Vargas F J, Mengo G, Granja M A, Gentile J A, Rannzini M E, Vazquez J C
Unit of Pediatric Cardiovascular Surgery, Hospital Italiano, Buenos Aires, Argentina.
Ann Thorac Surg. 1998 Jun;65(6):1755-7. doi: 10.1016/s0003-4975(98)00290-2.
Seven patients with the diagnosis of Ebstein's malformation of the tricuspid valve were operated on. Mean age was 12 years (range, 7 to 16 years). All were cyanotic, with severe tricuspid regurgitation. Thromboembolism was not present. No associated cardiac malformations were present.
Surgical repair included tricuspid annuloplasty associated with longitudinal plication of the atrialized portion of the right ventricle. This was attained by approximating the anterior-posterior commissure with either the posterior-septal commissure or the septal leaflet remnant. The thin atrialized ventricular wall thus excluded remained as a cul du sac and was plicated by suturing along the longitudinal axis of the heart. When present, the dysplastic posterior leaflet was included in the plication. In essence, a monocuspid right atrioventricular valve was fashioned out of the anterior leaflet. The remaining septal leaflet played a minimal functional role. No additional procedures for treatment of arrhythmia were associated with the technique described.
The postoperative course was uneventful in all patients. Mean follow-up is 4.3 years (range, 1 to 10 years). Doppler echocardiographic studies reveal satisfactory monocusp valve function in all patients, with adequate coaptation of the anterior leaflet and the septal structures.
This technique seems applicable to most forms of Ebstein's malformation and is reproducible. The technique relies on the adequate mobilization of the anterior leaflet. Occasionally it is necessary to free fibrous adhesions of the leaflet to the underlying ventricular surface.
对7例诊断为三尖瓣埃布斯坦畸形的患者进行了手术。平均年龄为12岁(范围7至16岁)。所有患者均有发绀,伴有严重的三尖瓣反流。不存在血栓栓塞。无相关心脏畸形。
手术修复包括三尖瓣环成形术,同时对右心室心房化部分进行纵向折叠。这是通过使前后交界与后间隔交界或间隔瓣叶残余部分靠近来实现的。这样留下的薄心房化心室壁形成一个盲端,并沿心脏纵轴缝合折叠。如有发育异常的后瓣叶,则将其纳入折叠。本质上,由前瓣叶形成一个单尖瓣右房室瓣。其余的间隔瓣叶发挥的功能作用极小。该技术未涉及治疗心律失常的额外操作。
所有患者术后病程平稳。平均随访4.3年(范围1至10年)。多普勒超声心动图研究显示所有患者的单尖瓣功能良好,前瓣叶与间隔结构贴合良好。
该技术似乎适用于大多数形式的埃布斯坦畸形,且可重复。该技术依赖于前瓣叶的充分游离。偶尔需要松解瓣叶与下方心室表面的纤维粘连。