Outcome assessment of different surgeries for neonates with pulmonary atresia and ventricular septal defect_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

QU Yanji ¹ , LIU Xiaoqing ¹ , ZHUANG Jian ² , CHEN Jimei ² , XU Gang ² , WEN Shusheng ² , TENG Yun ² ,  CEN Jianzheng ²

1. Department of Cardiovascular Epidemiology, Guangdong Cardiovascular Institute, Guangdong General Hospital, Guangdong Academic of Medical Science, Guangzhou, 510100, P.R.China;
2. Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong General Hospital, Guangdong Academic of Medical Science, Guangzhou, 510080, P.R.China;

Corresponding author：

CEN Jianzheng, Email: jamesofsolar@163.com

Keywords：

Heart disease, congenital; cardiac surgical procedures; pulmonary atresia; ventricular septal defect; neonates

DOI：

10.7507/1007-4848.201803010

Video：

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Objective To explore the feasibility and option of different surgeries for neonates with pulmonary atresia and ventricular septal defect (PA/VSD) through assessing the effect of common surgeries. Methods Fourteen neonates who underwent their first surgery in our center from July 2004 to October 2014 were included. Their basic characteristics, operation and pre- and postoperative clinical information were extracted. Follow up was conducted and the last visit was on October 10, 2016. Short- and midterm survival and total correction rate were compared among different surgeries. Results Among the 14 patients, there were 4 (28.6%) patients, 6 (42.9%) and 4 (28.6%) who underwent one-stage repair, right ventricular outflow tract (RVOT) reconstruction, and systemic to PA shunt operation respectively. The overall in-hospital mortality after the first operation was 28.6% (4/14). At last visit, no death occurred resulting the 5-year survival rate of 71.4% (10/14). The overall total correction rate for all neonates was 64.3% (9/14). Although no statistical difference was found in the mortality among the one-stage repair , RVOT reconstruction and systemic to PA shunt group(50.0% vs. 33.3% vs. 0.0%, P=0.280), the survival and hazard analysis implied better outcomes of the systemic to PA shunt palliation operation. There was no statistical difference in the total correction rate and months from the first palliative operation to correction between those who underwent RVOT reconstruction and systemic to PA shunt (75.0% vs. 50.0%, P=0.470; 32.0 months vs. 18.0 months, P=0.400). Conclusion Performing surgeries for neonates with PA/VSD is still a great challenge. However, the midterm survival rate was optimistic for the early survivors. Systematic to PA shunt seemed to be a better choice with lower mortality for the neonates with PA/VSD who need the surgery to survive.

Citation： QU Yanji, LIU Xiaoqing, ZHUANG Jian, CHEN Jimei, XU Gang, WEN Shusheng, TENG Yun, CEN Jianzheng. Outcome assessment of different surgeries for neonates with pulmonary atresia and ventricular septal defect. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2018, 25(12): 1054-1059. doi: 10.7507/1007-4848.201803010 Copy

1.	Leonard H, Derrick G, O'Sullivan J, et al. Natural and unnatural history of pulmonary atresia. Heart, 2000, 84(5): 499-503.
2.	Qu Y, Liu X, Zhuang J, et al. Incidence of congenital heart disease: the 9-year experience of the guangdong registry of congenital heart disease, China. PLoS One, 2016, 11(7): e0159257.
3.	Tchervenkov CI, Roy N. Congenital heart surgery nomenclature and database project: pulmonary atresia--ventricular septal defect. Ann Thorac Surg, 2000, 69(4 Suppl): S97-S105.
4.	Carotti A, Albanese SB, Filippelli S, et al. Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg, 2010, 140(5): 1092-1103.
5.	Alsoufi B, Mori M, McCracken C, et al. Results of primary repair versus shunt palliation in ductal dependent infants with pulmonary atresia and ventricular septal defect. Ann Thorac Surg, 2015, 100(2): 639-646.
6.	Jonas RA, Castaneda AR, Freed MD. Normothermic caval inflow occlusion. Application to operations for congenital heart disease. J Thorac Cardiovasc Surg, 1985, 89(5): 780-786.
7.	Watanabe N, Mainwaring RD, Reddy VM, et al. Early complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. Ann Thorac Surg, 2014, 97(3): 909-15.
8.	Gerelli S, van Steenberghe M, Murtuza B, et al. Neonatal right ventricle to pulmonary connection as a palliative procedure for pulmonary atresia with ventricular septal defect or severe tetralogy of Fallot. Eur J Cardiothorac Surg, 2014, 45(2): 278-288.
9.	Alkhulaifi AM, Lacour-Gayet F, Serraf A, et al. Systemic pulmonary shunts in neonates: early clinical outcome and choice of surgical approach. Ann Thorac Surg, 2000, 69(5): 1499-1504.
10.	Reddy VM, McElhinney DB, Amin Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation, 2000, 101(15): 1826-1832.
11.	Lofland GK. The management of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries by definitive single stage repair in early infancy. Eur J Cardiothorac Surg, 2000, 18(4): 480-486.
12.	Kwak JG, Lee CH, Lee C, et al. Surgical management of pulmonary atresia with ventricular septal defect: early total correction versus shunt. Ann Thorac Surg, 2011, 91(6): 1928-1934.
13.	Carotti A, Albanese SB, Minniti G, et al. Increasing experience with integrated approach to pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Eur J Cardiothorac Surg, 2003, 23(5): 719-726.
14.	Mohammadi S, Benhameid O, Campbell A, et al. Could we still improve early and interim outcome after prosthetic systemic-pulmonary shunt? A risk factors analysis. Eur J Cardiothorac Surg, 2008, 34(3): 545-549.
15.	Kim H, Sung SC, Chang YH, et al. Outcome of staged repair of tetralogy of fallot with pulmonary atresia and a ductus-dependent pulmonary circulation: should primary repair be considered? Korean J Thorac Cardiovasc Surg, 2011, 44(6): 392-398.

1. Leonard H, Derrick G, O'Sullivan J, et al. Natural and unnatural history of pulmonary atresia. Heart, 2000, 84(5): 499-503.
2. Qu Y, Liu X, Zhuang J, et al. Incidence of congenital heart disease: the 9-year experience of the guangdong registry of congenital heart disease, China. PLoS One, 2016, 11(7): e0159257.
3. Tchervenkov CI, Roy N. Congenital heart surgery nomenclature and database project: pulmonary atresia--ventricular septal defect. Ann Thorac Surg, 2000, 69(4 Suppl): S97-S105.
4. Carotti A, Albanese SB, Filippelli S, et al. Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg, 2010, 140(5): 1092-1103.
5. Alsoufi B, Mori M, McCracken C, et al. Results of primary repair versus shunt palliation in ductal dependent infants with pulmonary atresia and ventricular septal defect. Ann Thorac Surg, 2015, 100(2): 639-646.
6. Jonas RA, Castaneda AR, Freed MD. Normothermic caval inflow occlusion. Application to operations for congenital heart disease. J Thorac Cardiovasc Surg, 1985, 89(5): 780-786.
7. Watanabe N, Mainwaring RD, Reddy VM, et al. Early complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. Ann Thorac Surg, 2014, 97(3): 909-15.
8. Gerelli S, van Steenberghe M, Murtuza B, et al. Neonatal right ventricle to pulmonary connection as a palliative procedure for pulmonary atresia with ventricular septal defect or severe tetralogy of Fallot. Eur J Cardiothorac Surg, 2014, 45(2): 278-288.
9. Alkhulaifi AM, Lacour-Gayet F, Serraf A, et al. Systemic pulmonary shunts in neonates: early clinical outcome and choice of surgical approach. Ann Thorac Surg, 2000, 69(5): 1499-1504.
10. Reddy VM, McElhinney DB, Amin Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation, 2000, 101(15): 1826-1832.
11. Lofland GK. The management of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries by definitive single stage repair in early infancy. Eur J Cardiothorac Surg, 2000, 18(4): 480-486.
12. Kwak JG, Lee CH, Lee C, et al. Surgical management of pulmonary atresia with ventricular septal defect: early total correction versus shunt. Ann Thorac Surg, 2011, 91(6): 1928-1934.
13. Carotti A, Albanese SB, Minniti G, et al. Increasing experience with integrated approach to pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Eur J Cardiothorac Surg, 2003, 23(5): 719-726.
14. Mohammadi S, Benhameid O, Campbell A, et al. Could we still improve early and interim outcome after prosthetic systemic-pulmonary shunt? A risk factors analysis. Eur J Cardiothorac Surg, 2008, 34(3): 545-549.
15. Kim H, Sung SC, Chang YH, et al. Outcome of staged repair of tetralogy of fallot with pulmonary atresia and a ductus-dependent pulmonary circulation: should primary repair be considered? Korean J Thorac Cardiovasc Surg, 2011, 44(6): 392-398.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Outcome assessment of different surgeries for neonates with pulmonary atresia and ventricular septal defect

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