Skip to main content

Advertisement

SpringerLink
Log in

Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74–86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein’s anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein’s anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.

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

Similar content being viewed by others

References

  1. Paladini D, Volpe P, Marasini M et al (2006) Diagnosis, characterization and outcome of congenitally corrected transposition of the great arteries in the fetus: a multicenter series of 30 cases. Ultrasound Obstet Gynecol 27:281–285. https://doi.org/10.1002/uog.2715

    Article  CAS  PubMed  Google Scholar 

  2. Sharland G, Tingay R, Jones A, Simpson J (2005) Atrioventricular and ventriculoarterial discordance (congenitally corrected transposition of the great arteries): echocardiographic features, associations, and outcome in 34 fetuses. Heart 91:1453–1458. https://doi.org/10.1136/hrt.2004.052548

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Allan LD, Sharland GK, Milburn A et al (1994) Prospective diagnosis of 1,006 consecutive cases of congenital heart disease in the fetus. J Am Coll Cardiol 23:1452–1458. https://doi.org/10.1016/0735-1097(94)90391-3

    Article  CAS  PubMed  Google Scholar 

  4. Rutledge JM, Nihill MR, Fraser CD et al (2002) Outcome of 121 patients with congenitally corrected transposition of the great arteries. Pediatr Cardiol 23:137–145. https://doi.org/10.1007/s00246-001-0037-8

    Article  CAS  PubMed  Google Scholar 

  5. Chiappa E, Micheletti A, Sciarrone A et al (2004) The prenatal diagnosis of, and short-term outcome for, patients with congenitally corrected transposition. Cardiol Young 14:265–276

    Article  PubMed  Google Scholar 

  6. Wan AW, Jevremovic A, Seda E et al (2009) Comparison of impact of prenatal versus postnatal diagnosis of congenitally corrected transposition of the great arteries. Am J Cardiol 104:1276–1279. https://doi.org/10.1016/j.amjcard.2009.06.047

    Article  PubMed  Google Scholar 

  7. Egloff L, Rothlin M, Arbenz U et al (1980) Congenitally corrected transposition of the great arteries: a clinical and surgical study. Thorac Cardiovasc Surg 28:228–232

    Article  CAS  PubMed  Google Scholar 

  8. Graham TP, Bernard YD, Mellen BG et al (2000) Long-term outcome in congenitally corrected transposition of the great arteries. J Am Coll Cardiol 36:255–261. https://doi.org/10.1016/S0735-1097(00)00682-3

    Article  PubMed  Google Scholar 

  9. Lundstrom U, Bull C, Wyse RKH, Somerville J (1990) The natural and “unnatural” history of congenitally corrected transposition. Am J Cardiol 65:1222–1229. https://doi.org/10.1016/0002-9149(90)90978-A

    Article  CAS  PubMed  Google Scholar 

  10. Bjarke BB, Kidd BS (1976) Congenitally corrected transposition of the great arteries. A clinical study of 101 cases. Acta Paediatr Scand 65:153–160. https://doi.org/10.1177/1073191116659134

    Article  CAS  PubMed  Google Scholar 

  11. Cools B, Brown SC, Louw JJ et al (2012) Pulmonary artery banding as “open end” palliation of systemic right ventricles: an interim analysis. Eur J Cardio-thoracic Surg 41:913–918. https://doi.org/10.1093/ejcts/ezr078

    Article  Google Scholar 

  12. Kutty S, Danford DA, Diller GP, Tutarel O (2018) Contemporary management and outcomes in congenitally corrected transposition of the great arteries. Heart 104:1–8. https://doi.org/10.1136/heartjnl-2016-311032

    Article  Google Scholar 

  13. Orchard EA, Ormerod O, Myerson S, Westaby S (2010) Congenitally corrected transposition of the great arteries presenting in a nonagenarian. Circulation. https://doi.org/10.1161/CIRCULATIONAHA.109.919068

    Article  PubMed  Google Scholar 

  14. Matsumura Y, Kawahito K, Nakamura K, Hashimoto K (2013) Congenitally corrected transposition of the great arteries with coexisting aortic valve stenosis in a 77-year-old woman. Eur J Cardio-thoracic Surg 43:442. https://doi.org/10.1093/ejcts/ezs477

    Article  Google Scholar 

  15. Brizard CP, Lee A, Zannino D et al (2017) Long-term results of anatomic correction for congenitally corrected transposition of the great arteries: a 19-year experience. J Thorac Cardiovasc Surg 154:256–265.e4. https://doi.org/10.1016/j.jtcvs.2017.03.072

    Article  PubMed  Google Scholar 

  16. Hiramatsu T, Matsumura G, Konuma T et al (2012) Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries. Eur J Cardio-thoracic Surg 42:1004–1008. https://doi.org/10.1093/ejcts/ezs118

    Article  Google Scholar 

  17. Murtuza B, Barron DJ, Stumper O et al (2011) Anatomic repair for congenitally corrected transposition of the great arteries: a single-institution 19-year experience. J Thorac Cardiovasc Surg 142:1348–1357.e1. https://doi.org/10.1016/j.jtcvs.2011.08.016

    Article  PubMed  Google Scholar 

  18. Sharma R, Talwar S, Marwah A et al (2009) Anatomic repair for congenitally corrected transposition of the great arteries. J Thorac Cardiovasc Surg 137:404–412.e4. https://doi.org/10.1016/j.jtcvs.2008.09.048

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Fetal Cardiology Unit, Department of Congenital Heart Disease, Evelina London Children’s Healthcare, Guy’s and St Thomas’ NHS Foundation Trust, Westminster Bridge Road, London, UK

    Thomas G. Day, Tomas Woodgate, Olatejumoye Knee, Vita Zidere, Trisha Vigneswaran, Marietta Charakida, Owen Miller, Gurleen Sharland & John Simpson

  2. Harris Birthright Centre, King’s College London NHS Foundation Trust, London, UK

    Vita Zidere, Trisha Vigneswaran, Marietta Charakida & John Simpson

  3. School of Biomedical Engineering, Division of Imaging Sciences, King’s College London, London, UK

    Marietta Charakida & John Simpson

Authors
  1. Thomas G. Day
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Tomas Woodgate
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Olatejumoye Knee
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Vita Zidere
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Trisha Vigneswaran
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Marietta Charakida
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Owen Miller
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Gurleen Sharland
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. John Simpson
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to John Simpson.

Ethics declarations

Conflict of interest

None of the authors have any conflict of interest with respect to this paper.

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

Day, T.G., Woodgate, T., Knee, O. et al. Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections. Pediatr Cardiol 40, 1509–1515 (2019). https://doi.org/10.1007/s00246-019-02176-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-019-02176-2

Keywords

Search

Navigation