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.	劉迎龍. 先天性心臟病相關肺動脈高壓的臨床診治. 心肺血管病雜志, 2015, 34(11): 809-811.
4.	高偉, 顧紅, 胡大一, 等. 2015 年先天性心臟病相關性肺動脈高壓診治中國專家共識. 中國介入心臟病學雜志, 2015, 23(2): 61-69.
5.	張小貞, 李剛, 王樹偉, 等. 優化改良超濾用于嬰幼兒心臟手術的臨床效果研究. 中國體外循環雜志, 2018, 16(2): 76-79.
6.	Haeck ML, Vliegen HW. Diagnosis and treatment of pulmonary hypertension. Heart, 2015, 101(4): 311-319.
7.	張國富, 劉宏宇. 肺動脈高壓治療的進展. 中國胸心血管外科臨床雜志, 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.	劉義敏, 王輝, 楊宇強, 等. 內皮素受體拮抗劑在圍術期治療 6 個月以下嬰兒肺動脈高壓的臨床意義. 中國胸心血管外科臨床雜志, 2011, 18(5): 478-480.
12.	范長生, 吳久鴻. 波生坦片治療肺動脈高壓成本-效用分析. 藥品評價, 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. 劉迎龍. 先天性心臟病相關肺動脈高壓的臨床診治. 心肺血管病雜志, 2015, 34(11): 809-811.
4. 高偉, 顧紅, 胡大一, 等. 2015 年先天性心臟病相關性肺動脈高壓診治中國專家共識. 中國介入心臟病學雜志, 2015, 23(2): 61-69.
5. 張小貞, 李剛, 王樹偉, 等. 優化改良超濾用于嬰幼兒心臟手術的臨床效果研究. 中國體外循環雜志, 2018, 16(2): 76-79.
6. Haeck ML, Vliegen HW. Diagnosis and treatment of pulmonary hypertension. Heart, 2015, 101(4): 311-319.
7. 張國富, 劉宏宇. 肺動脈高壓治療的進展. 中國胸心血管外科臨床雜志, 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. 劉義敏, 王輝, 楊宇強, 等. 內皮素受體拮抗劑在圍術期治療 6 個月以下嬰兒肺動脈高壓的臨床意義. 中國胸心血管外科臨床雜志, 2011, 18(5): 478-480.
12. 范長生, 吳久鴻. 波生坦片治療肺動脈高壓成本-效用分析. 藥品評價, 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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