Skip to main content
SpringerLink
Log in

Importance of multidisciplinary management for pulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries and completely absent central pulmonary arteries

  • Original Article
  • Published:
General Thoracic and Cardiovascular Surgery Aims and scope Submit manuscript

Abstract

Background

To review long-term clinical outcomes for pulmonary atresia, ventricular septal defect (PA/VSD), major aorto-pulmonary collateral arteries (MAPCAs) and completely absent central pulmonary arteries (cPAs).

Methods

Of all 120 surgically treated patients with PA/VSD, MAPCAs between 1981 and 2011, 15 patients (12.5%) with completely absent cPAs were enrolled. The median age at initial surgery was 3.8 ± 4.9 years old (range 0.1–17.6) and 3.5 (2–6). Since 2003, consecutive five patients have been treated by current treatment strategy consisting of staged midline approach with percutaneous trans-catheter angioplasties (PTA), where complete unifocalization and right ventricle (RV)—PA conduit placement was performed via median sternotomy at first, then which was followed by VSD closure with one-way fenestrated patch and conduit replacement, after PTA for unifocalized MAPCAs, reconstructed cPAs, and RV-PA conduit. For patients survived after definitive repair, PTA was aggressively repeated to maintain RV systolic pressure.

Results

Median follow-up period was 7.8 years (0.3–21.7). Actuarial survival rate at 20 years after the initial surgical intervention was 27.5%, and all five patients treated by current treatment strategy survived to date. Of all 10 survivors after the definitive repair, serial catheter examination revealed that right to left ventricular systolic pressure ratio was maintained from 0.59 ± 0.09 (0.45–0.73) at early after the definitive repair to 0.57 ± 0.14 (0.35–0.81) at latest follow-up.

Conclusions

Staged midline approach with PTA seemed feasible strategy to go on to definitive repair. Patency of MAPCAs and reconstructed cPAs were maintained by the aggressive PTA after the definitive repair.

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

Similar content being viewed by others

Abbreviations

cPAs:

Central pulmonary arteries

MAPCA:

Major aorto-pulmonary collateral arteries

PA:

Pulmonary artery

PTA:

Percutaneous trans-catheter angioplasty

RV:

Right ventricle

VSD:

Ventricular septal defect

References

  1. Dragulescu A, Kammache I, Fouilloux V, Amedro P, Métras D, Kreitmann B, 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.

    Article  PubMed  Google Scholar 

  2. Liava’a M, Brizard CP, Konstantinov IE, Robertson T, Cheung MM, Weintraub R, et al. Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals: neonatal pulmonary artery rehabilitation without unifocalization. Ann Thorac Surg. 2012;93:185–91.

    Article  PubMed  Google Scholar 

  3. Zhang Y, Hua Z, Yang K, Zhang H, Yan J, Wang X, et al. Outcomes of the rehabilitative procedure for patients with pulmonary atresia, ventricular septal defect and hypoplastic pulmonary arteries beyond the infant period. Eur J Cardiothorac Surg. 2014;46:297–303.

    Article  PubMed  Google Scholar 

  4. Kim H, Sung SC, Choi KH, Lee HD, Ban GH, Chang YH. A central shunt to rehabilitate diminutive pulmonary arteries in patients with pulmonary atresia with ventricular septal defect. J Thorac Cardiovasc Surg. 2015;149:515–20.

    Article  PubMed  Google Scholar 

  5. d’Udekem Y, Alphonso N, Nørgaard MA, Cochrane AD, Grigg LE, Wilkinson JL, et al. 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 

  6. Yagihara T, Yamamoto F, Nishigaki K, Matsuki O, Uemura H, Isizaka T, et al. Unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 1996;112:392–402.

    Article  CAS  PubMed  Google Scholar 

  7. 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.

    Article  CAS  PubMed  Google Scholar 

  8. Shinkawa T, Yamagishi M, Shuntoh K, Takahashi A, Hayashida K, Kitamura N. One-stage unifocalization and palliative right ventricular outflow tract reconstruction. Ann Thorac Surg. 2005;79:1044–7.

    Article  PubMed  Google Scholar 

  9. 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 

  10. Davies B, Mussa S, Davies P, Stickley J, Jones TJ, Barron DJ, 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;138:1269–75.

    Article  PubMed  Google Scholar 

  11. Carotti A, Albanese SB, Filippelli S, Ravà L, Guccione P, Pongiglione G, et al. 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. Carrillo SA, Mainwaring RD, Patrick WL, Bauser-Heaton HD, Peng L, Reddy VM, et al. Surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals with absent intrapericardial pulmonary arteries. Ann Thorac Surg. 2015;100:606–14.

    Article  PubMed  Google Scholar 

  13. Gentles TL, Lock JE, Perry SB. High pressure balloon angioplasty for branch pulmonary artery stenosis: early experience. J Am Coll Cardiol. 1993;22:867–72.

    Article  CAS  PubMed  Google Scholar 

  14. Holzer RJ, Gauvreau K, Kreutzer J, Leahy R, Murphy J, Lock JE, et al. Balloon angioplasty and stenting of branch pulmonary arteries: adverse events and procedural characteristics: results of a multi-institutional registry. Circ Cardiovasc Interv. 2011;4:287–96.

    Article  PubMed  Google Scholar 

  15. Bergersen L, Gauvreau K, Justino H, Nugent A, Rome J, Kreutzer J, et al. Randomized trial ofcutting balloon compared with high-pressure angioplasty for the treatment of resistant pulmonary artery stenosis. Circulation. 2011;124:2388–96.

    Article  PubMed  Google Scholar 

  16. Maglione J, Bergersen L, Lock JE, McElhinney DB. Ultra-high-pressure balloon angioplasty for treatment of resistant stenoses within or adjacent to previously implanted pulmonary arterial stents. Circ Cardiovasc Interv. 2009;2:52–8.

    Article  PubMed  Google Scholar 

  17. 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.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, 5-7-1, Fujishiro-dai, Suita, Osaka, 565-8565, Japan

    Takaya Hoashi, Koji Kagisaki, Masatoshi Shimada & Hajime Ichikawa

  2. Department of Pediatric Cardiology, Sakakibara Heart Institute, Fuchu, Tokyo, Japan

    Satoshi Yazaki

  3. Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita, Japan

    Masataka Kitano & Isao Shiraishi

Authors
  1. Takaya Hoashi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Satoshi Yazaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Koji Kagisaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Masataka Kitano
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Masatoshi Shimada
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Isao Shiraishi
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Hajime Ichikawa
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Takaya Hoashi.

Ethics declarations

Conflict of interest

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The author(s) received no financial support for the research, authorship, and/or publication of this article.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hoashi, T., Yazaki, S., Kagisaki, K. et al. Importance of multidisciplinary management for pulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries and completely absent central pulmonary arteries. Gen Thorac Cardiovasc Surg 65, 337–342 (2017). https://doi.org/10.1007/s11748-017-0765-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11748-017-0765-1

Keywords

Search

Navigation