Huang Shu-Chien, Wu En-Ting, Chen Shyh-Jye, Huang Chi-Hsiang, Shih Jin-Chung, Chou Hen-Wen, Chang Chung-I, Chiu Ing-Sh, Chen Yih-Sharng
Department of Surgery, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan.
Department of Pediatrics, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan.
Ann Thorac Surg. 2017 Sep;104(3):917-925. doi: 10.1016/j.athoracsur.2017.01.081. Epub 2017 Apr 21.
Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair.
Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan. The principle of cone reconstruction was applied with mobilization of all three leaflets and reattachment to the normal tricuspid annulus. The atrialized right ventricle was not plicated. In patients with pulmonary stenosis, the interatrial communication was not totally closed (n = 5), and a systemic-pulmonary shunt was added if needed (n = 3).
All patients presented with intractable heart failure or severe cyanosis requiring mechanical ventilation, or both. All patients had marked adherence of the anterior leaflet to the right ventricular free wall. Intracardiac anomalies including ventricular septal defect (n = 2) and tetralogy of Fallot (n = 1) were also repaired simultaneously. Six of the 7 patients (86%) survived. There were no late deaths or repeat TVPs for a median follow-up of 4.3 years (range, 0.8 to 9.9 years).
Reconstruction of the tricuspid valve is an acceptable surgical strategy in patients with severe neonatal Ebstein anomaly. Fenestrated atrial septal defect and systemic-pulmonary shunt can help overcome anatomic pulmonary stenosis and high pulmonary resistance in the neonatal period. This surgical strategy has a good survival outcome and preserves the possibility of complete biventricular repair.
患有严重埃布斯坦畸形的新生儿面临手术挑战，强烈主张采用斯塔恩斯手术作为单心室姑息治疗。尽管新生儿三尖瓣成形术（TVP）的病例报道极少，但锥形重建用于TVP已成为广泛接受的技术。本报告描述了一种针对新生儿埃布斯坦畸形的手术策略，旨在实现双心室修复。
自2007年以来，7例患有严重埃布斯坦畸形的新生儿或幼儿在台湾台北国立台湾大学医院接受了TVP。应用锥形重建原则，将所有三个瓣叶游离并重新附着于正常三尖瓣环。未对房化右心室进行折叠。对于合并肺动脉狭窄的患者，房间隔交通未完全关闭（n = 5），必要时增加体肺分流（n = 3）。
所有患者均表现为顽固性心力衰竭或严重紫绀，需要机械通气，或两者兼具。所有患者的前瓣叶均与右心室游离壁明显粘连。同时还修复了包括室间隔缺损（n = 2）和法洛四联症（n = 1）在内的心脏内异常。7例患者中有6例（86%）存活。中位随访4.3年（范围0.8至9.9年），无晚期死亡或再次进行TVP的情况。
三尖瓣重建是治疗严重新生儿埃布斯坦畸形患者可接受的手术策略。开窗房间隔缺损和体肺分流有助于克服新生儿期的解剖性肺动脉狭窄和高肺阻力。这种手术策略具有良好的生存结局，并保留了完全双心室修复的可能性。
