Servicio de Cardiología.
Servicio de Cirugía Cardiovascular. Hospital Nacional de Pediatría Prof. Dr. Juan P. Garrahan, Ciudad Autónoma de Buenos Aires, Argentina.
Arch Cardiol Mex. 2024;94(4):403-411. doi: 10.24875/ACM.23000203.
Ebstein's anomaly is a congenital defect characterized by a lack of delamination and apical displacement of the tricuspid valve, tricuspid insufficiency, right atrial enlargement, and ventricular dysfunction. To analyze the results and evolution of the different surgical strategies, data were collected from 45 patients operated on during 1990-2018. Twenty-six patients were included with a median age at initial surgery of 11.3 years (range: 13 days-18.6 years).
Procedures were plastic 10 patients (38%), cone technique reconstruction 11 (42%), and replacement 5 (19%). Additional interventions were required in 7 patients: cavo-pulmonary anastomosis 4 and Cox-maze 4. Nine patients (34.6%) required reoperation due to severe tricuspid insufficiency. Six had previous plastic, two bioprothesis, and one, cone.
Overall mortality was 11.5% (3) at a median of 10.1 years post-surgery (range: 5.7-10.12) associated with arrhythmias (p = 0.05), right (p = 0.008), left (p = 0.0001) ventricular dysfunction and reoperations (p = 0.03). None were previous conus. Median follow-up was 6.5 years (range: 1-29.1). Ninety-one-point-six percent were in functional class I/II and 79.2% in sinus rhythm.
The results of the different classic techniques were similar, although not free of complications and reoperations. Cone reconstruction proved to be effective, with low surgical mortality, less need for reoperations, and durability in the medium term.
Ebstein 畸形是一种先天性缺陷，其特征为三尖瓣瓣叶缺如和 apical 移位、三尖瓣关闭不全、右心房扩大和心室功能障碍。为分析不同手术策略的结果和演变，收集了 1990 年至 2018 年间接受手术的 45 例患者的数据。26 例患者纳入研究，初始手术时的中位年龄为 11.3 岁（范围：13 天至 18.6 岁）。
手术方式包括 10 例患者的成形术（38%）、11 例患者的圆锥技术重建术（42%）和 5 例患者的置换术（19%）。7 例患者需要附加干预：4 例腔肺吻合术和 4 例 Cox 迷宫术。9 例患者（34.6%）因严重三尖瓣关闭不全需要再次手术。其中 6 例之前接受过成形术，2 例接受过生物假体，1 例接受过圆锥技术重建术。
总的死亡率为 11.5%（3 例），中位随访时间为术后 10.1 年（范围：5.7-10.12 年），与心律失常相关（p = 0.05），与右心室（p = 0.008）、左心室（p = 0.0001）功能障碍和再次手术相关（p = 0.03）。无患者之前接受过圆锥技术重建术。中位随访时间为 6.5 年（范围：1-29.1 年）。91.6%的患者心功能处于 I/II 级，79.2%的患者处于窦性心律。
不同经典技术的结果相似，但并非没有并发症和再次手术。圆锥技术重建术效果确切，手术死亡率低，再次手术需求少，中期效果持久。
