Skip to main content

Advertisement

SpringerLink
Log in

Comparison of Fontan Surgical Options for Patients with Apicocaval Juxtaposition

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

Abstract

Apicocaval juxtaposition (ACJ) is a rare form of viscerocardiac malpositions in association with single-ventricle congenital heart defects. The Fontan surgery is the common palliation, and possible surgical options include ipsilateral, contralateral, and intra-atrial conduits. Concerns include lower hemodynamic performances or risks of conduit compression by the cardiac mass. This study investigates the hemodynamics and clinical outcomes of ACJ patients and potential surgical improvements. Ten consecutive ACJ patients were included, along with a reference cohort of ten non-ACJ patients. Magnetic resonance images were acquired at 6 ± 0.6 year follow-up for anatomical analysis and hemodynamic assessments using computational fluid dynamics. Metrics of interest are deformation index (DI), indexed power loss (iPL), and hepatic flow distribution (HFDoff). A “virtual” surgery was performed to explore potential hemodynamic improvements using a straightened conduit. DI for ACJ patients fell within the DI range of non-ACJ patients. Contralateral conduits had insignificantly higher iPL (0.070 [0.032,0.137]) than ipsilateral conduits (0.041 [0.013,0.095]) and non-ACJ conduits (0.034 [0.011,0.061]). HFDoff was similar for the ipsilateral (21 [12,35]), contralateral (26 [7,41]), and non-ACJ Fontan conduits (17 [0,48]). Virtual surgery demonstrated that a straightened conduit reduced HFDoff and iPL for the contralateral and ipsilateral conduits, potentially leading to improved clinical outcomes. In this limited sample, the hemodynamic performance of ACJ patients was not significantly different from their non-ACJ counterparts. The use of a straightened conduit option could potentially improve patient outcomes. Additionally, the fear of significant compression of conduits for ACJ patients was unsupported.

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

Similar content being viewed by others

References

  1. Li D, Fan Q, Hirata Y, Ono M, An Q (2017) Arrhythmias after Fontan operation with intra-atrial lateral tunnel versus extra-cardiac conduit: a systematic review and meta-analysis. Pediatr Cardiol 38(4):873–880. https://doi.org/10.1007/s00246-017-1595-8

    Article  PubMed  Google Scholar 

  2. Katogi T (2012) Extracardiac conduit Fontan procedure versus intra-atrial lateral tunnel Fontan procedure. Gen Thorac Cardiovasc Surg 60(12):792–795. https://doi.org/10.1007/s11748-012-0161-9

    Article  PubMed  Google Scholar 

  3. Ochiai Y, Imoto Y, Sakamoto M, Kajiwara T, Sese A, Watanabe M, Ohno T, Joo K (2009) Mid-term follow-up of the status of Gore-Tex graft after extracardiac conduit Fontan procedure. Eur J Cardiothorac Surg 36(1):63–67. https://doi.org/10.1016/j.ejcts.2009.02.013discussion 67-68

    Article  PubMed  Google Scholar 

  4. de Leval MR (2013) Re: total cavopulmonary connection in patients with apicocaval juxtaposition: optimal conduit route using preoperative angiogram and flow simulation. Eur J Cardiothorac Surg 44(1):e52. https://doi.org/10.1093/ejcts/ezt170

    Article  PubMed  Google Scholar 

  5. Gil-Jaurena JM, Perez-Caballero R, Pita A, Gonzalez-Lopez M (2016) Extracardiac Fontan in apicocaval juxtaposition. Asian Cardiovasc Thorac Ann 24(2):178–180. https://doi.org/10.1177/0218492314553613

    Article  PubMed  Google Scholar 

  6. Wei ZA, Trusty PM, Tree M, Haggerty CM, Tang E, Fogel M, Yoganathan AP (2017) Can time-averaged flow boundary conditions be used to meet the clinical timeline for Fontan surgical planning? J Biomech 50:172–179. https://doi.org/10.1016/j.jbiomech.2016.11.025

    Article  PubMed  Google Scholar 

  7. Tree M, Wei ZA, Trusty PM, Raghav V, Fogel M, Maher K, Yoganathan A (2018) Using a novel in vitro Fontan model and condition-specific real-time MRI Data to examine hemodynamic effects of respiration and exercise. Ann Biomed Eng 46(1):135–147. https://doi.org/10.1007/s10439-017-1943-0

    Article  PubMed  Google Scholar 

  8. Rijnberg FM, Hazekamp MG, Wentzel JJ, de Koning PJH, Westenberg JJM, Jongbloed MRM, Blom NA, Roest AAW (2018) Energetics of blood flow in cardiovascular disease: concept and clinical implications of adverse energetics in patients with a Fontan circulation. Circulation 137(22):2393–2407. https://doi.org/10.1161/CIRCULATIONAHA.117.033359

    Article  Google Scholar 

  9. Trusty PM, Wei Z, Rychik J, Russo PA, Surrey LF, Goldberg DJ, Fogel MA, Yoganathan AP (2018) Impact of hemodynamics and fluid energetics on liver fibrosis after Fontan operation. J Thorac Cardiovasc Surg 156(1):267–275. https://doi.org/10.1016/j.jtcvs.2018.02.078

    Article  PubMed  Google Scholar 

  10. Trusty PM, Wei Z, Rychik J, Graham A, Russo PA, Surrey LF, Goldberg DJ, Yoganathan AP, Fogel MA (Accepted) Cardiac magnetic resonance derived metrics are predictive of liver fibrosis in Fontan patients. Ann Thorac Surg

  11. Yang W, Ja F, Shadden SC, Vignon-Clementel IE, Marsden AL (2013) Optimization of a Y-graft design for improved hepatic flow distribution in the fontan circulation. J Biomech Eng 135(1):011002–011002. https://doi.org/10.1115/1.4023089

    Article  PubMed  Google Scholar 

  12. Menon PG, Yoshida M, Pekkan K (2013) Presurgical evaluation of Fontan connection options for patients with apicocaval juxtaposition using computational fluid dynamics. Artif Organs 37(1):E1–8. https://doi.org/10.1111/j.1525-1594.2012.01555.x

    Article  PubMed  Google Scholar 

  13. Yoshida M, Menon PG, Chrysostomou C, Pekkan K, Wearden PD, Oshima Y, Okita Y, Morell VO (2013) Total cavopulmonary connection in patients with apicocaval juxtaposition: optimal conduit route using preoperative angiogram and flow simulation. Eur J Cardiothorac Surg 44(1):e46–52. https://doi.org/10.1093/ejcts/ezt118

    Article  PubMed  Google Scholar 

  14. Tang E, Wei Z, Whitehead KK, Khiabani RH, Restrepo M, Mirabella L, Bethel J, Paridon SM, Marino BS, Fogel MA, Yoganathan AP (2017) Effect of Fontan geometry on exercise haemodynamics and its potential implications. Heart. https://doi.org/10.1136/heartjnl-2016-310855

    Article  PubMed  PubMed Central  Google Scholar 

  15. Marino BS, Fogel M, Mercer-Rosa LM-R, Wei ZAW, Trusty PM, Tree M, Tang E, Restrepo M, Whitehead KK, Paridon SM, Yoganathan A (2017) Poor Fontan geometry, hemodynamics, and computational fluid dynamics are associated with worse quality of life. Paper presented at the circulation, 14 Nov 2017

  16. Pike NA, Vricella LA, Feinstein JA, Black MD, Reitz BA (2004) Regression of severe pulmonary arteriovenous malformations after Fontan revision and “hepatic factor” rerouting. Ann Thorac Surg 78(2):697–699. https://doi.org/10.1016/j.athoracsur.2004.02.003

    Article  PubMed  Google Scholar 

  17. Trusty PM, Wei Z, Tree M, Kanter KR, Fogel MA, Yoganathan AP, Slesnick TC (2017) Local hemodynamic differences between commercially available Y-grafts and traditional Fontan baffles under simulated exercise conditions: implications for exercise tolerance. Cardiovasc Eng Technol 8(3):390–399. https://doi.org/10.1007/s13239-017-0310-5

    Article  PubMed  Google Scholar 

  18. Trusty PM, Wei Z, Sales M, Kanter KR, Fogel MA, Yoganathan AP, Slesnick TC (2019) Y-graft modification to the Fontan procedure: Increasingly balanced flow over time. J Thorac Cardiovasc Surg. https://doi.org/10.1016/j.jtcvs.2019.06.063

    Article  PubMed  Google Scholar 

  19. Tang E, Restrepo M, Haggerty CM, Mirabella L, Bethel J, Whitehead KK, Fogel MA, Yoganathan AP (2014) Geometric characterization of patient-specific total cavopulmonary connections and its relationship to hemodynamics. JACC: Cardiovasc Imaging 7(3):215–224. https://doi.org/10.1016/j.jcmg.2013.12.010

    Article  Google Scholar 

  20. Kottayil BP, Sunil GS, Kappanayil M, Mohanty SH, Francis E, Vaidyanathan B, Balachandran R, Nair SG, Kumar RK (2014) Two-ventricle repair for complex congenital heart defects palliated towards single-ventricle repair. Interact Cardiovasc Thorac Surg 18(3):266–271. https://doi.org/10.1093/icvts/ivt495

    Article  PubMed  Google Scholar 

  21. Heiberg E, Sjögren J, Ugander M, Carlsson M, Engblom H, Arheden H (2010) Design and validation of segment—freely available software for cardiovascular image analysis. BMC Med Imaging 10(1):1

    Article  Google Scholar 

  22. Wei ZA, Tree M, Trusty PM, Wu W, Singh-Gryzbon S, Yoganathan A (2017) The advantages of viscous dissipation rate over simplified power loss as a Fontan hemodynamic metric. Ann Biomed Eng. https://doi.org/10.1007/s10439-017-1950-1

    Article  PubMed  PubMed Central  Google Scholar 

  23. Wei ZA, Huddleston C, Trusty PM, Singh-Gryzbon S, Fogel MA, Veneziani A, Yoganathan AP (2019) Analysis of inlet velocity profiles in numerical assessment of Fontan hemodynamics. Ann Biomed Eng. https://doi.org/10.1007/s10439-019-02307-z

    Article  PubMed  PubMed Central  Google Scholar 

  24. Luffel M, Sati M, Rossignac J, Yoganathan AP, Haggerty CM, Restrepo M, Slesnick TC, Kanter KR, Del Nido P, Fogel MA (2016) SURGEM: a solid modeling tool for planning and optimizing pediatric heart surgeries. CAD Comput Aided Des 70:3–12. https://doi.org/10.1016/j.cad.2015.06.018

    Article  Google Scholar 

  25. Trusty PM, Slesnick TC, Wei ZA, Rossignac J, Kanter KR, Fogel MA, Yoganathan AP (2018) Fontan surgical planning: previous accomplishments, current challenges, and future directions. J Cardiovasc Transl Res 11(2):133–144. https://doi.org/10.1007/s12265-018-9786-0

    Article  PubMed  PubMed Central  Google Scholar 

  26. Trusty PM, Wei ZA, Slesnick TC, Kanter KR, Spray TL, Fogel MA, Yoganathan AP (2019) The first cohort of prospective Fontan surgical planning patients with follow-up data: How accurate is surgical planning? J Thorac Cardiovasc Surg 157(3):1146–1155. https://doi.org/10.1016/j.jtcvs.2018.11.102

    Article  PubMed  Google Scholar 

  27. Chen W, Lu Y, Ma L, Yang S, Xia Y, Zou M, Chen X (2018) Conduit route selection for total cavopulmonary connection in patients with apicocaval juxtaposition. Semin Thorac Cardiovasc Surg. https://doi.org/10.1053/j.semtcvs.2018.07.004

    Article  PubMed  Google Scholar 

  28. Morizumi S, Kato H, Kanemoto S, Noma M, Abe M, Sakakibara Y, Hiramatsu Y (2012) Appropriate route selection for extracardiac total cavopulmonary connection in apicocaval juxtaposition. Ann Thorac Surg 94(1):179–184. https://doi.org/10.1016/j.athoracsur.2012.03.026

    Article  PubMed  Google Scholar 

  29. Sakurai T, Kado H, Nakano T, Hinokiyama K, Oda S, Sugiura J, Ushijima T, Ueda Y (2010) The impact of extracardiac conduit-total cavopulmonary connection on apicocaval juxtaposition. Eur J Cardiothorac Surg 38(4):439–444. https://doi.org/10.1016/j.ejcts.2010.02.032

    Article  PubMed  Google Scholar 

  30. Kawahira Y, Nishigaki K, Ueno T (2006) Extracardiac Fontan procedure bridging the vertebra for apico-caval juxtaposition. Ann Thorac Surg 82(1):350–352. https://doi.org/10.1016/j.athoracsur.2005.07.059

    Article  PubMed  Google Scholar 

  31. Kitayama H, Oku H, Matsumoto T, Onoe M (2001) Total cavopulmonary connection using a pedicled pericardial conduit for a patient with apicocaval juxtaposition. Ann Thorac Surg 72(4):1393–1394. https://doi.org/10.1016/s0003-4975(00)02591-1

    Article  CAS  PubMed  Google Scholar 

  32. Wei Z, Whitehead KK, Khiabani RH, Tree M, Tang E, Paridon SM, Fogel MA, Yoganathan AP (2016) Respiratory effects on Fontan circulation during rest and exercise using real-time cardiac magnetic resonance imaging. Ann Thorac Surg 101(5):1818–1825. https://doi.org/10.1016/j.athoracsur.2015.11.011

    Article  PubMed  PubMed Central  Google Scholar 

  33. Tang E, Wei ZA, Trusty PM, Whitehead KK, Mirabella L, Veneziani A, Fogel MA, Yoganathan AP (2019) The effect of respiration-driven flow waveforms on hemodynamic metrics used in Fontan surgical planning. J Biomech 82:87–95. https://doi.org/10.1016/j.jbiomech.2018.10.013

    Article  PubMed  Google Scholar 

  34. Wei Z, Trusty P, Zhang Y, Tang E, Whitehead K, Fogel M, Yoganathan A (Accepted) Is free-breathing phase-contrast MRI needed for Fontan preintervention planning? J Cardiovasc Magn Reson

  35. Long CC, Hsu MCCMC, Bazilevs Y, Feinstein JA, Marsden AL (2012) Fluid—structure interaction simulations of the Fontan procedure using variable wall properties. Int J Numer Methods Biomed Eng 28(January):513–527. https://doi.org/10.1002/cnm

    Article  CAS  Google Scholar 

  36. Mirabella L, Haggerty CM, Passerini T, Piccinelli M, Powell AJ, Del Nido PJ, Veneziani A, Yoganathan AP (2013) Treatment planning for a TCPC test case: a numerical investigation under rigid and moving wall assumptions. Int J Numer Methods Biomed Eng 29(2):197–216. https://doi.org/10.1002/cnm.2517

    Article  Google Scholar 

Download references

Acknowledgements

The authors acknowledge the use of ANSYS software, which was provided through an Academic Partnership between ANSYS, Inc., and the Cardiovascular Fluid Mechanics Lab at the Georgia Institute of Technology.

Funding

This study was supported by the National Heart, Lung, and Blood Institute Grants HL067622 and HL098252.

Author information

Authors and Affiliations

  1. Department of Biomedical Engineering, Georgia Institute of Technology, Suite 200, 387 Technology Circle, Atlanta, GA, 30313-2412, USA

    Zhenglun Alan Wei, Camille Johnson, Phillip Trusty, Morgan Stephens, Wenjun Wu & Ajit P. Yoganathan

  2. Amrita Institute of Medical Sciences and Research Centre, Kochi, India

    Ritchie Sharon, Balaji Srimurugan, Brijesh P. Kottayil, G. S. Sunil & Mahesh Kappanayil

  3. Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Mark A. Fogel

Authors
  1. Zhenglun Alan Wei
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Camille Johnson
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Phillip Trusty
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Morgan Stephens
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Wenjun Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Ritchie Sharon
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Balaji Srimurugan
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Brijesh P. Kottayil
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. G. S. Sunil
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Mark A. Fogel
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Ajit P. Yoganathan
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Mahesh Kappanayil
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ajit P. Yoganathan.

Ethics declarations

Conflict of interest

All authors declare that they have no conflicts of interest.

Ethical approval

All procedures performed in studies involving human participants were complied with the Institutional Review Boards of the participating institutions: Georgia Institute of Technology and Children’s Hospital of Philadelphia (IRB Number H05236, Understanding/Improving Fontan Flow Dynamics II) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.

Informed consent

Informed consent was obtained from all individual participants included in the 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

Wei, Z.A., Johnson, C., Trusty, P. et al. Comparison of Fontan Surgical Options for Patients with Apicocaval Juxtaposition. Pediatr Cardiol 41, 1021–1030 (2020). https://doi.org/10.1007/s00246-020-02353-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-020-02353-8

Keywords

Search

Navigation