Ebaid M, Azeka E, Ikari N M, Sosa E A, Marcial M B, Atik E
Instituto do Coração do Hospital das Clínicas, FMUSP, Brazil.
Arq Bras Cardiol. 1999 Aug;73(2):219-24. doi: 10.1590/s0066-782x1999000800010.
Ebstein's anomaly with coarctation of the aorta is an extremely unusual condition. In this report, the clinical and surgical features of 3 male patients, aged 7 months, 4 years and 14 years, are discussed. All patients were in situs solitus. The first 2 patients had atrioventricular and ventriculoarterial discordance and progressed to heart failure in the neonatal period. The third had atrioventricular and ventriculoarterial concordance, as well as Wolf-Parkinson-White syndrome, with frequent episodes of paroxysmal tachycardia. The 3 patients underwent surgery for correction of the coarctation of the aorta. The patient with atrioventricular and ventriculoarterial concordance underwent tricuspid valvuloplasty using a DeVega-like technique. In addition, ablation of 2 anomalous pathways (Kent bundle), which were detected by the electrophysiologic study, was also subsequently performed. The 3 patients showed a good postoperative outcome for 2 years, although, in those with discordance, the surgical procedure did not influence the dysplasia of the tricuspid valve, because this valve showed light to moderate dysfunction.
埃布斯坦畸形合并主动脉缩窄是一种极为罕见的病症。在本报告中，讨论了3例男性患者（年龄分别为7个月、4岁和14岁）的临床及手术特征。所有患者均为正常位心。前2例患者存在房室和心室动脉不一致，并在新生儿期进展为心力衰竭。第3例患者存在房室和心室动脉一致，以及预激综合征，伴有频繁的阵发性心动过速发作。这3例患者均接受了主动脉缩窄矫正手术。房室和心室动脉一致的患者采用类似德维加技术进行了三尖瓣成形术。此外，还对电生理研究检测到的2条异常通路（肯特束）进行了消融。这3例患者术后2年预后良好，不过，在房室不一致的患者中，手术并未影响三尖瓣的发育异常，因为该瓣膜显示为轻度至中度功能障碍。
