Early and mid-term results of Fontan operation versus anatomic correction for congenitally corrected transposition of the great arteries: A retrospective cohort study in a single center_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

WANG Tao ,  CHEN Xinxin , ZOU Minghui , QU Jiangbo

Heart Center, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, 510623, P. R. China;

Corresponding author：

CHEN Xinxin, Email: zingerchen@163.com

Keywords：

Congenitally corrected transposition of the great arteries; anatomic correction; Fontan operation; cardiopulmonary exercise testing

DOI：

10.7507/1007-4848.202201026

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Objective To compare the early and mid-term results between Fontan operation and anatomic correction for congenitally corrected transposition of the great arteries (ccTGA). Methods The clinical data of 53 patients with ccTGA who underwent anatomic correction and Fontan operation from January 2009 to September 2021 in our hospital were reviewed, including 41 males and 12 females with a mean age of 55.02 (3-168) months. They were divided into an anatomic correction group (16 patients) and a Fontan operation group (37 patients) according to the operation. The hospitalization mortality, survival rate, postoperative complications, and free rate from re-intervention between the two groups were compared. Another 180 healthy children were recruited as a control group, and 14 children were matched with the propensity score matching method as a Fontan control group. The results of cardiopulmonary exercise testing (CPET) between the Fontan operation group and the Fontan control group were compared. Results There were 2 (12.5%) early deaths and 3 (18.8%) early re-intervention in the anatomic correction group, while 1 death and 2 re-intervention in the Fontan operation group. In addition, there were 9 patients (56.3%) in the anatomic correction group and 6 (16.2%) patients in the Fontan operation group suffering from arrhythmia after operation, respectively. Compared with the anatomic correction group, cardiopulmonary bypass time, aortic cross-clamping time, intubation time and ICU stay were significantly shortened in the Fontan operation group (P<0.05). CPET results showed that, percent predicted max VO₂ in the Fontan operation group was lower than that in the Fontan control group (0.84±0.11 vs. 0.99±0.12, P＜0.05). The patients were followed up for 0.5-126.0 months. Two patients were lost in the Fontan operation group. There was no death and 1 re-intervention in the anatomic correction group, while no death or re-intervention in the Fontan operation group. The 1-year, 5-year and 10-year transplant-free survival rate of the anatomic correction group and the Fontan operation group was 87.5%, 87.5%, 87.5% and 97.3%, 97.3%, 97.3%, respectively (P＞0.05). The 48 patients were classified as grade Ⅰ-Ⅱ in cardiac function in the last follow-up. Conclusion There is no statistical difference in the transplant-free survival rate between the anatomic correction and the Fontan operation group. The postoperative complications in the Fontan operation group are decreased than those in the anatomic correction group. The Fontan operation is also a good choice, even though the patients with ccTGA meet the condition of the procedure of anatomic correction.

Citation： WANG Tao, CHEN Xinxin, ZOU Minghui, QU Jiangbo. Early and mid-term results of Fontan operation versus anatomic correction for congenitally corrected transposition of the great arteries: A retrospective cohort study in a single center. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2023, 30(9): 1308-1315. doi: 10.7507/1007-4848.202201026 Copy

1.	Connelly MS, Liu PP, Williams WG, et al. Congenitally corrected transposition of the great arteries in the adult: Functional status and complications. J Am Coll Cardiol, 1996, 27(5): 1238-1243.
2.	Alghamdi AA, McCrindle BW, Van Arsdell GS. Physiologic versus anatomic repair of congenitally corrected transposition of the great arteries: Meta-analysis of individual patient data. Ann Thorac Surg, 2006, 81(4): 1529-1535.
3.	Karl TR. The role of the Fontan operation in the treatment of congenitally corrected transposition of the great arteries. Ann Pediatr Cardiol, 2011, 4(2): 103-110.
4.	Sano T, Riesenfeld T, Karl TR, et al. Intermediate-term outcome after intracardiac repair of associated cardiac defects in patients with atrioventricular and ventriculoarterial discordance. Circulation, 1995, 92(9 Suppl): Ⅱ272-Ⅱ278.
5.	Hraska V, Duncan BW, Mayer JE, et al. Long-term outcome of surgically treated patients with corrected transposition of the great arteries. J Thorac Cardiovasc Surg, 2005, 129(1): 182-191.
6.	Simmons MA, Rollinson N, Fishberger S, et al. Modern incidence of complete heart block in patients with L-looped ventricles: Does univentricular status matter? Congenit Heart Dis, 2015, 10(5): E237-E242.
7.	De León LE, Mery CM, Verm RA, et al. Mid-term outcomes in patients with congenitally corrected transposition of the great arteries: A single center experience. J Am Coll Surg, 2017, 224(4): 707-715.
8.	Hörer J, Schreiber C, Krane S, et al. Outcome after surgical repair/palliation of congenitally corrected transposition of the great arteries. Thorac Cardiovasc Surg, 2008, 56(7): 391-397.
9.	Hsu KH, Chang CI, Huang SC, et al. 17-year experience in surgical management of congenitally corrected transposition of the great arteries: A single-centre's experience. Eur J Cardiothorac Surg, 2016, 49(2): 522-527.
10.	Shin'oka T, Kurosawa H, Imai Y, et al. Outcomes of definitive surgical repair for congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections: Risk analyses in 189 patients. J Thorac Cardiovasc Surg, 2007, 133(5): 1318-1328.
11.	Talwar S, Bansal A, Choudhary SK, et al. Results of Fontan operation in patients with congenitally corrected transposition of great arteries. Interact Cardiovasc Thorac Surg, 2016, 22(2): 188-193.
12.	Spigel Z, Binsalamah ZM, Caldarone C. Congenitally corrected transposition of the great arteries: Anatomic, physiologic repair, and palliation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2019, 22: 32-42.
13.	肖旭文, 曲江波, 石慧, 等. 儿童先天性心脏病手术后运动耐量的评价. 临床小儿外科杂志, 2021, 20(6): 538-544.
14.	Cooper DM, Weiler-Ravell D, Whipp BJ, et al. Aerobic parameters of exercise as a function of body size during growth in children. J Appl Physiol Respir Environ Exerc Physiol, 1984, 56(3): 628-634.
15.	Qu J, Shi H, Chen X, et al. Evaluation of physical fitness in children with congenital heart diseases versus healthy population. Semin Thorac Cardiovasc Surg, 2020, 32(4): 906-915.
16.	Graham TP, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: A multi-institutional study. J Am Coll Cardiol, 2000, 36(1): 255-261.
17.	Prieto LR, Hordof AJ, Secic M, et al. Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries. Circulation, 1998, 98(10): 997-1005.
18.	Graham TP, Parrish MD, Boucek RJ, et al. Assessment of ventricular size and function in congenitally corrected transposition of the great arteries. Am J Cardiol, 1983, 51(2): 244-251.
19.	Bautista-Hernandez V, Marx GR, Gauvreau K, et al. Determinants of left ventricular dysfunction after anatomic repair of congenitally corrected transposition of the great arteries. Ann Thorac Surg, 2006, 82(6): 2059-2065.
20.	Hiramatsu T, Matsumura G, Konuma T, et al. Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries. Eur J Cardiothorac Surg, 2012, 42(6): 1004-1008.
21.	Murtuza B, Barron DJ, Stumper O, et al. Anatomic repair for congenitally corrected transposition of the great arteries: A single-institution 19-year experience. J Thorac Cardiovasc Surg, 2011, 142(6): 1348-1357.
22.	Lenoir M, Bouhout I, Gaudin R, et al. Outcomes of the anatomical repair in patients with congenitally corrected transposition of the great arteries: Lessons learned in a high-volume centre. Eur J Cardiothorac Surg, 2018, 54(3): 532-538.
23.	DiBardino DJ, Heinle JS, Fraser CD. The hemi-Mustard, bi-directional Glenn, and Rastelli operations used for correction of congenitally corrected transposition, achieving a "ventricle and a half " repair. Cardiol Young, 2004, 14(3): 330-332.
24.	Malhotra SP, Reddy VM, Qiu M, et al. The hemi-Mustard/bidirectional Glenn atrial switch procedure in the double-switch operation for congenitally corrected transposition of the great arteries: Rationale and midterm results. J Thorac Cardiovasc Surg, 2011, 141(1): 162-170.
25.	Cools B, Brown SC, Louw J, et al. Pulmonary artery banding as 'open end' palliation of systemic right ventricles: An interim analysis. Eur J Cardiothorac Surg, 2012, 41(4): 913-918.
26.	Mongeon FP, Connolly HM, Dearani JA, et al. Congenitally corrected transposition of the great arteries ventricular function at the time of systemic atrioventricular valve replacement predicts long-term ventricular function. J Am Coll Cardiol, 2011, 57(20): 2008-2017.
27.	Quinn DW, McGuirk SP, Metha C, et al. The morphologic left ventricle that requires training by means of pulmonary artery banding before the double-switch procedure for congenitally corrected transposition of the great arteries is at risk of late dysfunction. J Thorac Cardiovasc Surg, 2008, 135(5): 1137-1144.
28.	Poirier NC, Mee RB. Left ventricular reconditioning and anatomical correction for systemic right ventricular dysfunction. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2000, 3: 198-215.
29.	Brawn WJ, Barron DJ, Jones TJ, et al. The fate of the retrained left ventricle after double switch procedure for congenitally corrected transposition of the great arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2008: 69-73.
30.	Ibrahimiye AN, Mainwaring RD, Patrick WL, et al. Left ventricular retraining and double switch in patients with congenitally corrected transposition of the great arteries. World J Pediatr Congenit Heart Surg, 2017, 8(2): 203-209.
31.	Huang SC. Commentary: Choosing complex biventricular repair versus Fontan-the unmet needs. J Thorac Cardiovasc Surg, 2019, 158(4): 1171-1172.
32.	Imamura M, Drummond-Webb JJ, Murphy DJ, et al. Results of the double switch operation in the current era. Ann Thorac Surg, 2000, 70(1): 100-105.
33.	Sharma R, Talwar S, Marwah A, et al. Anatomic repair for congenitally corrected transposition of the great arteries. J Thorac Cardiovasc Surg, 2009, 137(2): 404-412.
34.	Mavroudis C, Backer CL. Physiologic versus anatomic repair of congenitally corrected transposition of the great arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2003, 6: 16-26.

1. Connelly MS, Liu PP, Williams WG, et al. Congenitally corrected transposition of the great arteries in the adult: Functional status and complications. J Am Coll Cardiol, 1996, 27(5): 1238-1243.
2. Alghamdi AA, McCrindle BW, Van Arsdell GS. Physiologic versus anatomic repair of congenitally corrected transposition of the great arteries: Meta-analysis of individual patient data. Ann Thorac Surg, 2006, 81(4): 1529-1535.
3. Karl TR. The role of the Fontan operation in the treatment of congenitally corrected transposition of the great arteries. Ann Pediatr Cardiol, 2011, 4(2): 103-110.
4. Sano T, Riesenfeld T, Karl TR, et al. Intermediate-term outcome after intracardiac repair of associated cardiac defects in patients with atrioventricular and ventriculoarterial discordance. Circulation, 1995, 92(9 Suppl): Ⅱ272-Ⅱ278.
5. Hraska V, Duncan BW, Mayer JE, et al. Long-term outcome of surgically treated patients with corrected transposition of the great arteries. J Thorac Cardiovasc Surg, 2005, 129(1): 182-191.
6. Simmons MA, Rollinson N, Fishberger S, et al. Modern incidence of complete heart block in patients with L-looped ventricles: Does univentricular status matter? Congenit Heart Dis, 2015, 10(5): E237-E242.
7. De León LE, Mery CM, Verm RA, et al. Mid-term outcomes in patients with congenitally corrected transposition of the great arteries: A single center experience. J Am Coll Surg, 2017, 224(4): 707-715.
8. Hörer J, Schreiber C, Krane S, et al. Outcome after surgical repair/palliation of congenitally corrected transposition of the great arteries. Thorac Cardiovasc Surg, 2008, 56(7): 391-397.
9. Hsu KH, Chang CI, Huang SC, et al. 17-year experience in surgical management of congenitally corrected transposition of the great arteries: A single-centre's experience. Eur J Cardiothorac Surg, 2016, 49(2): 522-527.
10. Shin'oka T, Kurosawa H, Imai Y, et al. Outcomes of definitive surgical repair for congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections: Risk analyses in 189 patients. J Thorac Cardiovasc Surg, 2007, 133(5): 1318-1328.
11. Talwar S, Bansal A, Choudhary SK, et al. Results of Fontan operation in patients with congenitally corrected transposition of great arteries. Interact Cardiovasc Thorac Surg, 2016, 22(2): 188-193.
12. Spigel Z, Binsalamah ZM, Caldarone C. Congenitally corrected transposition of the great arteries: Anatomic, physiologic repair, and palliation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2019, 22: 32-42.
13. 肖旭文, 曲江波, 石慧, 等. 儿童先天性心脏病手术后运动耐量的评价. 临床小儿外科杂志, 2021, 20(6): 538-544.
14. Cooper DM, Weiler-Ravell D, Whipp BJ, et al. Aerobic parameters of exercise as a function of body size during growth in children. J Appl Physiol Respir Environ Exerc Physiol, 1984, 56(3): 628-634.
15. Qu J, Shi H, Chen X, et al. Evaluation of physical fitness in children with congenital heart diseases versus healthy population. Semin Thorac Cardiovasc Surg, 2020, 32(4): 906-915.
16. Graham TP, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: A multi-institutional study. J Am Coll Cardiol, 2000, 36(1): 255-261.
17. Prieto LR, Hordof AJ, Secic M, et al. Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great arteries. Circulation, 1998, 98(10): 997-1005.
18. Graham TP, Parrish MD, Boucek RJ, et al. Assessment of ventricular size and function in congenitally corrected transposition of the great arteries. Am J Cardiol, 1983, 51(2): 244-251.
19. Bautista-Hernandez V, Marx GR, Gauvreau K, et al. Determinants of left ventricular dysfunction after anatomic repair of congenitally corrected transposition of the great arteries. Ann Thorac Surg, 2006, 82(6): 2059-2065.
20. Hiramatsu T, Matsumura G, Konuma T, et al. Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries. Eur J Cardiothorac Surg, 2012, 42(6): 1004-1008.
21. Murtuza B, Barron DJ, Stumper O, et al. Anatomic repair for congenitally corrected transposition of the great arteries: A single-institution 19-year experience. J Thorac Cardiovasc Surg, 2011, 142(6): 1348-1357.
22. Lenoir M, Bouhout I, Gaudin R, et al. Outcomes of the anatomical repair in patients with congenitally corrected transposition of the great arteries: Lessons learned in a high-volume centre. Eur J Cardiothorac Surg, 2018, 54(3): 532-538.
23. DiBardino DJ, Heinle JS, Fraser CD. The hemi-Mustard, bi-directional Glenn, and Rastelli operations used for correction of congenitally corrected transposition, achieving a "ventricle and a half " repair. Cardiol Young, 2004, 14(3): 330-332.
24. Malhotra SP, Reddy VM, Qiu M, et al. The hemi-Mustard/bidirectional Glenn atrial switch procedure in the double-switch operation for congenitally corrected transposition of the great arteries: Rationale and midterm results. J Thorac Cardiovasc Surg, 2011, 141(1): 162-170.
25. Cools B, Brown SC, Louw J, et al. Pulmonary artery banding as 'open end' palliation of systemic right ventricles: An interim analysis. Eur J Cardiothorac Surg, 2012, 41(4): 913-918.
26. Mongeon FP, Connolly HM, Dearani JA, et al. Congenitally corrected transposition of the great arteries ventricular function at the time of systemic atrioventricular valve replacement predicts long-term ventricular function. J Am Coll Cardiol, 2011, 57(20): 2008-2017.
27. Quinn DW, McGuirk SP, Metha C, et al. The morphologic left ventricle that requires training by means of pulmonary artery banding before the double-switch procedure for congenitally corrected transposition of the great arteries is at risk of late dysfunction. J Thorac Cardiovasc Surg, 2008, 135(5): 1137-1144.
28. Poirier NC, Mee RB. Left ventricular reconditioning and anatomical correction for systemic right ventricular dysfunction. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2000, 3: 198-215.
29. Brawn WJ, Barron DJ, Jones TJ, et al. The fate of the retrained left ventricle after double switch procedure for congenitally corrected transposition of the great arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2008: 69-73.
30. Ibrahimiye AN, Mainwaring RD, Patrick WL, et al. Left ventricular retraining and double switch in patients with congenitally corrected transposition of the great arteries. World J Pediatr Congenit Heart Surg, 2017, 8(2): 203-209.
31. Huang SC. Commentary: Choosing complex biventricular repair versus Fontan-the unmet needs. J Thorac Cardiovasc Surg, 2019, 158(4): 1171-1172.
32. Imamura M, Drummond-Webb JJ, Murphy DJ, et al. Results of the double switch operation in the current era. Ann Thorac Surg, 2000, 70(1): 100-105.
33. Sharma R, Talwar S, Marwah A, et al. Anatomic repair for congenitally corrected transposition of the great arteries. J Thorac Cardiovasc Surg, 2009, 137(2): 404-412.
34. Mavroudis C, Backer CL. Physiologic versus anatomic repair of congenitally corrected transposition of the great arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2003, 6: 16-26.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Early and mid-term results of Fontan operation versus anatomic correction for congenitally corrected transposition of the great arteries: A retrospective cohort study in a single center

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