References
1. Davies, B., et al., Unifocalization of major aortopulmonary
collateral arteries in pulmonary atresia with ventricular septal defect
is essential to achieve excellent outcomes irrespective of native
pulmonary artery morphology. J Thorac Cardiovasc Surg, 2009.138 (6): p. 1269-75 e1.
2. Malhotra, S.P. and F.L. Hanley, Surgical management of
pulmonary atresia with ventricular septal defect and major
aortopulmonary collaterals: a protocol-based approach. Semin Thorac
Cardiovasc Surg Pediatr Card Surg Annu, 2009: p. 145-51.
3. Ishibashi, N., et al., Clinical results of staged repair with
complete unifocalization for pulmonary atresia with ventricular septal
defect and major aortopulmonary collateral arteries. Eur J Cardiothorac
Surg, 2007. 32 (2): p. 202-8.
4. Abella, R.F., et al., Primary repair of pulmonary atresia with
ventricular septal defect and major aortopulmonary collaterals: a useful
approach. J Thorac Cardiovasc Surg, 2004. 127 (1): p. 193-202.
5. Duncan, B.W., et al., Staged repair of tetralogy of Fallot with
pulmonary atresia and major aortopulmonary collateral arteries. J
Thorac Cardiovasc Surg, 2003. 126 (3): p. 694-702.
6. Hofferberth, S.C., et al., Pulmonary atresia with ventricular
septal defect and major aortopulmonary collaterals: collateral vessel
disease burden and unifocalisation strategies. Cardiol Young, 2018.28 (9): p. 1091-1098.
7. Brizard, C.P., M. Liava’a, and Y. d’Udekem, Pulmonary atresia,
VSD and Mapcas: repair without unifocalization. Semin Thorac Cardiovasc
Surg Pediatr Card Surg Annu, 2009: p. 139-44.
8. Sawatari, K., et al., Staged operation for pulmonary atresia
and ventricular septal defect with major aortopulmonary collateral
arteries. New technique for complete unifocalization. J Thorac
Cardiovasc Surg, 1989. 98 (5 Pt 1): p. 738-50.
9. Nakata, S., et al., A new method for the quantitative
standardization of cross-sectional areas of the pulmonary arteries in
congenital heart diseases with decreased pulmonary blood flow. J Thorac
Cardiovasc Surg, 1984. 88 (4): p. 610-9.
10. DeRuiter, M.C., et al., The restricted surgical relevance of
morphologic criteria to classify systemic-pulmonary collateral arteries
in pulmonary atresia with ventricular septal defect. J Thorac
Cardiovasc Surg, 1994. 108 (4): p. 692-9.
11. DeRuiter, M.C., et al., Development of the pharyngeal arch
system related to the pulmonary and bronchial vessels in the avian
embryo. With a concept on systemic-pulmonary collateral artery
formation. Circulation, 1993. 87 (4): p. 1306-19.
12. Berger, R.M., et al., Heparin as a risk factor for perigraft
seroma complicating the modified Blalock-Taussig shunt. J Thorac
Cardiovasc Surg, 1998. 116 (2): p. 286-92; discussion 292-3.
13. Mei, J., et al., A novel two-stage complete repair method for
pulmonary atresia with ventricular septal defect and major
aortopulmonary collateral arteries. Chin Med J (Engl). 123 (3):
p. 259-64.
14. Watanabe, N., et al., Early complete repair of pulmonary
atresia with ventricular septal defect and major aortopulmonary
collaterals. Ann Thorac Surg, 2014. 97 (3): p. 909-15;
discussion 914-5.
15. Fouilloux, V., et al., Management of patients with pulmonary
atresia, ventricular septal defect, hypoplastic pulmonary arteries and
major aorto-pulmonary collaterals: Focus on the strategy of
rehabilitation of the native pulmonary arteries. Arch Cardiovasc Dis,
2012. 105 (12): p. 666-75.
16. Reddy, V.M., J.R. Liddicoat, and F.L. Hanley, Midline
one-stage complete unifocalization and repair of pulmonary atresia with
ventricular septal defect and major aortopulmonary collaterals. J
Thorac Cardiovasc Surg, 1995. 109 (5): p. 832-44; discussion
844-5.
17. Dragulescu, A., et al., Long-term results of pulmonary artery
rehabilitation in patients with pulmonary atresia, ventricular septal
defect, pulmonary artery hypoplasia, and major aortopulmonary
collaterals. J Thorac Cardiovasc Surg, 2011. 142 (6): p.
1374-80.
18. Mainwaring, R.D., et al., Late outcomes in patients undergoing
aortopulmonary window for pulmonary atresia/stenosis and major
aortopulmonary collaterals. Ann Thorac Surg, 2012. 94 (3): p.
842-8.
19. Barron, D.J., et al., Unifocalization cannot rely exclusively
on native pulmonary arteries: the importance of recruitment of major
aortopulmonary collaterals in 249 casesdagger. Eur J Cardiothorac Surg,
2019. 56 (4): p. 679-687.
20. Barron, D.J. and P. Botha, Approaches to Pulmonary Atresia
With Major Aortopulmonary Collateral Arteries. Semin Thorac Cardiovasc
Surg Pediatr Card Surg Annu, 2018. 21 : p. 64-74.
21. Carotti, A., Surgical Management of Fallot’s Tetralogy With
Pulmonary Atresia and Major Aortopulmonary Collateral Arteries:
Multistage Versus One-Stage Repair. World J Pediatr Congenit Heart
Surg, 2020. 11 (1): p. 34-38.
22. Metras, D., et al., Pulmonary atresia with ventricular septal
defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary
collaterals. Eur J Cardiothorac Surg, 2001. 20 (3): p. 590-6;
discussion 596-7.
23. de Jong, P.L., et al., The effect of a central aortopulmonary
shunt in staged repair of pulmonary atresia, ventricular septal defect
and systemic-pulmonary collateral arteries. CVE, 1999. 4 (1):
p. 27-29.
24. Fang, M., et al., Development of pulmonary arteries after a
central end-to-side shunt in patients with pulmonary atresia,
ventricular septal defect, and diminutive pulmonary arteries. Thorac
Cardiovasc Surg, 2014. 62 (3): p. 211-5.
25. Fan, F., et al., Systemic-to-pulmonary shunt vs right
ventricle to pulmonary artery connection in the treatment of pulmonary
atresia, ventricular septal defect, and major aortopulmonary collateral
arteries. J Card Surg, 2020. 35 (2): p. 345-351.
26. Soquet, J., et al., Achievements and Limitations of a Strategy
of Rehabilitation of Native Pulmonary Vessels in Pulmonary Atresia,
Ventricular Septal Defect, and Major Aortopulmonary Collateral
Arteries. Ann Thorac Surg, 2017. 103 (5): p. 1519-1526.
27. Soquet, J., D.J. Barron, and Y. d’Udekem, A Review of the
Management of Pulmonary Atresia, Ventricular Septal Defect, and Major
Aortopulmonary Collateral Arteries. Ann Thorac Surg, 2019.108 (2): p. 601-612.
28. Mainwaring, R.D., et al., Fate of right ventricle to pulmonary
artery conduits after complete repair of pulmonary atresia and major
aortopulmonary collaterals. Ann Thorac Surg, 2015. 99 (5): p.
1685-91.
29. van de Woestijne, P.C., et al., Right ventricular outflow
tract reconstruction with an allograft conduit in patients after
tetralogy of Fallot correction: long-term follow-up. Ann Thorac Surg,
2011. 92 (1): p. 161-6.
30. Kauw, D., et al., 22q11.2 deletion syndrome is associated with
increased mortality in adults with tetralogy of Fallot and pulmonary
atresia with ventricular septal defect. Int J Cardiol, 2020.306 : p. 56-60.
Figure Legends
Figure 1
Flowchart of our patient population. Subsequent series of patients
following the staged protocol of unifocalization and correction for
pulmonary atresia, ventricular septal defect and systemic-pulmonary
collateral arteries.
Figure 2
Kaplan-Meier curve of the survival after complete correction.
Figure 3
Kaplan-Meier curve of the freedom from pulmonary valve replacement after
correction.
Table 1