Skip to main content
SpringerLink
Log in

Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection

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

Abstract

Objectives

A few studies have described the outcomes of systemic-to-pulmonary artery shunt (SPS) for functional single ventricular heart with extracardiac total anomalous pulmonary venous connection (TAPVC). This study aimed to examine the outcomes of SPS with extracardiac TAPVC and identify the predictors of mortality before bidirectional Glenn operation (BDG).

Methods

Medical records of 41 children with single ventricular heart and extracardiac TAPVC who underwent SPS between 1998 and 2019 were reviewed retrospectively. The median age and body weight at SPS were 36 days and 3.4 kg, respectively. Surgical outcomes and predictors of mortality were investigated.

Results

Four operative deaths (10%) and 10 late deaths (27%) occurred before BDG. Of all the children, 19 underwent BDG at a median of 10 months since SPS and eight are waiting for BDG. In the multivariate analysis, preoperative pulmonary venous obstruction (p = 0.01) at initial surgery was most predictive of death before BDG. Patients who underwent simultaneous SPS and TAPVC repair were younger, had high preoperative rate of pulmonary venous obstruction, and more deaths before BDG.

Conclusions

Survival outcomes of SPS for SVH with extracardiac TAPVC were improved as a whole due to the increase in knowledge and technique of management SPS. However, the patients who have preoperative pulmonary venous obstruction (PVO) and need SPS and TAPVC repair concomitantly in the early postnatal period have poor outcomes and still challenging. In such a case, staged TAPVC repair and SPS may be beneficial.

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

Similar content being viewed by others

References

  1. Alsoufi B, Gillespie S, Kogon B, Schlosser B, Sachdeva R, Kim D, et al. Results of palliation with an initial modified Blalock–Taussig shunt in neonates with single ventricle anomalies associated with restrictive pulmonary blood flow. Ann Thorac Surg. 2015;99:1639–47.

    Article  Google Scholar 

  2. Petrucci O, O’Brien SM, Jacobs ML, Jacobs JP, Manning PB, Eghtesady P. Risk factors for mortality and morbidity after the neonatal Blalock–Taussig shunt procedure. Ann Thorac Surg. 2011;92:642–52.

    Article  Google Scholar 

  3. Chittithavorn V, Duangpakdee P, Rergkliang C, Pruekprasert N. Risk factors for in-hospital shunt thrombosiss and mortality in patients weighing less than 3kg with functionally uneventricular heart undergoing a modified Blalock–Taussig shunt. Interact Cardiovasc Thorac Surg. 2017;25:407–13.

    Article  Google Scholar 

  4. Fenton KN, Sierwers RD, Rebovich B, Pigula FA. Interim mortality in infants with systemic-to-pulmonary artery shunts. Ann Thorac Surg. 2003;76:152–7.

    Article  Google Scholar 

  5. O’Connor MJ, Ravishankar C, Ballweg JA, Gillespie MJ, Gaynor JW, Tabbutt S, et al. Early systemic-to-pulmonary artery shunt intervention in neonates with congenital heart disease. J Thorac Cardiovasc Surg. 2011;142:106–12.

    Article  Google Scholar 

  6. Nakayama Y, Hiramatsu T, Iwata Y, Okamura T, Konuma T, Matsumura G, et al. Surgical results for functional univentricular heart with total anomalous pulmonary venous connection over 25-year experience. Ann Thorac Surg. 2012;93:606–13.

    Article  Google Scholar 

  7. Nakata T, Fujimoto Y, Hirose K, Osaki M, Tosaka Y, Ide Y, et al. Functional single ventricle with extracardiac total anomalous pulmonary venous connection. Eur J Cardiothorac Surg. 2009;36:49–56.

    Article  Google Scholar 

  8. Morales DL, Braud BE, Booth JH, Graves DE, Heinle JS, Mckenzie ED, et al. Heterotaxy patients with total anomalous pulmonary venous return: Improving surgical results. J Ann Thorac Surg. 2006;82:1621–8.

    Article  Google Scholar 

  9. Lodge AJ, Rychik J, Nicolson SC, Ittenbach RF, Spray TL, Gaynor JW. Improving outcomes in functional single ventricle and total anomalous pulmonary venous connection. Ann Thorac Surg. 2004;78:1688–95.

    Article  Google Scholar 

  10. Jang SI, Song JY, Kim SJ, Choi EY, Shim WS, Lee C, et al. The recent surgical result of total anomalous pulmonary venous return. Korean Circ J. 2010;40:31–5.

    Article  Google Scholar 

  11. Dorobantu DM, Pandey R, Sharabiani MT, Mahani AS, Angelini GD, Martin RP, et al. Indication and results of systemic to pulmonary shunts: results from a national database. Eur J Cardiothorac Surg. 2016;49:1553–63.

    Article  Google Scholar 

  12. Do N, Hill KD, Wallace AS, Vricella L, Cameron D, Quintessenza J, et al. Shunt failure—risk factors and outcomes: an analysis of the society of thoracic surgeons congenital heart surgery database. Ann Thorac Surg. 2018;105:857–64.

    Article  Google Scholar 

  13. Alsoufi B, Gillespie S, Mori M, Clabby M, Kanter K, Kogon B. Factors affecting death and progression towards next stage following modified Blalock–Taussig shunt in neonates. Eur J Cardiothorac Surg. 2016;50:169–77.

    Article  Google Scholar 

  14. Uno Y, Masuoka A, Hotoda K, Katogi T, Suzuki T. Evaluation of 60 cases of systemic-pulmonary shunt with cardiopulmonary bypasss. Gen Thorac Cardiovasc Surg. 2016;64:592–6.

    Article  Google Scholar 

  15. Harada T, Nakano T, Oda S, Kado H. Surgical results of total anomalous pulmonary venous connection repair in 256 patients. Interact Cardiovasc Thorac Surg. 2019;28:421–6.

    Article  Google Scholar 

  16. Najm HK, Caldarone CA, Smallhorn J, Coles JG. A sutureless technique for the relief of pulmonary vein stenosis with the use of in situ pericardium. J Thorac Cardiovasc Surg. 1998;115:468–70.

    Article  CAS  Google Scholar 

  17. Honjo O, Atlin CR, Hamilton BCS, Al-Radi O, Viola N, Coles JG, et al. Primary sutureless repair for infants with mixed total anomalous pulmonary venous drainage. Ann Thorac Surg. 2010;90:862–8.

    Article  Google Scholar 

  18. Nakano T, Kado H, Shiokawa Y, Fukae K, Nishimura Y, Miyamoto K, et al. The low resistance strategy for the perioperative management of the Norwood procedure. Ann Thorac Surg. 2004;77:908–12.

    Article  Google Scholar 

  19. Kanda Y. Investigation of the freely available easy-to-use software ‘EZR’ for medical statics. Bone Marrow Transplant. 2013;48:452–8.

    Article  CAS  Google Scholar 

  20. Khan MS, Bryant R, Kim SH, Hill KD, Jacobs JP, Jacobs ML, et al. Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg. 2015;99:2134–40.

    Article  Google Scholar 

  21. Gaynor JW, Collins MH, Rychik J, Gaughan JP, Spray TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. 1999;117:506–14.

    Article  CAS  Google Scholar 

  22. Yamaki S, Tsunemoto M, Shimada M, Ishizawa R, Endo M, Nakayama S, et al. Quantitative analysis of pulmonary vascular disease in total anomalous pulmonary venous connection in sixty infants. J Thorac Cardiovasc Surg. 1992;104:728–35.

    Article  CAS  Google Scholar 

  23. Gersony WM. Editorial: obstruction to pulmonary venous return obscured by decreased pulmonary blood flow. Chest. 1973;64:283.

    Article  CAS  Google Scholar 

  24. Freedom RM, Olley PM, Coceani F, Rowe RD. The prostaglandin challenge. Test to unmask obstructed total anomalous pulmonary venous connections in asplenia syndrome. Br Heart J. 1978;40:91–4.

    Article  CAS  Google Scholar 

  25. Caldarone CA, Najm HK, Kadletz M, Smallhorn JF, Freedom RM, Williams WG, et al. Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies. Ann Thorac Surg. 1998;66:1521–6.

    Article  CAS  Google Scholar 

  26. Hoashi T, Kagisaki K, Oda T, Kitano M, Kurosaki K, Shiraishi I, et al. Long-term results of treatments for functional single ventricle associated with extracardiac type total anomalous pulmonary venous connection. Eur J Cardiothorac Surg. 2013;43:965–70.

    Article  Google Scholar 

  27. Hancock Frisen CL, Zurakowski D, Thiagarajan RR, Forbess JM, del Nido PJ, Mayer JE, et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg. 2005;79:596–606.

    Article  Google Scholar 

  28. Husain SA, Maldonado E, Rasch D, Michalek J, Taylor R, Curzon C, et al. Total anomalous pulmonary venous connection: factors associated with mortality and recurrent pulmonary venous obstruction. Ann Thorac Surg. 2012;94:825–32.

    Article  Google Scholar 

  29. Guocheng S, Zhongqun Z, Jimei C, Yanqiu O, Haifa H, Zhiqiang N, et al. Total anomalous pulmonary venous connection. The current management strategies in a pediatric cohort of 768 patients. Circulation. 2017;135:48–58.

    Article  Google Scholar 

  30. Matsui H, Yasukochi S, Takigiku K, Tazawa S. Urgent stenting via jugular vein for stabilization in critical neonates with total anomalous pulmonary venous connection and severe pulmonary venous obstruction. J Cardiol. 2013;8:e51–e5353.

    Google Scholar 

  31. Sarmast H, Takriti A. A new palliative surgical technique for high risk total anomalous pulmonary venous connection (Sarmast–Tkriti shunt). J Cardiothorac Surg. 2019;14:127.

    Article  Google Scholar 

Download references

Acknowledgements

We would like to thank Editage (www,editage.jp) for English language editing.

Author information

Authors and Affiliations

  1. Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, 5-1-1 Kashiiteriha, Higashi-ku, Fukuoka, 813-0017, Japan

    Takuya Okamoto, Toshihide Nakano, Masami Goda, Shinichiro Oda & Hideaki Kado

Authors
  1. Takuya Okamoto
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Toshihide Nakano
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Masami Goda
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Shinichiro Oda
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Hideaki Kado
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Takuya Okamoto.

Ethics declarations

Conflict of interest

All authors have no conflict of interest to disclose with respect to this study.

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

Okamoto, T., Nakano, T., Goda, M. et al. Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection. Gen Thorac Cardiovasc Surg 69, 646–653 (2021). https://doi.org/10.1007/s11748-020-01474-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11748-020-01474-4

Keywords

Search

Navigation