Skip to main content
SpringerLink
Log in

An Observation from Liver Biopsies Two Decades Post-Fontan

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

Abstract

This brief report describes an observation from liver biopsy results in nonfailing Fontan patients, currently in their second postoperative decade. In three patients, with either atriopulmonary or atrioventricular connections and functional left ventricles, we found no portal fibrosis. In contrast, we found portal fibrosis in three clinically similar, nonfailing Fontan patients with lateral tunnel connections and functional left ventricles. We recognize the results may be secondary to chance; nevertheless, we speculate about possible relevancy.

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

Similar content being viewed by others

References

  1. Bjork VO, Olin CL, Bjarke BB, Thoren CA (1979) Right atrial-right ventricular anastomosis for correction of tricuspid atresia. J Thorac Cardiovasc Surg 77:452–458

    CAS  PubMed  Google Scholar 

  2. Kreutzer GO, Vargas FJ, Schlichter AJ, Laura JP, Suarez JC, Coronel AR, Kreutzer EA (1982) Atriopulmonary anastomosis. J Thorac Cardiovasc Surg 83:427–436

    CAS  PubMed  Google Scholar 

  3. Puga FJ, Chiavarelli M, Hagler DJ (1987) Modification of the Fontan operation applicable to patients with left atrioventricular valve atresia or single atrioventricular valve. Circulation 76(suppl III):III-53–III-60

    CAS  Google Scholar 

  4. De Leval MR, Kilner P, Gewillig M, Bull C (1988) Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. J Thorac Cardiovasc Surg 96:682–695

    PubMed  Google Scholar 

  5. Marcelletti C, Corno A, Giannico S, Marino B (1990) Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg 100:228–232

    CAS  PubMed  Google Scholar 

  6. Evans WN, Winn BJ, Yumiaco NS, Galindo A, Rothman A, Acherman RJ, Restrepo H (2014) Transvenous hepatic biopsy in stable Fontan patients undergoing cardiac catheterization. Pediatr Cardiol 35:1273–1278

    Article  PubMed  Google Scholar 

  7. Evans WN, Acherman RJ, Winn BJ, Yumiaco NS, Galindo A, Rothman A, Restrepo H (2015) Fontan hepatic fibrosis and pulmonary vascular development. Pediatr Cardiol 36:657–661

    Article  PubMed  Google Scholar 

  8. Evans WN, Acherman RJ, Reardon LC, Ciccolo ML, Galindo A, Rothman A, Winn BJ, Yumiaco NS, Restrepo H (2016) Fontan outcomes and pulmonary blood flow at birth. Pediatr Cardiol 37:30–36

    Article  PubMed  Google Scholar 

  9. Wu FM, Jonas MM, Opotowsky AR, Harmon A, Raza R, Ukomadu C, Landzberg MJ, Singh MN, Valente AM, Egidy Assenza G, Perez-Atayde AR (2015) Portal and centrilobular hepatic fibrosis in Fontan circulation and clinical outcomes. J Heart Lung Transplant 34:883–891

    Article  PubMed  Google Scholar 

  10. Kiesewetter CH, Sheron N, Vettukattill JJ, Hacking N, Stedman B, Millward-Sadler H, Haw M, Cope R, Salmon AP, Sivaprakasam MC, Kendall T, Keeton BR, Iredale JP, Veldtman GR (2007) Hepatic changes in the failing Fontan circulation. Heart 93:579–584

    Article  PubMed  Google Scholar 

  11. Kendall TJ, Stedman B, Hacking N, Haw M, Vettukattill JJ, Salmon AP, Cope R, Sheron N, Millward-Sadler H, Veldtman GR, Iredale JP (2008) Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study. J Clin Pathol 61:504–508

    Article  CAS  PubMed  Google Scholar 

  12. Schwartz MC, Sullivan L, Cohen MS, Russo P, John AS, Guo R, Guttenberg M, Rand EB (2012) Hepatic pathology may develop before the Fontan operation in children with functional single ventricle: an autopsy study. J Thorac Cardiovasc Surg 143:904–909

    Article  PubMed  Google Scholar 

  13. Johnson JA, Cetta F, Graham RP, Smyrk TC, Driscoll DJ, Phillips SD, John AS (2013) Identifying predictors of hepatic disease in patients after the Fontan operation: a postmortem analysis. J Thorac Cardiovasc Surg 146:140–145

    Article  PubMed  Google Scholar 

  14. Schwartz MC, Sullivan LM, Glatz AC, Rand E, Russo P, Goldberg DJ, Rome JJ, Cohen MS (2013) Portal and sinusoidal fibrosis are common on liver biopsy after Fontan surgery. Pediatr Cardiol 34:135–142

    Article  PubMed  Google Scholar 

  15. Bronicki RA (2014) Cardiopulmonary interactions. In: Wheeler DS, Wong HR, Shanley TP (eds) Pediatric critical care medicine, volume 2, respiratory cardiovascular and central nervous systems. Springer, London, p 324

    Google Scholar 

  16. Honda T, Itatani K, Takanashi M, Mineo E, Kitagawa A, Ando H, Kimura S, Nakahata Y, Oka N, Miyaji K, Ishii M (2014) Quantitative evaluation of hemodynamics in the Fontan circulation: a cross-sectional study measuring energy loss in vivo. Pediatr Cardiol 35:361–367

    Article  PubMed  Google Scholar 

  17. Quinton E, Nightingale P, Hudsmith L, Thorne S, Marshall H, Clift P, de Bono J (2015) Prevalence of atrial tachyarrhythmia in adults after Fontan operation. Heart 101:1672–1677

    Article  PubMed  Google Scholar 

  18. Schwartz MC, Glatz AC, Daniels K, Goldberg DJ, Rand E, Epelman MS, Cohen MS (2015) Hepatic Abnormalities Are Present Before and Early After the Fontan Operation. Ann Thorac Surg 100:2298–2304

    Article  PubMed  Google Scholar 

  19. Chopski SG, Fox CS, Riddle ML, McKenna KL, Patel JP, Rozolis JT, Throckmorton AL (2015) Pressure-flow experimental performance of new intravascular blood pump designs for Fontan patients. Artif Organs. doi:10.1111/aor.12549

    Google Scholar 

Download references

Acknowledgments

We wish to thank the interventional radiologists of Radiology Specialists at Sunrise Children’s Hospital and Medical Center, who performed the transvenous hepatic biopsies, including Drs. Demetrice Davis, Steven Davis, Kelly Gardner, Sunil Gujrathi, Aaron Peterson, and Matthew Rainey.

Author information

Authors and Affiliations

  1. Children’s Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA

    William N. Evans, Ruben J. Acherman, Leigh C. Reardon, Alvaro Galindo, Abraham Rothman, Michael L. Ciccolo, Sergio A. Carrillo & Humberto Restrepo

  2. Division of Pediatric Cardiology, Department of Pediatrics, University of Nevada School of Medicine, 2040 W. Charleston Blvd Ste. 402, Las Vegas, NV, 89109, USA

    William N. Evans, Ruben J. Acherman, Alvaro Galindo, Abraham Rothman, Michael L. Ciccolo, Sergio A. Carrillo & Humberto Restrepo

  3. Ahmanson/UCLA Adult Congenital Heart Disease Center, UCLA Division of Cardiology and Division of Pediatric Cardiology, 100 UCLA Medical Plaza, Suite 630, Los Angeles, CA, 90095, USA

    Leigh C. Reardon

  4. Laboratory Medicine Consultants, 3059 S. Maryland Parkway, Suite 100, Las Vegas, NV, 89109, USA

    Brody J. Winn & Noel S. Yumiaco

Authors
  1. William N. Evans
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ruben J. Acherman
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Leigh C. Reardon
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Alvaro Galindo
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Abraham Rothman
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Michael L. Ciccolo
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Sergio A. Carrillo
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Brody J. Winn
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Noel S. Yumiaco
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Humberto Restrepo
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to William N. Evans.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Evans, W.N., Acherman, R.J., Reardon, L.C. et al. An Observation from Liver Biopsies Two Decades Post-Fontan. Pediatr Cardiol 37, 1119–1122 (2016). https://doi.org/10.1007/s00246-016-1403-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-016-1403-x

Keywords

Search

Navigation