Division of Pediatric Cardiothoracic Surgery, University of Tennessee Health Sciences Center/Le Bonheur Children's Hospital, Memphis, Tenn, USA.
J Thorac Cardiovasc Surg. 2011 May;141(5):1163-9. doi: 10.1016/j.jtcvs.2011.01.029. Epub 2011 Feb 25.
The purpose of this study was to review the long-term outcome of patients with Ebstein anomaly who underwent complete repair as neonates and young infants.
Between March 1994 and May 2010, 32 patients (23 neonates and 9 young infants) underwent surgery for Ebstein anomaly. Mean weight was 3.9 ± 2.0 kg (range, 1.9-8.6 kg). The Great Ormond Street Echocardiography score was greater than 1.5 in 22 of the 23 neonates and greater than 1.0 in all infants. All associated cardiac defects were repaired including pulmonary atresia in 15 and ventricular septal defect in 4. Primary outcome measures included (1) early and late survival, (2) freedom from reoperation, (3) durability of tricuspid valve repair, and (4) functional status.
Early survival was 78.1% (25/32). There was 1 late death. Fifteen-year survival estimate was 74% ± 8%. For patients with pulmonary atresia, early and late survival was 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively; for those without pulmonary atresia, early and late survival was 60% ± 12% versus 85% ± 10 (P = .06), respectively. The mean follow-up was 5.9 ± 4.5 years (0.1-16 years). A biventricular repair was achieved in 90.6% (29/32) patients. Median preoperative tricuspid regurgitation was 4/4 and at late follow-up, 1/4. Freedom from reoperation at 15 years was 74% ± 10% for patients undergoing biventricular repair. All survivors are in New York Heart Association class I or II.
Biventricular repair of Ebstein anomaly in symptomatic neonates is feasible with good early and late survival, especially in those without pulmonary atresia. Tricuspid valve repair is durable, and functional status is excellent.
本研究旨在回顾完全修复新生儿和婴儿期埃布斯坦畸形患者的长期结果。
1994 年 3 月至 2010 年 5 月期间，32 例患者（23 例新生儿和 9 例婴儿）接受了埃布斯坦畸形手术。平均体重为 3.9 ± 2.0 公斤（范围 1.9-8.6 公斤）。23 例新生儿中有 22 例大奥蒙德街超声心动图评分大于 1.5，所有婴儿评分均大于 1.0。所有相关的心脏缺陷均得到修复，包括 15 例肺动脉瓣闭锁和 4 例室间隔缺损。主要结果测量包括（1）早期和晚期存活率，（2）免于再次手术，（3）三尖瓣修复的耐久性，以及（4）功能状态。
早期存活率为 78.1%（25/32）。有 1 例晚期死亡。15 年生存率估计为 74% ± 8%。对于患有肺动脉瓣闭锁的患者，早期和晚期存活率分别为 60% ± 12%（9/15）和 94.1%（16/17）（P<.05）；对于没有肺动脉瓣闭锁的患者，早期和晚期存活率分别为 60% ± 12%和 85% ± 10（P=0.06）。平均随访时间为 5.9 ± 4.5 年（0.1-16 年）。90.6%（29/32）的患者实现了双心室修复。中位术前三尖瓣反流为 4/4，在晚期随访时为 1/4。行双心室修复的患者 15 年免于再次手术的生存率为 74% ± 10%。所有幸存者均为纽约心脏协会心功能 I 级或 II 级。
对于有症状的新生儿，可行的埃布斯坦畸形的双心室修复具有良好的早期和晚期存活率，尤其是在没有肺动脉瓣闭锁的患者中。三尖瓣修复是持久的，功能状态是极好的。
