Skip to main content
SpringerLink
Log in

Pulmonary Atresia, Ventricular Septal Defect and Major Aorto-Pulmonary Collateral Arteries

  • Chapter
  • First Online:
Book cover Surgery of Conotruncal Anomalies

  • 1121 Accesses

Abstract

The term pulmonary atresia and ventricular septal defect (VSD) covers a spectrum of conditions with various degrees of severity. The terminology of pulmonary atresia, VSD and major aortopulmonary collateral arteries (MAPCAs) is usually restricted to patients with diminutive central pulmonary arteries whose pulmonary circulation is dependent from systemic-pulmonary collateral arteries. It therefore excludes patients with ductus-dependent pulmonary circulation. The natural history of the condition is severe. In the 90s, only a fifth of the patients born with pulmonary atresia, VSD and MAPCAs survived to 30 years of age.

Significant progress has been made in the management of patients with pulmonary atresia, VSD and MAPCAs. Our understanding of the variations in pulmonary vasculature has evolved, although there remains debate about the best methods of classification. There is an ongoing debate on optimal surgical strategies between surgical rehabilitation of the native hypoplastic PA that our group favors and unifocalisation of MAPCAs early in infancy. There continues to be debate on the most effective strategy to provide long-term survival in this patient group.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 249.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.00
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Sullivan ID, Wren C, Stark J, de Leval MR, Macartney FJ, Deanfield JE. Surgical unifocalization in pulmonary atresia and ventricular septal defect. A realistic goal? Circulation. 1988;78:III5–13.

    Article  CAS  PubMed  Google Scholar 

  2. Iyer KS, Mee RB. Staged repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals. Ann Thorac Surg. 1991;51:65–72.

    Article  CAS  PubMed  Google Scholar 

  3. Reddy VM, Liddicoat JR, Hanley FL. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg. 1995;109:832–44; discussion 844–5.

    Article  CAS  PubMed  Google Scholar 

  4. d’Udekem Y, Alphonso N, Nørgaard MA, Cochrane AD, Grigg LE, Wilkinson JL, Brizard CP. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: unifocalization brings no long-term benefits. J Thorac Cardiovasc Surg. 2005;130:1496–502.

    Article  PubMed  Google Scholar 

  5. Song S-W, Park HK, Park Y-H, Cho BK. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries. Circ J. 2009;73:516–22.

    Article  PubMed  Google Scholar 

  6. Nørgaard MA, Alphonso N, Cochrane AD, Menahem S, Brizard CP, d’Udekem Y. Major aorto-pulmonary collateral arteries of patients with pulmonary atresia and ventricular septal defect are dilated bronchial arteries. Eur J Cardiothorac Surg. 2006;29:653–8.

    Article  PubMed  Google Scholar 

  7. Brawn WJ, Jones T, Davies B, Barron D. How we manage patients with major aorta pulmonary collaterals. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009;12:152–7.

    Google Scholar 

  8. Malhotra SP, Hanley FL. 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;12:145–51.

    Google Scholar 

  9. Brizard CP, Liava’a M, d’Udekem Y. Pulmonary atresia, VSD and MAPCAs: repair without unifocalization. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009;12:139–44.

    Google Scholar 

  10. Ishibashi N, Shin’oka T, Ishiyama M, Sakamoto T, Kurosawa H. 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;32:202–8.

    Article  PubMed  Google Scholar 

  11. Carotti A, Albanese SB, Filippelli S, Ravà L, Guccione P, Pongiglione G, Di Donato RM. Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2010;140:1092–103.

    Article  PubMed  Google Scholar 

  12. Liava’a M, Brizard CP, Konstantinov IE, Robertson T, Cheung MM, Weintraub R, d’Udekem Y. Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals: neonatal pulmonary artery rehabilitation without unifocalization. ATS Elsevier Inc. 2012;93:185–91.

    Google Scholar 

  13. Metras D, Chetaille P, Kreitmann B, Fraisse A, Ghez O, Riberi A. Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals. Eur J Cardiothorac Surg. 2001;20:590–6; discussion 596–7.

    Article  CAS  PubMed  Google Scholar 

  14. Reddy VM, McElhinney DB, Amin Z, Moore P, Parry AJ, Teitel DF, Hanley FL. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation. 2000;101:1826–32.

    Article  CAS  PubMed  Google Scholar 

  15. Griselli M, McGuirk SP, Winlaw DS, Stümper O, De Giovanni JV, Miller P, Dhillon R, Wright JG, Barron DJ, Brawn WJ. The influence of pulmonary artery morphology on the results of operations for major aortopulmonary collateral arteries and complex congenital heart defects. J Thorac Cardiovasc Surg. 2004;127:251–8.

    Article  PubMed  Google Scholar 

  16. Mainwaring RD, Reddy VM, Perry SB, Peng L, Hanley FL. Late outcomes in patients undergoing aortopulmonary window for pulmonary atresia/stenosis and major aortopulmonary collaterals. Ann Thorac Surg. 2012;94:842–8.

    Article  PubMed  Google Scholar 

  17. Watanabe N, Mainwaring RD, Reddy VM, Palmon M, Hanley FL. Early complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. Ann Thorac Surg. 2014;97:909–15; discussion 914–5.

    Article  PubMed  Google Scholar 

  18. Fouilloux V, Bonello B, Kammache I, Fraisse A, Macé L, Kreitmann B. 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;105:666–75.

    Article  PubMed  Google Scholar 

  19. Grosse-Wortmann L, Yoo S-J, van Arsdell G, Chetan D, MacDonald C, Benson L, Honjo O. Preoperative total pulmonary blood flow predicts right ventricular pressure in patients early after complete repair of tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2013;146:1185–90.

    Article  PubMed  Google Scholar 

  20. Ben D, Mussa S, Davies P, Stickley J, Jones TJ, Barron DJ, Brawn WJ. 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;138:1269–75.

    Article  Google Scholar 

  21. Reddy VM, Petrossian E, McElhinney DB, Moore P, Teitel DF, Hanley FL. One-stage complete unifocalization in infants: when should the ventricular septal defect be closed? J Thorac Cardiovasc Surg. 1997;113:858–66; discussion 866-8.

    Article  CAS  PubMed  Google Scholar 

  22. Honjo O, Al-Radi OO, MacDonald C, Tran K-CD, Sapra P, Davey LD, Chaturvedi RR, Caldarone CA, Van Arsdell GS. The functional intraoperative pulmonary blood flow study is a more sensitive predictor than preoperative anatomy for right ventricular pressure and physiologic tolerance of ventricular septal defect closure after complete unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. Circulation. 2009;120:S46–52.

    Article  PubMed  Google Scholar 

  23. Schulze-Neick I, Ho SY, Bush A, Rosenthal M, Franklin RC, Redington AN, Penny DJ. Severe airflow limitation after the unifocalization procedure: clinical and morphological correlates. Circulation. 2000;102:III142–7.

    Article  CAS  PubMed  Google Scholar 

  24. Bull K, Somerville J, Ty E, Spiegelhalter D. Presentation and attrition in complex pulmonary atresia. J Am Chem Soc. 1995;25:491–9.

    CAS  Google Scholar 

  25. Mainwaring RD, Reddy VM, Peng L, Kuan C, Palmon M, Hanley FL. Hemodynamic assessment after complete repair of pulmonary atresia with major aortopulmonary collaterals. Ann Thorac Surg. 2013;95:1397–402. 24.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Cardiac Surgery, Royal Children’s Hospital, Flemington Road, Parkville, Melbourne, VIC, 3052, Australia

    Yves d’Udekem MD, PhD, FRACS

  2. Department of Cardiology, Royal Children’s Hospital, Flemington Road, Parkville, Melbourne, VIC, 3052, Australia

    Lucas Jon Eastaugh MBBS, FRACP, FCSANZ

Authors
  1. Yves d’Udekem MD, PhD, FRACS
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lucas Jon Eastaugh MBBS, FRACP, FCSANZ
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Yves d’Udekem MD, PhD, FRACS .

Editor information

Editors and Affiliations

  1. Royal Brompton Hospital, London, United Kingdom

    Francois Lacour-Gayet

  2. C.S. Mott Children’s Hospital, University of Michigan, Ann Arbor, Michigan, USA

    Edward L. Bove

  3. German Pediatric Heart Center, Asklepios Klinik Sankt Augustin Gmb, Sankt Augustin, Germany

    Viktor Hraška

  4. University of Pittsburgh Medical School, Pittsburgh, Pennsylvania, USA

    Victor O. Morell

  5. Department of Cardiothoracic Surgery, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

    Thomas L. Spray

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

d’Udekem, Y., Eastaugh, L.J. (2016). Pulmonary Atresia, Ventricular Septal Defect and Major Aorto-Pulmonary Collateral Arteries. In: Lacour-Gayet, F., Bove, E., Hraška, V., Morell, V., Spray, T. (eds) Surgery of Conotruncal Anomalies. Springer, Cham. https://doi.org/10.1007/978-3-319-23057-3_8

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-23057-3_8

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-23056-6

  • Online ISBN: 978-3-319-23057-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation