Surgical treatment for congenital supravalvular aortic stenosis in children with Doty procedure
10.3760/cma.j.issn.2095-428X.2014.18.017
- VernacularTitle:Doty法矫治小儿先天性主动脉瓣上狭窄的疗效
- Author:
Feng YUAN
;
Song BAI
;
Jian GUO
;
Xiaofeng LI
- Publication Type:Journal Article
- Keywords:
Congenital heart disease;
Congenital supravalvular aortic stenosis;
Surgical treatment
- From:
Chinese Journal of Applied Clinical Pediatrics
2014;29(18):1421-1424
- CountryChina
- Language:Chinese
-
Abstract:
Objective To summarize the clinical effect of Doty procedure on surgical treatment for patients with congenital supravalvular aortic stenosis (SVAS).Methods Between Jan.2009 and Jun.2013,12 cases of consecutive patients underwent surgical corrections of SVAS using Doty procedure.Among them,Williams' syndrome were present in 5 patients.Nine cases were male,and 3 cases were female.Mean age was(5.3 ±4.2) years(9 months to 12 years) and mean weight was (11.5 ± 5.3) kg (7.5-32.0 kg).Echocardiography and Computed Tomography confirmed the diagnosis.SVAS was discrete in 9 patients and diffuse in 3 patients.Mean peak preoperative gradient was 6.86-16.23 (11.07± 3.68) kPa,with 3 patients above 13.33 kPa.Bicuspid aortic valve was present in 2 patients,mild aortic valve stenosis in 1 patient,mild aortic regurgitation in 2 patients,1 patient with subaortic membrane,3 patients with stenosis of the left or the right pulmonary artery,1 patient with pulmonary valve stenosis,2 patients with PDA,moderate to severe mitral insufficiency in 1 patients.Surgery was performed using Doty procedure through median sternotomy with cardiopulmonary bypass.Results Mean time of cardiopulmonary bypass was (85 ± 26)min (68-129 min).Mean time of aortic clamp was (51 ± 16) min(43-68 min).There was no early death and low cardiac output syndrome.Mild ST segment change was present in 5 patients and occasional premature beat was in 3 patients.Ventilator was applied for 7 to 38 h and Cardiac Intensive Care Unit treatment was done for 1 to 4 d.The mean peak supravalvular gradient measured postoperatively was (2.18 ± 1.09) kPa.Follow-up data were available for 11 patients,ranged from 6 months to 3 years.The gradients across supravalvular were lower than 3.33 kPa,except for 2 patients with severe SVAS preoperatively.There was no late death,dizzy or palpitation during follow-up.Conclusion Doty produce for SVAS can get satisfactory clinical results.