关键词: 心脏缺损, 先天性, Ebstein畸形, 三尖瓣闭锁不全, 儿童, Cone重建

Abstract: Abstract: Objective To summarize the cone reconstruction of Ebstein’ s anomaly in children. Methods The clinical data of 12 consecutive children (under 14 years old) underwent cone reconstruction in our hospital from August 2013 to October 2017 were retrospectively analyzed. There were 4 males and 8 females, aged (6.8 ± 4.2) years, with body mass (24.1±11.8) kg, were included in this cohort. According to Carpentier’ s classification, 1, 7 and 4 patients were classified as A, B and C types, respectively. The combined deformities included 8 atrial septal defect, 2 patent ductus arteriosus, 1 pulmonary stenosis and 1 mitral regurgitation. There were 3 patients with moderate tricuspid regurgitations and 9 patients with severe tricuspid regurgitations. There were 4 patients with cyanosis. According to NYHA classification, 4, 7 and 1 patients were classified as Ⅱ, Ⅲ and Ⅳdegree respectively. Results No death occurred in this cohort. Among these patients, 11 received successful cone reconstruction and 1 underwent tricuspid valve replacement due to failure of valvuloplasty. There were 2 patients received distal fenestrations of the cone, 2 received valvular augmentations by autologous pericardium, 2 received artificial chordae tendineaes and 2 received bidirectional Glenn shunts respectively. During (24.4±17.0)-month-follow up, no death or reoperation occurred. Among the 11 patients who successfully underwent cone reconstruction, there were 2 cases of tricuspid regurgitation (no regurgitation) and 2 cases of micro-regurgitation, 3 cases of mild-moderate regurgitation, 1 case of moderate regurgitation. And there were 10 cases of grade Ⅰ and 1 case of grade Ⅱ cardiac function. The tricuspid regurgitation and cardiac function were significantly improved after operation compared with those before operation (P＜0.05). Conclusion The proper application of targeting surgical techniques based on individualized characteristics is the key point of Cone reconstruction.

Key words: heart defects, congenital, ebstein anomaly, tricuspid valve insufficiency, child, cone reconstruction