Disease characteristics and surgical effect of 22 children with Shone′s syndrome
10.3760/cma.j.cn101070-20201225-01952
- VernacularTitle:儿童Shone综合征22例疾病特点与手术效果分析
- Author:
Yuefeng CAO
1
;
Lei LI
;
Junwu SU
;
Xiangming FAN
Author Information
1. 首都医科大学附属北京安贞医院小儿心脏中心，北京 100029
- Keywords:
Shone′s syndrome;
Supravalar mitral ring;
Parachute mitral valve;
Left ventricular outflow tract obstruction
- From:
Chinese Journal of Applied Clinical Pediatrics
2022;37(6):443-445
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the characteristics and surgical outcomes of Shone′s syndrome in children, and to explore the surgical treatment strategy and technical key.Methods:Retrospective study.Children with Shone′s syndrome treated in the Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University from May 2013 to June 2019 were retrospectively analyzed for their baseline characteristics and surgical data.The data were compared by Student t-test. Results:A total of 22 children with Shone′s syndrome were recruited, involving 15 males (68.2%) and 7 females (31.8%). There were 6 cases (27.3%) of complete form of Shone′s syndrome and 16 cases (72.7%) of incomplete form.No deaths were reported.The postoperative mitral valve velocity [(149.7±38.2) cm/s vs.(234.9±34.0) cm/s, t=7.341, P<0.05], left ventricular outflow tract velocity [(202.0±105.0) cm/s vs.(328.6±120.3) cm/s, t=6.575, P<0.05] and aortic arch coarctation velocity [(186.1±60.9) cm/s vs.(347.9±100.8) cm/s, t=7.630, P<0.05]were significantly lower than those of preoperative levels.There were no complications occurred at 1-year follow-up, and 91.7% of the patients were followed up for 3 years, and 80.2% were followed up for 5 years without complications, 2 cases needed reoperation. Conclusions:Surgical treatment of Shone′s syndrome achieved satisfactory outcomes.Early diagnosis and early intervention are beneficial to children with Shone′s syndrome, although they need to be followed up and have the risk of reoperation in the long term.
