亡病例。术后随访1～3年,在解剖矫治及三尖瓣环环缩成形的10例患者中,三尖瓣无反流9例,轻度反流1例。所有患者心功能I～Ⅱ级。结论三尖瓣下移畸形需再次手术的患者,部分仍可施行三尖瓣成形术,如果三尖瓣病变严重,无法行解剖矫治,应选择三尖瓣替换手术。

Objective To summarize the experiences of reoperations on Ebstein＇s anomaly. Methods Clinical data of 17 cases of Ebstein＇s anomaly （6 males and 11 females, aged from 3 to 54 years old, averaged 23） were analyzed retrospectively. All the patients felt short breath and palpitation after exertion, and cyanosis was found in two patients. In the first operation, downwards displaced tricuspid valve leaflet was suspended and atrialized right ventricle was replicated in 14 cases, tricuspid anuloplasty and atrial septal defect were repaired in 2 cases, and tricuspid valve was replaced in 1 case. The interval time between two operations was 1-20 years in an average of 8 years. Before second operation, the cardiac function was NYHA Ⅱ in 6 cases, NYHA Ⅲ in 11 cases. Eight cases received Ebsteirfs anomaly anatomic correction, including transplanting downward displaced tricuspid valve and excising atrialized right ventricle. Seven eases received tricuspid valve replacement. Two cases received tricuspid valveplasty. All of operations were performed on arrested heart with moderate hypothermic cardiopulmonary bypass. The time of cardiopulmonary bypass was 70-287 （89.3±11.1）min, the time of aorta clamping was 70-287（64.0±8.6）min. The transesophageal echocardiography was routinely performed before the operations and after the hearts rebeating. Results No case died in hospital, and all the patients were followed up for 1-3 years. Transesophageal echocardiography showed the tricuspid valve was closed well （non-reflow in 9 cases, mild reflow in 1 case） in 10 cases which received Ebstein＇s anatomic correction and tricus-pid valveplasty. The cardiac function states of patients were significantly improved （NYHA Ⅰ-Ⅱ）. Conclusion Most of patients with reoperations on Ebstein＇s anomaly can received anatomic correction. Tricuspid valve replacement is available if the pathology of tricuspid valve is severe.