Surgical treatment for congenital heart diseases with pulmonary artery hypertension in Down syndrome infants_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

XU Yanbin , ZHOU Li ,  WANG Shuwei , HANG Yongbin , CAO Junying , LI Gang

Department of Cardiothoracic Surgery, No.153 Hospital of Liberation Army, Zhengzhou, 450042, P.R.China;

Corresponding author：

WANG Shuwei, Email: wangsw007@163.com

Keywords：

Infants; Down syndrome; congenital heart disease; pulmonary artery hypertension

DOI：

10.7507/1007-4848.201808020

Video：

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Objective To explore the treatment method of congenital heart disease (CHD) with pulmonary artery hypertension (PAH) in infants with Down syndrome (DS). Methods The clinical data of 60 CHD patients with PAH from March 2015 to August 2016 in our hospital were retrospectively analyzed. There were 30 infants with DS classified as a DS group (trial group, 17 males and 13 females with a mean age of 1.15±0.25 years) and the other 30 patients without DS were classified as a control group (20 males and 10 females with a mean age of 1.24±0.30 years). All the patients underwent surgical treatment and fasudil combined with sildenafil were used to prevent pulmonary hypertension crisis postoperatively. Results There was no significant difference in cardiopulmonary bypass time, aortic cross-clamping time, modified ultrafiltration time and the incidence of postoperative respiratory complications between the two groups. The pulmonary systolic blood pressure significantly decreased at 24 h after operation in the two groups (both P<0.05). The arterial oxygen pressure and oxygenation index of the trial group were lower than those of the control group at 6 h after operation (both P<0.05). The mechanical ventilation time and intensive care time of the trial group were significantly longer than those of the control group (P=0.007 and P=0.000, respectively). There were no reoperations or early death. Conclusion The effects of surgical repair of CHD with PAH in infants with DS are satisfactory by grasping the indication, protecting lung function and controlling PAH in the early postoperative period, although there is a high incidence of pulmonary complications.

Citation： XU Yanbin, ZHOU Li, WANG Shuwei, HANG Yongbin, CAO Junying, LI Gang. Surgical treatment for congenital heart diseases with pulmonary artery hypertension in Down syndrome infants. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2019, 26(5): 461-464. doi: 10.7507/1007-4848.201808020 Copy

1.	Saji T. Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome. Pediatr Int, 2014, 56(3): 297-303.
2.	Fudge JC Jr, Li S, Jaggers J, et al. Congenital heart surgery outcomes in Down syndrome: analysis of a national clinical database. Pediatrics, 2010, 126(2): 315-322.
3.	刘迎龙. 先天性心脏病相关肺动脉高压的临床诊治. 心肺血管病杂志, 2015, 34(11): 809-811.
4.	高伟, 顾红, 胡大一, 等. 2015 年先天性心脏病相关性肺动脉高压诊治中国专家共识. 中国介入心脏病学杂志, 2015, 23(2): 61-69.
5.	张小贞, 李刚, 王树伟, 等. 优化改良超滤用于婴幼儿心脏手术的临床效果研究. 中国体外循环杂志, 2018, 16(2): 76-79.
6.	Haeck ML, Vliegen HW. Diagnosis and treatment of pulmonary hypertension. Heart, 2015, 101(4): 311-319.
7.	张国富, 刘宏宇. 肺动脉高压治疗的进展. 中国胸心血管外科临床杂志, 2015, 22(12): 1157-1162.
8.	Cua CL, Rogers LK, Chicoine LG, et al. Down syndrome patients with pulmonary hypertension have elevated plasma levels of asymmetric dimethylarginine. Eur J Pediatr, 2011, 170(7): 859-863.
9.	Cannon BC, Feltes TF, Fraley JK, et al. Nitric oxide in the evaluation of congenital heart disease with pulmonary hypertension: factors related to nitric oxide response. Pediatr Cardiol, 2005, 26(5): 565-569.
10.	D’Alto M, Romeo E, Argiento P, et al. Therapy for pulmonary arterial hypertension due to congenital heart disease and Down’s syndrome. Int J Cardiol, 2013, 164(3): 323-326.
11.	刘义敏, 王辉, 杨宇强, 等. 内皮素受体拮抗剂在围术期治疗 6 个月以下婴儿肺动脉高压的临床意义. 中国胸心血管外科临床杂志, 2011, 18(5): 478-480.
12.	范长生, 吴久鸿. 波生坦片治疗肺动脉高压成本-效用分析. 药品评价, 2016, 13(8): 13-19.
13.	Ziino AJ, Ivanovska J, Belcastro R, et al. Effects of rho-kinase inhibition on pulmonary hypertension, lung growth, and structure in neonatal rats chronically exposed to hypoxia. Pediatr Res, 2010, 67(2): 177-182.
14.	Firth AL, Choi IW, Park WS. Animal models of pulmonary hypertension: Rho kinase inhibition. Prog Biophys Mol Biol, 2012, 109(3): 67-75.
15.	Mouchaers KT, Schalij I, de Boer MA, et al. Fasudil reduces monocrotaline-induced pulmonary arterial hypertension: comparison with bosentan and sildenafil. Eur Respir J, 2010, 36(4): 800-807.
16.	Antoniu SA. Targeting RhoA/ROCK pathway in pulmonary arterial hypertension. Expert Opin Ther Targets, 2012, 16(4): 355-363.
17.	Galambos C. Alveolar capillary dysplasia in a patient with Down’s syndrome. Pediatr Dev Pathol, 2006, 9(3): 254-255.
18.	Shehata BM, Abramowsky CR. Alveolar capillary dysplasia in an infant with trisomy 21. Pediatr Dev Pathol, 2005, 8(6): 696-700.

1. Saji T. Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome. Pediatr Int, 2014, 56(3): 297-303.
2. Fudge JC Jr, Li S, Jaggers J, et al. Congenital heart surgery outcomes in Down syndrome: analysis of a national clinical database. Pediatrics, 2010, 126(2): 315-322.
3. 刘迎龙. 先天性心脏病相关肺动脉高压的临床诊治. 心肺血管病杂志, 2015, 34(11): 809-811.
4. 高伟, 顾红, 胡大一, 等. 2015 年先天性心脏病相关性肺动脉高压诊治中国专家共识. 中国介入心脏病学杂志, 2015, 23(2): 61-69.
5. 张小贞, 李刚, 王树伟, 等. 优化改良超滤用于婴幼儿心脏手术的临床效果研究. 中国体外循环杂志, 2018, 16(2): 76-79.
6. Haeck ML, Vliegen HW. Diagnosis and treatment of pulmonary hypertension. Heart, 2015, 101(4): 311-319.
7. 张国富, 刘宏宇. 肺动脉高压治疗的进展. 中国胸心血管外科临床杂志, 2015, 22(12): 1157-1162.
8. Cua CL, Rogers LK, Chicoine LG, et al. Down syndrome patients with pulmonary hypertension have elevated plasma levels of asymmetric dimethylarginine. Eur J Pediatr, 2011, 170(7): 859-863.
9. Cannon BC, Feltes TF, Fraley JK, et al. Nitric oxide in the evaluation of congenital heart disease with pulmonary hypertension: factors related to nitric oxide response. Pediatr Cardiol, 2005, 26(5): 565-569.
10. D’Alto M, Romeo E, Argiento P, et al. Therapy for pulmonary arterial hypertension due to congenital heart disease and Down’s syndrome. Int J Cardiol, 2013, 164(3): 323-326.
11. 刘义敏, 王辉, 杨宇强, 等. 内皮素受体拮抗剂在围术期治疗 6 个月以下婴儿肺动脉高压的临床意义. 中国胸心血管外科临床杂志, 2011, 18(5): 478-480.
12. 范长生, 吴久鸿. 波生坦片治疗肺动脉高压成本-效用分析. 药品评价, 2016, 13(8): 13-19.
13. Ziino AJ, Ivanovska J, Belcastro R, et al. Effects of rho-kinase inhibition on pulmonary hypertension, lung growth, and structure in neonatal rats chronically exposed to hypoxia. Pediatr Res, 2010, 67(2): 177-182.
14. Firth AL, Choi IW, Park WS. Animal models of pulmonary hypertension: Rho kinase inhibition. Prog Biophys Mol Biol, 2012, 109(3): 67-75.
15. Mouchaers KT, Schalij I, de Boer MA, et al. Fasudil reduces monocrotaline-induced pulmonary arterial hypertension: comparison with bosentan and sildenafil. Eur Respir J, 2010, 36(4): 800-807.
16. Antoniu SA. Targeting RhoA/ROCK pathway in pulmonary arterial hypertension. Expert Opin Ther Targets, 2012, 16(4): 355-363.
17. Galambos C. Alveolar capillary dysplasia in a patient with Down’s syndrome. Pediatr Dev Pathol, 2006, 9(3): 254-255.
18. Shehata BM, Abramowsky CR. Alveolar capillary dysplasia in an infant with trisomy 21. Pediatr Dev Pathol, 2005, 8(6): 696-700.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Surgical treatment for congenital heart diseases with pulmonary artery hypertension in Down syndrome infants

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