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儿童三尖瓣下移畸形141例外科治疗结果分析

The experience of surgical treatment for Ebstein anomaly in 141 children

摘要:

目的 探讨儿童三尖瓣下移畸形的外科治疗策略.方法 回顾性分析2003年1月至2015年12月在上海交通大学医学院附属上海儿童医学中心心胸外科接受外科治疗的141例三尖瓣下移畸形患者的资料.男性65例,女性76例;年龄(6.9±1.6)岁(范围:10个月至15岁),体重(19.6±4.7)kg(范围:6.5~59.0 kg).三尖瓣隔瓣、后瓣下移1.0~5.0 cm,三尖瓣反流轻度26例、中度46例、重度69例.治疗策略分为双心室功能矫治77例,一个半心室功能矫治56例和单心室功能矫治8例,主要包括瓣膜锥形重建94例,瓣膜提升37例,瓣膜置换2例,闭合三尖瓣8例.结果 术后早期双心室功能组因循环波动,死亡1例;改为一个半心室功能矫治3例,术后存活.因完全性房室传导阻滞安置心脏永久起搏器3例.出院140例(三尖瓣反流轻度118例,中度14例,三尖瓣闭合8例).137例获得随访,随访时间18~172个月.瓣膜锥形重建91例中,三尖瓣反流轻度75例,中度15例,重度1例;瓣膜提升36例中,三尖瓣反流轻度21例,中度12例,重度3例.对4例三尖瓣重度反流者再次手术锥形重建3例,术后轻度反流;另1例因前瓣发育差,瓣膜无法成形,闭合三尖瓣,从一个半心室功能矫治改为单心室功能矫治,血氧饱和度上升.机械瓣置换2例中,因瓣膜失功死亡1例,拆除1例(闭合三尖瓣,改为单心室功能矫治,术后活动耐量好).结论 三尖瓣下移接受瓣膜锥形重建能获得良好的成形效果,采用一个半心室功能的手术策略可有效维持三尖瓣下移畸形术后早期血流动力学的稳定性.对三尖瓣严重发育不良导致的三尖瓣重度反流和低氧血症患者,术后如出现循环功能障碍,改为单心室功能矫治可能是唯一的选择.

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abstracts:

Objective To explore the surgical strategy for Ebstein anomaly in children.Methods From January 2003 to December 2015,a total of 141 cases of Ebstein anomaly were treated at Department of Pediatric Cardiothoracic Surgery,Shanghai Children's Medical Center,School of Medicine,Shanghai Jiaotong University.There were 65 male and 76 female patients,with age of (6.9± 1.6) years (ranging from 10 months to 15 years),weight of (19.6±4.7) kg (ranging from 6.5 to 59.0 kg).All patients were diagnosed by 2 dimensional Doppler echocardiography and the septal leaflet and posterior leaflet displaced downward from 1.0 to 5.0 cm.The tricuspid valve regurgitation (TR) were mild in 26 cases,moderate in 46 cases and severe in 69 cases.Tricuspid valvuloplasty were performed in 131 cases (94 cone reconstruction,37 valve hoist),tricuspid valve replacement in 2 cases and tricuspid valve closed in 8 cases.Surgical strategy were divided into biventricular heart function in 77 cases,one and a half ventricular heart function in 56 cases,and single ventricular heart function in 8 cases.Results Three patients were changed to one and a half ventricular repair from biventricular repair due to unstable hemodynamics in the early postoperative period.One case died in biventricular group.The complete atrioventricular block were occurred in 3 patients and paceruaker were applied.One hundred and forty cases discharged from hospital.There were mild TR in 118 cases,moderate in 14 cases and closed in 8 cases.One hundred and thirty-seven cases were followed up regularly in 18 to 172 months.Ninety-one cases were treated by cone reconstruction (mild TR in 75 cases,moderate in 15 cases and severe in 1 case).Thirty-six cases were operated by tricuspid valve hoist (mild TR in 21 cases,moderate in 12 cases and severe in 3 cases).In the patients with severe TR (4 cases),3 cases were reoperated by cone reconstruction.One case's valve was closed because of the dysplasia of the anterior valve and then from one and a half ventricular heart function to single ventricular function,the oxygen saturation was increased.Two patients underwent tricuspid valve replacement,1 died and the other's mechanical valve was removed,and changed to single ventricular function repair.Conclusions Although tricuspid cone reconstruction can achieve good results,the stable hemodynamic of early postoperative can be effectively maintained by using the surgical strategy of one and a half ventricular repair.To the patients with severe tricuspid regurgitation and hypoxemia due to severe tricuspid valve dysplasia,transforming to a functional single ventricle may be the only choice when there comes to the unstable hemodynamic.

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