Skip to main content

Advertisement

SpringerLink
Log in

Ventricular septal defect with pulmonary atresia: approaches, results, prognosticators and current status

  • Review Article
  • Published:
Indian Journal of Thoracic and Cardiovascular Surgery Aims and scope Submit manuscript

A Correspondence to this article was published on 14 June 2022

Abstract

Ventricular septal defect with pulmonary atresia and major aortopulmonary collateral arteries is a complex congenital cardiac anomaly with a wide spectrum of anatomical variations. Akin to the same, the management options are also very diverse ranging from aggressive single-stage repair with unifocalisation to surgical palliation and/or staged repair and also heart transplant. There is no consensus on the best management option. This review aims at highlighting the various surgical options and proposing a management pathway suited for the subcontinent patients.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8

Similar content being viewed by others

References

  1. Barron DJ, Botha P. Approaches to pulmonary atresia with major aortopulmonary collateral arteries. Semi Thorac Cardiovasc Surg. 2018;21:64–74.

    Article  Google Scholar 

  2. Tchervenkov CI, Roy N. Congenital heart surgery nomenclature and database project: pulmonary atresia-ventricular septal defect. Ann Thorac Surg. 2000;69:97–105.

    Article  Google Scholar 

  3. Bauser-Heaton H, Borquez A, Han B, et al. Programmatic approach to management of tetralogy of Fallot with major aortopulmonary collateral arteries: a 15-year experience with 458 patients. Circ Cardiovasc Interv. 2017;10:e004952.

    Article  CAS  Google Scholar 

  4. Kriett JM, Jamieson SW. Transplantation for congenital heart disease with special observations on pulmonary atresia and ventricular septal defect. Prog Pediatr Cardiol. 1992;1:61–6.

    Article  Google Scholar 

  5. Januszewska K, Malec E, Juchem G, et al. Heart-lung transplantation in patients with pulmonary atresia and ventricular septal defect. J Thorac Cardiovasc Surg. 2009;138:738–43.

    Article  Google Scholar 

  6. 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;109:832–45.

    Article  CAS  Google Scholar 

  7. Brown RT, Fellows KE. Cervical collateral arteries in pulmonary atresia with ventricular septal defect. Am J Cardiol. 1988;62:1310–1.

  8. Amin Z, McElhinney DB, Reddy VM, et al. Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect. Ann Thorac Surg. 2000;70:119–23.

  9. Kirklin JW, Bargeron LM, Pacifico AD. The enlargement of small pulmonary arteries by preliminary palliative operations. Circulation. 1977;56:612–7.

    Article  CAS  Google Scholar 

  10. McGoon DC, Baird DK, Davis GD. Surgical management of large bronchial collateral arteries with pulmonary stenosis or atresia. Circulation. 1975;52:109–18.

  11. Gill CC, Moodie DS, McGoon DC. Staged surgical management of pulmonary atresia with diminutive pulmonary arteries. J Thorac Cardiovasc Surg. 1977;73:436–42.

  12. d’Udekem Y, Alphonso N, Norgaard MA, et al. Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: Unifocalisation brings no long-term benefits. J Thorac Cardiovasc Surg. 2005;130:1496–502.

    Article  Google Scholar 

  13. Soquet J. Liava’a M, Eastaugh L, 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;103:1519–26.

    Article  Google Scholar 

  14. 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  Google Scholar 

  15. Gupta A, Odim J, Levi D, Chang RK, Laks H. Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 104 patients. J Thorac Cardiovasc Surg. 2003;126:1746–52.

    Article  Google Scholar 

  16. Babliak OD, Mykychak YB, Motrechko OO, Yemets IM. Surgical treatment of pulmonary atresia with major aortopulmonary collateral arteries in 83 consecutive patients. Eur J Cardiothorac Surg. 2017;52:96–104.

    Article  Google Scholar 

  17. Song SW, Park HK, Park YH, Cho BK. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries. Circ J. 2009;73:516–22.

  18. Dragulescu A, Kammache I, Fouilloux V, 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;142:1374–80.

  19. Chen Q, Ma K, Hua Z, et al. Multistage pulmonary artery rehabilitation in patients with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary artery. Eur J Cardiothorac Surg. 2016;50:160–6.

  20. Mainwaring RD, Patrick WL, Roth SJ, et al. Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg. 2018;156:1194–1204.

    Article  Google Scholar 

  21. Jia Q, Cen J, Zhuang J, et al. Significant survival advantage of high pulmonary vein index and the presence of native pulmonary artery in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: results from preoperative computed tomography angiography. Eur J Cardiothorac Surg. 2017;52:225–32.

    Article  Google Scholar 

  22. Murthy K, Reddy KP, Nagarajan R, Goutami V, Cherian K. Management of ventricular septal defect with pulmonary atresia and major aorto pulmonary collateral arteries: Challenges and controversies. Ann Pediatr Cardiol. 2010;3:127–35.

  23. Piehler JM, Danielson GK, McGoon DC, Wallace RB, Fulton RE, Mair DD. Management of pulmonary atresia with ventricular septal defect and hypoplastic pulmonary arteries by right ventricular outflow construction. J Thorac Cardiovasc Surg. 1980;80:552–67.

    Article  CAS  Google Scholar 

  24. Nakata S, Imai Y, Takanashi Y, 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;88:610–9.

    Article  CAS  Google Scholar 

  25. 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;113:858–68.

    Article  CAS  Google Scholar 

  26. Zhu J, Meza J, Kato A, et al. Pulmonary flow study predicts survival in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2016;152:1494–503.

Download references

Funding

None.

Author information

Authors and Affiliations

  1. Department of Cardiac Surgery, Alder Hey Children’s Hospital, East Prescot Road, Liverpool, Merseyside, L14 3AB, UK

    Ansh Garg

  2. Department of Congenital and Pediatric Cardiac Surgery, Indraprastha Apollo Hospital, New Delhi, India

    Rajesh Sharma

Authors
  1. Ansh Garg
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Rajesh Sharma
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ansh Garg.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Ethics committee approval

Not required being a review article.

Informed consent

Not required being a review article.

Human and animal rights statement

Not required being a review article. No animals were involved.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Garg, A., Sharma, R. Ventricular septal defect with pulmonary atresia: approaches, results, prognosticators and current status. Indian J Thorac Cardiovasc Surg 38, 28–37 (2022). https://doi.org/10.1007/s12055-020-01133-6

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12055-020-01133-6

Keywords

Search

Navigation