Effect of pulmonary arterial hypertension on early outcomes of children with functional single ventricle after Fontan operation: A case control study_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

ZHANG Mingjie ,  XU Zhuoming , HUANG Rui , SUN Chongrui

Department of Cardiothoracic Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, 200127, P.R.China;

Corresponding author：

XU Zhuoming, Email: zmxyfb@163.com

Keywords：

Fontan operation; pulmonary arterial hypertension; early outcomes

DOI：

10.7507/1007-4848.201709033

Video：

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Objective To explore the effect of pulmonary arterial hypertension on the children with functional single ventricle in the early period after Fontan operation. Methods Forty-three children with pulmonary arterial hypertension after Fontan operation were enrolled in our department between January 2015 and December 2016. There were 24 males and 19 females at a median age of 4.3 years ranging from 2.5 to 4.8 years. The pulmonary arterial pressure was evaluated by cardiac catheterization. There were 23 children diagnosed without pulmonary hypertension (a non-PAH group) including 16 males and 7 females, while 20 patients were diagnosed with pulmonary hypertension (a PAH group) including 8 males and 12 females. Postoperative parameters related to outcomes were compared between the two groups. Results There was no death in the non-PAH group, but the mortality of children in the PAH group was 20.0% (4/20, χ²=5.34, P=0.02). The central venous pressure (t=–2.50, P=0.02), N-terminal prohormone of brain natriuretic peptide (NT-proBNP, Z=–3.50, P<0.01), peritoneal dialysis rate (χ²=5.40, P=0.02), incidence of arrhythmia (χ²=4.40, P=0.03) in the PAH group were significantly higher than those of the non-PAH group. The early postoperative utilization rate of pulmonary vascular targeting agents in the PAH group was significantly higher than that in the non-PAH group (χ²=6.30, P=0.04). Conclusion Pulmonary arterial hypertension is one of the most important factors which influence the early postoperative prognosis of children with functional single ventricle after Fontan operation.

Citation： ZHANG Mingjie, XU Zhuoming, HUANG Rui, SUN Chongrui. Effect of pulmonary arterial hypertension on early outcomes of children with functional single ventricle after Fontan operation: A case control study. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2018, 25(2): 123-127. doi: 10.7507/1007-4848.201709033 Copy

1.	Gorenflo M, Gu H, Xu Z. Peri-operative pulmonary hypertension in paediatrics: current strategies in children with congenital heart disease. Cardiology, 2010, 116(1): 10-17.
2.	Samánek M. Children with congenital heart disease: probability of natural survival. Pediatr Cardiol, 1992, 13(3): 152-158.
3.	Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax, 1971, 26(2): 240-248.
4.	Pundi KN, Johnson JN, Dearani JA, et al. 40-year follow-up after the fontan operation: long-term outcomes of 1, 052 patients. J Am Coll Cardiol, 2015, 66(15): 1700-1710.
5.	Deal BJ, Costello JM, Webster G, et al. Intermediate-term outcome of 140 consecutive Fontan conversions with arrhythmia operations. Ann Thorac Surg, 2016, 101(2): 717-724.
6.	Petit CJ.Staged single-ventricle palliation in 2011: outcomes and expectations. Congenit Heart Dis, 2011, 6(5): 406-416.
7.	Krieger EV, Leary PJ, Opotowsky AR. Pulmonary hypertension in congenital heart disease: beyond eisenmenger syndrome. Cardiol Clin, 2015, 33(4): 599-609.
8.	Myers PO, Tissot C, Beghetti M. Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease. Circ J, 2014, 78(1): 4-11.
9.	Gewillig M, Brown SC, Eyskens B, Heying R, et al. The Fontan circulation: who controls cardiac output? Interact Cardiovasc Thoracic Surg, 2010, 10(3): 428-433.
10.	Xu Z, Zhu L, Liu X, et al. Iloprost for children with pulmonary hypertension after surgery to correct congenital heart disease. Pediatr Pulmonol, 2015, 50(6): 588-595.
11.	Bizzarro M, Gross I, Barbosa FT. Inhaled nitric oxide for the postoperative management of pulmonary hypertension in infants and children with congenital heart disease. Cochrane Database Syst Rev, 2014, (7): CD005055.
12.	Brunner N, de Jesus Perez VA, Richter A, et al. Perioperative pharmacological management of pulmonary hypertensive crisis during congenital heart surgery. Pulm Circ, 2014, 4(1): 10-24.
13.	Hill SA, Booth RA, Santaguida PL, et al. Use of BNP and NT-proBNP for the diagnosis of heart failure in the emergency department: a systematic review of the evidence. Heart Fail Rev, 2014, 19(4): 421-438.
14.	Zhou FJ, Zhou CY, Tian YJ, et al. Diagnostic value of analysis of H-FABP, NT-proBNP, and cTnI in heart function in children with congenital heart disease and pneumonia. Eur Rev Med Pharmacol Sci, 2014, 18(10): 1513-1516.
15.	Sargento L, Longo S, Lousada N, et al. Serial measurements of the Nt-ProBNP during the dry state in patients with systolic heart failure are predictors of the long-term prognosis. Biomarkers, 2014, 19(4): 302-313.
16.	Song MK, Bae EJ, Kwon BS, et al. Intra-atrial reentrant tachycardia in adult patients after Fontan operation. Int J Cardiol, 2015, 187: 157-163.
17.	Chen H, Hong H, Zhu Z, et al. Extracardiac Fontan with direct cavopulmonary connections: midterm results. Eur J Cardiothorac Surg, 2013, 43(2): 318-323.
18.	Latus H, Gerstner B, Kerst G, et al. Effect of inhaled Nitric Oxide on blood flow dynamics in patients after the fontan procedure using cardiovascular magnetic resonance flow measurements. Pediatr Cardiol, 2016, 37(3): 504-511.
19.	Kim YH, Chae MH, Choi DY. Inhaled iloprost for the treatment of patient with Fontan circulation. Korean J Pediatr, 2014, 57(10): 461-463.
20.	Shabanian R, Shahbaznejad L, Razaghian A, et al. Sildenafil and ventriculo-arterial coupling in Fontan-palliated patients: a noninvasive echocardiographic assessment. Pediatr Cardiol, 2013, 34(1): 129-134.
21.	Derk G, An R, Aboulhosn J. Endothelin receptor antagonism in single ventricle physiology with Fontan palliation: A systematic review and meta-analysis. Clin Trials Regulat Sci Cardiol, 2015, 4: 1-5.

1. Gorenflo M, Gu H, Xu Z. Peri-operative pulmonary hypertension in paediatrics: current strategies in children with congenital heart disease. Cardiology, 2010, 116(1): 10-17.
2. Samánek M. Children with congenital heart disease: probability of natural survival. Pediatr Cardiol, 1992, 13(3): 152-158.
3. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax, 1971, 26(2): 240-248.
4. Pundi KN, Johnson JN, Dearani JA, et al. 40-year follow-up after the fontan operation: long-term outcomes of 1, 052 patients. J Am Coll Cardiol, 2015, 66(15): 1700-1710.
5. Deal BJ, Costello JM, Webster G, et al. Intermediate-term outcome of 140 consecutive Fontan conversions with arrhythmia operations. Ann Thorac Surg, 2016, 101(2): 717-724.
6. Petit CJ.Staged single-ventricle palliation in 2011: outcomes and expectations. Congenit Heart Dis, 2011, 6(5): 406-416.
7. Krieger EV, Leary PJ, Opotowsky AR. Pulmonary hypertension in congenital heart disease: beyond eisenmenger syndrome. Cardiol Clin, 2015, 33(4): 599-609.
8. Myers PO, Tissot C, Beghetti M. Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease. Circ J, 2014, 78(1): 4-11.
9. Gewillig M, Brown SC, Eyskens B, Heying R, et al. The Fontan circulation: who controls cardiac output? Interact Cardiovasc Thoracic Surg, 2010, 10(3): 428-433.
10. Xu Z, Zhu L, Liu X, et al. Iloprost for children with pulmonary hypertension after surgery to correct congenital heart disease. Pediatr Pulmonol, 2015, 50(6): 588-595.
11. Bizzarro M, Gross I, Barbosa FT. Inhaled nitric oxide for the postoperative management of pulmonary hypertension in infants and children with congenital heart disease. Cochrane Database Syst Rev, 2014, (7): CD005055.
12. Brunner N, de Jesus Perez VA, Richter A, et al. Perioperative pharmacological management of pulmonary hypertensive crisis during congenital heart surgery. Pulm Circ, 2014, 4(1): 10-24.
13. Hill SA, Booth RA, Santaguida PL, et al. Use of BNP and NT-proBNP for the diagnosis of heart failure in the emergency department: a systematic review of the evidence. Heart Fail Rev, 2014, 19(4): 421-438.
14. Zhou FJ, Zhou CY, Tian YJ, et al. Diagnostic value of analysis of H-FABP, NT-proBNP, and cTnI in heart function in children with congenital heart disease and pneumonia. Eur Rev Med Pharmacol Sci, 2014, 18(10): 1513-1516.
15. Sargento L, Longo S, Lousada N, et al. Serial measurements of the Nt-ProBNP during the dry state in patients with systolic heart failure are predictors of the long-term prognosis. Biomarkers, 2014, 19(4): 302-313.
16. Song MK, Bae EJ, Kwon BS, et al. Intra-atrial reentrant tachycardia in adult patients after Fontan operation. Int J Cardiol, 2015, 187: 157-163.
17. Chen H, Hong H, Zhu Z, et al. Extracardiac Fontan with direct cavopulmonary connections: midterm results. Eur J Cardiothorac Surg, 2013, 43(2): 318-323.
18. Latus H, Gerstner B, Kerst G, et al. Effect of inhaled Nitric Oxide on blood flow dynamics in patients after the fontan procedure using cardiovascular magnetic resonance flow measurements. Pediatr Cardiol, 2016, 37(3): 504-511.
19. Kim YH, Chae MH, Choi DY. Inhaled iloprost for the treatment of patient with Fontan circulation. Korean J Pediatr, 2014, 57(10): 461-463.
20. Shabanian R, Shahbaznejad L, Razaghian A, et al. Sildenafil and ventriculo-arterial coupling in Fontan-palliated patients: a noninvasive echocardiographic assessment. Pediatr Cardiol, 2013, 34(1): 129-134.
21. Derk G, An R, Aboulhosn J. Endothelin receptor antagonism in single ventricle physiology with Fontan palliation: A systematic review and meta-analysis. Clin Trials Regulat Sci Cardiol, 2015, 4: 1-5.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Effect of pulmonary arterial hypertension on early outcomes of children with functional single ventricle after Fontan operation: A case control study

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