Skip to main content

Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort

Pediatric Cardiology (2022)Cite this article

Abstract

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009–2019 in 19 United States centers were reviewed. Competing risks analysis was performed to determine cumulative risk of each end-state, and multivariable regression analyses were performed to identify factors associated with each end-state and transplant-free survival. We reviewed 295 patients. Median tricuspid valve Z-score was − 3.06 (25%, 75%: − 4.00, − 1.52). Final end-state was biventricular repair for 45 patients (15.2%), one-and-a half ventricle for 16 (5.4%), Fontan for 75 (25.4%), cardiac transplantation for 29 (9.8%), and death for 54 (18.3%). Seventy-six patients (25.7%) remained in mixed circulation. Cumulative risk estimate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1, 6 months, 1 year, and 5 years, respectively. Tricuspid valve Z-score was inversely, and coronary atresia positively associated with death or transplantation [odds ratio (OR) = 0.46, (95% confidence interval (CI) = 0.29–0.75, p < 0.001) and OR = 3.75 (95% CI 1.46–9.61, p = 0.011), respectively]. Right ventricular coronary dependency and left coronary atresia had a significant effect on transplant-free survival (log-rank p < 0.001). In a contemporary multicenter cohort of patients with PAIVS, consisting predominantly of patients with moderate-to-severe right ventricular hypoplasia, we observed favorable survival outcomes. Right ventricular coronary dependency and left, but not right, coronary atresia significantly worsens transplant-free survival.

This is a preview of subscription content, access via your institution.

Access options

Buy single article

Instant access to the full article PDF.

USD 39.95

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

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

References

  1. Daubeney PEF, Delany DJ, Anderson RH et al (2002) Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study. J Am Coll Cardiol 39(10):1670–1679. https://doi.org/10.1016/S0735-1097(02)01832-6

    Article  PubMed  Google Scholar 

  2. Giglia TM, Jenkins KJ, Matitiau A et al (1993) Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum. Circulation 88(5 Pt 1):2248–2256. https://doi.org/10.1161/01.CIR.88.5.2248

    CAS  Article  PubMed  Google Scholar 

  3. Zuberbuhler JR, Anderson RH (1979) Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J 41:281–288

    CAS  Article  Google Scholar 

  4. Calder AL, Sage MD (1987) Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol 59(5):436–442. https://doi.org/10.1016/0002-9149(87)90952-0

    CAS  Article  PubMed  Google Scholar 

  5. Gentles TL, Colan SD, Giglia TM, Mandell VS, Mayer JE, Sanders SP (1993) Right ventricular decompression and left ventricular function in pulmonary atresia with intact ventricular septum. The influence of less extensive coronary anomalies. Circulation 88(5 Pt 2):183–188

    Google Scholar 

  6. Giglia TM, Mandell VS, Connor AR, Mayer JE, Lock JE (1992) Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 86(5):1516–1528. https://doi.org/10.1161/01.cir.86.5.1516

    CAS  Article  PubMed  Google Scholar 

  7. Ashburn DA, Blackstone EH, Wells WJ et al (2004) Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 127(4):1000–1008. https://doi.org/10.1016/J.JTCVS.2003.11.057

    Article  PubMed  Google Scholar 

  8. Jahangiri M, Zurakowski D, Bichell D et al (1999) Improved results with selective management in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 118(6):1046–1052. https://doi.org/10.1016/S0022-5223(99)70100-5

    CAS  Article  PubMed  Google Scholar 

  9. Hannan RL, Zabinsky JA, Stanfill RM et al (2009) Midterm results for collaborative treatment of pulmonary atresia with intact ventricular septum. Ann Thorac Surg 87(4):1227–1233. https://doi.org/10.1016/J.ATHORACSUR.2009.01.040

    Article  PubMed  Google Scholar 

  10. Dyamenahalli U, McCrindle BW, McDonald C et al (2004) Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients. Cardiol Young 14(3):299–308. https://doi.org/10.1017/S1047951104003087

    Article  PubMed  Google Scholar 

  11. Daubeney PEF, Wang D, Delany DJ et al (2005) Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study. J Thorac Cardiovasc Surg 130(4):1071.e1-1071.e9. https://doi.org/10.1016/J.JTCVS.2005.05.044

    Article  Google Scholar 

  12. Wright LK, Knight JH, Thomas AS, Oster ME, St Louis JD, Kochilas LK (2019) Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum. Heart (British Cardiac Society) 105(13):1007–1013. https://doi.org/10.1136/HEARTJNL-2018-314124

    Article  Google Scholar 

  13. Barron DJ, Vanderlaan RD (2021) Commentary: despite best intentions: developing better strategies for patients with pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg. https://doi.org/10.1016/J.JTCVS.2021.12.038

    Article  PubMed  Google Scholar 

  14. Liavaa M, Brooks P, Konstantinov I, Brizard C, Dudekem Y (2011) Changing trends in the management of pulmonary atresia with intact ventricular septum: the Melbourne experience. Eur J Cardio-Thorac Surg 40(6):1406–1411. https://doi.org/10.1016/J.EJCTS.2011.02.036

    Article  Google Scholar 

  15. Boston Children’s Hospital Heart Center. Z-score calculator. Available at https://zscore.chboston.org/. Accessed on 15 Jul 2021

  16. Gray RJ (1988) A class of K-sample tests for comparing the cumulative incidence of a competing risk. Ann Stat 16:1141–1154

    Article  Google Scholar 

  17. Bob Gray (2020) cmprsk: subdistribution analysis of competing risks. R package version 2.2-10. Available at https://CRAN.R-project.org/package=cmprsk

  18. Harrell Jr. FE (2020) rms: regression modeling strategies. R package version 6.0-0. Available at https://CRAN.R-project.org/package=rms

  19. R Core Team (2019) R: a language and environment for statistical computing. R Foundation for Statistical Computing, Vienna

    Google Scholar 

  20. Pawade A, Capuani A, Penny DJ, Karl TR, Mee RBB (1993) Pulmonary atresia with intact ventricular septum: surgical management based on right ventricular infundibulum. J Card Surg 8(3):371–383. https://doi.org/10.1111/J.1540-8191.1993.TB00379.X

    CAS  Article  PubMed  Google Scholar 

  21. Hanley FL, Sade RM, Freedom RM, Blackstone EH, Kirklin JW, The Congenital Heart Surgeons Society (1993) Outcomes in critically ill neonates with pulmonary stenosis and intact ventricular septum: a multiinstitutional study. J Am Coll Cardiol 22(1):183–192. https://doi.org/10.1016/0735-1097(93)90833-M

    CAS  Article  PubMed  Google Scholar 

  22. Awori MN, Mehta NP, Mitema FO, Kebba N (2017) Optimal Z-score use in surgical decision-making in pulmonary atresia with intact ventricular septum. World J Pediatr Congenit Heart Surg 8(3):385–388. https://doi.org/10.1177/2150135117701407

    Article  PubMed  Google Scholar 

  23. Guleserian KJ, Armsby LB, Thiagarajan RR, del Nido PJ, Mayer JE (2006) Natural history of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach. Ann Thorac Surg 81(6):2250–2258. https://doi.org/10.1016/J.ATHORACSUR.2005.11.041

    Article  PubMed  Google Scholar 

  24. Cheung EW, Richmond ME, Turner ME, Bacha EA, Torres AJ (2014) Pulmonary atresia/intact ventricular septum: influence of coronary anatomy on single-ventricle outcome. Ann Thorac Surg 98(4):1371–1377. https://doi.org/10.1016/J.ATHORACSUR.2014.06.039

    Article  PubMed  Google Scholar 

  25. Elias P, Poh CL, du Plessis K et al (2018) Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death. Eur J Cardio-Thorac Surg 53(6):1230–1236. https://doi.org/10.1093/EJCTS/EZY038

    Article  Google Scholar 

Download references

Acknowledgements

The authors acknowledge the staff of the Heart Institute Research Core at Cincinnati Children’s Hospital Medical Center for the regulatory, data management and statistical support provided. The authors also acknowledge Dr. Andrew Redington, Heart Institute Co-Director, Cincinnati Children’s Hospital Medical Center for critically appraising the manuscript.

Funding

This study received intramural funding from Heart Institute Research Core at Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA.

Author information

Authors and Affiliations

  1. The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Ilias Iliopoulos & Huaiyu Zang

  2. Division of Critical Care, Department of Pediatrics, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, IN, USA

    Christopher W. Mastropietro

  3. Department of Pediatrics, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, USA

    Saul Flores

  4. Department of Pediatrics, Columbia University College of Physicians and Surgeons, Morgan Stanley Children’s Hospital of New York, New York, NY, USA

    Eva Cheung

  5. Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA

    Venugopal Amula

  6. Division of Critical Care, Department of Pediatrics, University of Washington, Seattle Children’s Hospital, Seattle, WA, USA

    Monique Radman

  7. Division of Cardiology, Department of Pediatrics, Stanford University School of Medicine, Lucille Packard Children’s Hospital, Palo Alto, CA, USA

    David Kwiatkowski

  8. Division of Cardiac Critical Care, Children’s National Health System, Washington, DC, USA

    Bao Nguyen Puente

  9. Division of Cardiology, Department of Pediatrics, Shawn Jenkins Children’s Hospital, Medical University of South Carolina, Charleston, SC, USA

    Jason R. Buckley & John M. Costello

  10. Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Kiona Y. Allen

  11. Department of Pediatrics, Chicago Medical School, Advocate Children’s Hospital, Chicago, IL, USA

    Rohit Loomba

  12. Division of Cardiology, Department of Pediatrics, University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, TN, USA

    Karan B. Karki

  13. Division of Pediatric Critical Care, Department of Pediatrics, Saint Louis University, Cardinal Glennon Children’s Hospital, Saint Louis, MO, USA

    Saurabh Chiwane

  14. Department of Pediatrics, Central Michigan University, Children’s Hospital of Michigan, Detroit, MI, USA

    Katherine Cashen

  15. Division of Critical Care, LSU Health Sciences, Children’s Hospital, New Orleans, LA, USA

    Kurt Piggott

  16. Department of Pediatrics, Children’s Hospital of Illinois, Peoria, IL, USA

    Yamini Kapileshwarkar

  17. Department of Pediatrics, Cleveland Clinic, Cleveland, OH, USA

    Keshava Murty Narayana Gowda

  18. Department of Pediatrics, University of Iowa Stead Family Children’s Hospital, Iowa City, IA, USA

    Aditya Badheka

  19. Department of Pediatrics, Mercy Medical Center, Des Moines, IA, USA

    Rahul Raman

  20. Cardiac Intensive Care, The Heart Institute, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45229, USA

    Ilias Iliopoulos

Authors
  1. Ilias Iliopoulos
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Christopher W. Mastropietro
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Saul Flores
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Eva Cheung
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Venugopal Amula
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Monique Radman
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. David Kwiatkowski
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Bao Nguyen Puente
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Jason R. Buckley
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Kiona Y. Allen
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Rohit Loomba
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Karan B. Karki
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Saurabh Chiwane
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Katherine Cashen
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Kurt Piggott
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Yamini Kapileshwarkar
    View author publications

    You can also search for this author in PubMed Google Scholar

  17. Keshava Murty Narayana Gowda
    View author publications

    You can also search for this author in PubMed Google Scholar

  18. Aditya Badheka
    View author publications

    You can also search for this author in PubMed Google Scholar

  19. Rahul Raman
    View author publications

    You can also search for this author in PubMed Google Scholar

  20. Huaiyu Zang
    View author publications

    You can also search for this author in PubMed Google Scholar

  21. John M. Costello
    View author publications

    You can also search for this author in PubMed Google Scholar

Consortia

The Collaborative Research from the Pediatric Cardiac Intensive Care Society Investigators

Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by II and HZ. The first draft of the manuscript was written by II and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Ilias Iliopoulos.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest to disclose.

Ethical Approval

The study was performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments.

Additional information

Publisher's Note

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

Supplementary Information

Below is the link to the electronic supplementary material.

Supplementary file1 (DOCX 47 kb)

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Iliopoulos, I., Mastropietro, C.W., Flores, S. et al. Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort. Pediatr Cardiol (2022). https://doi.org/10.1007/s00246-022-02954-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s00246-022-02954-5

Keywords

  • Pulmonary atresia
  • Congenital heart surgery
  • Heart defect
  • Postoperative outcomes
  • Pediatric cardiology