Treatment and effect of cardiovascular surgery in patients with Williams syndrome
- VernacularTitle:Williams 综合征患者的心血管手术治疗及效果
- Author:
Hujun CUI
1
,
2
;
Jimei CHEN
1
,
2
;
Yueheng WU
2
,
3
;
Yifan LI
4
,
5
;
Kan ZHOU
1
,
2
;
Jianzheng CEN
1
,
2
;
Gang XU
1
,
2
;
Shusheng WEN
1
,
2
;
Jian ZHUANG
1
,
2
Author Information
1. Department of Cardiac Surgery, Guangdong Provincial People&rsquo
2. s Hospital, Guangdong Academy of Medical Sciences, Guangdong Cardiovascular Disease Institute, Guangzhou, 510000, P.R.China
3. Prenatal Diagnosis Center, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong Provincial People&rsquo
4. Department of Pediatric Cardiology, Guangdong Provincial People&rsquo
5. s Hospital, Guangdong Academy of Medical Sciences, Guangdong
- Publication Type:Journal Article
- Keywords:
Williams syndrome;
supravalvular aortic stenosis;
cardiac surgical procedure
- From:
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery
2021;28(07):811-814
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the effects of cardiovascular surgery on Williams syndrome (WS). Methods The clinical data of 68 WS patients undergoing cardiovascular surgery in the Department of Cardiac Surgery, Guangdong Provincial People's Hospital from January 2010 to January 2020 were retrospectively analyzed. There were 48 males and 20 females with a median age of 2.8 years ranging from 3 months to 33 years. Except one patient undergoing the coarctation repair, the rest 67 patients underwent surgical interventions to correct supravalvular aortic stenosis (SAVS) and pulmonary artery stenosis with hypothermic cardiopulmonary bypass, concommitant with 3 patients of relief of left ventricular outflow tract obstruction, 2 patients of relief of right ventricular outflow tract obstruction, 2 patients of mitral valvuloplasty, 3 patients of ventricular septal defect repair and 1 patient of arterial catheter ligation. Results Two (2.9%) patients died of sudden cardiac arrest on the next day after surgery. One (1.5%) patient died of cardiac insufficiency due to severe aortic arch stenosis 3 years after surgery. The effect of SAVS was satisfactory. Two (2.9%) patients progressed to moderate aortic valvular regurgitation during postoperative follow-up. A total of 5 (7.4%) patients were re-intervened after operation for arch stenosis or pulmonary stenosis. Conclusion WS patients should be diagnosed early, followed up and assessed for cardiovascular system diseases, and timely surgical treatment has a good clinical effect.
