Hancock Friesen Camille L, Chen Robert, Howlett Jonathan G, Ross David B
Division of Cardiac Surgery, Halifax, Nova Scotia, Canada.
Ann Thorac Surg. 2004 Jun;77(6):2167-71. doi: 10.1016/j.athoracsur.2003.11.025.
Nonneonatal Ebstein's anomaly is a rare congenital anomaly for which numerous operative techniques have been described to repair the aberrant tricuspid valve, exclude the atrialized right ventricle, and address right heart dysfunction. We reviewed short-term outcomes in surgical patients treated by a simplified operative technique.
Our operative approach to Ebstein's anomaly is approximation of the anteroposterior commissure to the remnant septal leaflet with closure of the cul-de-sac longitudinally. Plication of the atrialized right ventricle (1 patient) and a bidirectional cavopulmonary connection (2 patients) were performed only if necessary. All patients were followed postoperatively by their cardiac surgeon and cardiologist.
Seven patients with a mean age of 39 years (range, 3.6 to 63.8 years) underwent repair. Preoperatively all patients had 4+ tricuspid valve regurgitation and were New York Heart Association class III. Mean postoperative hospital stay was 7 +/- 2 days (range, 4 to 11 days). Mean follow-up is 42 +/- 18 months (range, 7 to 58 months). At last follow-up 5 patients are New York Heart Association class I and average tricuspid valve regurgitation is mild.
Plication of the posterior annulus without plication of the atrialized right ventricle, resiting the tricuspid valve, or performing prophylactic cavopulmonary connection appears to be a reasonable operative approach to nonneonatal Ebstein's anomaly. Long-term follow-up of this cohort is necessary to determine the durability of such a surgical approach.
非新生儿期的埃布斯坦畸形是一种罕见的先天性畸形,针对这种畸形已经描述了多种手术技术来修复异常的三尖瓣、排除房化右心室并解决右心功能障碍。我们回顾了采用简化手术技术治疗的手术患者的短期结局。
我们治疗埃布斯坦畸形的手术方法是将前后瓣叶交界与残留的隔叶对合,并纵向闭合盲端。仅在必要时才对房化右心室进行折叠术(1例患者)和双向腔肺连接术(2例患者)。所有患者术后均由心脏外科医生和心脏病专家进行随访。
7例平均年龄为39岁(范围为3.6至63.8岁)的患者接受了修复手术。术前所有患者均有4级以上三尖瓣反流,且均为纽约心脏协会心功能Ⅲ级。术后平均住院时间为7±2天(范围为4至11天)。平均随访时间为42±18个月(范围为7至58个月)。在最后一次随访时,5例患者为纽约心脏协会心功能Ⅰ级,平均三尖瓣反流为轻度。
不折叠房化右心室、重新定位三尖瓣或进行预防性腔肺连接而仅折叠后瓣环,似乎是治疗非新生儿期埃布斯坦畸形的一种合理手术方法。需要对该队列进行长期随访以确定这种手术方法的持久性。