Skip to main content
Log in

Timing of Fontan Completion in Children with Functionally Univentricular Hearts and Isomerism: The Impact of Age, Weight, and Pre-Fontan Arterial Oxygen Saturation

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

Abstract

Isomerism, or heterotaxy, impacts morbidity and mortality after various stages of univentricular palliation. Timing of Fontan completing in these patients based on preoperative factors has not been investigated previously. The aim of this study was to determine the impact of preoperative factors on various outcomes including length of hospital stay and duration of chest tubes. A cross-sectional study was conducted. Patients with isomerism having undergone Fontan at the Children’s Hospital of Wisconsin between 1998 and 2014 were identified. Preoperative, operative, and postoperative data were collected on these patients. Linear regression analysis was conducted to determine preoperative characteristics associated with various postoperative outcomes. Receiver operator curve analysis was also performed to determine the sensitivity and specificity of age and pre-Fontan arterial oxygen saturation in predicting increased length of hospitalization and increased duration of chest tubes. Younger age and lower pre-Fontan arterial oxygen saturation were associated with increased length of hospitalization while younger age, lower pre-Fontan arterial oxygen saturation, interrupted inferior caval vein, and worse pre-Fontan atrioventricular valve regurgitation were associated with increased length of chest tubes. Age, arterial oxygen saturation, pulmonary arteriovenous malformations, interruption of the inferior caval vein, and atrioventricular valve regurgitation should all be taken into consideration when timing Fontan completion in patients with isomerism. Arterial oxygen saturations between 82 and 84% with an approximate age of 3 years appear to be a time at which it is reasonable to consider Fontan in patients with isomerism.

This is a preview of subscription content, log in via an institution to check access.

Access this article

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. Evans WN, Acherman RJ, Restrepo H (2015) Heterotaxy in southern Nevada: prenatal detection and epidemiology. Pediatr Cardiol 36:930–934

    Article  PubMed  Google Scholar 

  2. Lin AE, Krikov S, Riehle-Colarusso T, Frias JL, Belmont J, Anderka M, Geva T, Getz KD, Botto LD, National Birth Defects Prevention Study (2014) Laterality defects in the national birth defects prevention study (1998–2007): birth prevalence and descriptive epidemiology. Am J Med Genet A 164A:2581–2591

    Article  PubMed  Google Scholar 

  3. Lopez KN, Marengo LK, Canfield MA, Belmont JW, Dickerson HA (2015) Racial disparities in heterotaxy syndrome. Birth Defects Res A. https://doi.org/10.1002/bdra.23416

    Article  Google Scholar 

  4. Loomba RS, Hlavacek AM, Spicer DE, Anderson RH (2015) Isomerism or heterotaxy: which term leads to better understanding? Cardiol Young. https://doi.org/10.1017/S1047951115001122

    Article  PubMed  Google Scholar 

  5. Jacobs JP, Anderson RH, Weinberg PM, Walters HL 3rd, Tchervenkov CI, Del Duca D, Franklin RC, Aiello VD, Beland MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ (2007) The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young 17(Suppl 2):1–28

    PubMed  Google Scholar 

  6. Uemura H, Ho SY, Devine WA, Anderson RH (1995) Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. Am J Cardiol 76:846–849

    Article  CAS  PubMed  Google Scholar 

  7. Loomba R, Shah PH, Anderson RH (2015) Fetal magnetic resonance imaging of malformations associated with heterotaxy. Cureus 7:e269

    PubMed  PubMed Central  Google Scholar 

  8. Loomba RS, Ahmed MM, Spicer DE, Backer CL, Anderson RH (2016) Manifestations of bodily isomerism. Cardiovasc Pathol. https://doi.org/10.1016/j.carpath.2016.01.003

    Article  PubMed  Google Scholar 

  9. Loomba RS, Shah PH, Anderson RH (2016) Radiologic considerations in heterotaxy: the need for detailed anatomic evaluation. Cureus 8:1–12

    Google Scholar 

  10. Kothari SS (2014) Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol 7:187–192

    Article  PubMed  PubMed Central  Google Scholar 

  11. Nagel BH, Williams H, Stewart L, Paul J, Stumper O (2005) Splenic state in surviving patients with visceral heterotaxy. Cardiol Young 15:469–473

    Article  PubMed  Google Scholar 

  12. Loomba RS, Anderson RH (2015) Letter to the editor regarding “Situs inversus with levocardia, infrahepatic interruption of the inferior vena cava, and azygos continuation: a case report”. Surg Radiol Anat SRA. https://doi.org/10.1007/s00276-015-1492-x

    Article  PubMed  Google Scholar 

  13. Loomba R (2015) Thrombocytosis in the setting of isomerism and a functionally univentricular heart. Cureus 7:1–10

    Google Scholar 

  14. Loomba RS, Aggarwal S, Gupta N, Buelow M, Alla V, Arora RR, Anderson RH (2015) Arrhythmias in adult congenital patients with bodily isomerism. Pediatr Cardiol. https://doi.org/10.1007/s00246-015-1281-7

    Article  PubMed  Google Scholar 

  15. Loomba RS, Willes RJ, Kovach JR, Anderson RH (2015) Chronic arrhythmias in the setting of heterotaxy: differences between right and left isomerism. Congenit Heart Dis. https://doi.org/10.1111/chd.12288

    Article  PubMed  Google Scholar 

  16. Smith A, Ho SY, Anderson RH, Connell MG, Arnold R, Wilkinson JL, Cook AC (2006) The diverse cardiac morphology seen in hearts with isomerism of the atrial appendages with reference to the disposition of the specialised conduction system. Cardiol Young 16:437–454

    Article  PubMed  Google Scholar 

  17. Loomba RS, Nijhawan K, Anderson R (2016) Impact of era, type of isomerism, and ventricular morphology on survival in heterotaxy: implications for therapeutic management. World J Pediatr Congenit Heart Surg 7:54–62

    Article  PubMed  Google Scholar 

  18. Sasaki J, Dykes JC, Sosa LJ, Salvaggio JL, Tablante MD, Ojito J, Khan DM, Hannan RL, Rossi AF, Burke RP, Wernovsky G (2016) Risk factors for longer hospital stay following the Fontan operation. Pediatr Crit Care Med. https://doi.org/10.1097/PCC.0000000000000701

    Article  PubMed  Google Scholar 

  19. Fedderly RT, Whitstone BN, Frisbee SJ, Tweddell JS, Litwin SB (2001) Factors related to pleural effusions after Fontan procedure in the era of fenestration. Circulation 104:I148–I151

    Article  CAS  PubMed  Google Scholar 

  20. Fu S, Feng ZC, Dietmar S (2010) Factors influencing pleural effusion after Fontan operation: an analysis with 95 patients. Chin Acad Med Sci 25:38–43

    Article  Google Scholar 

  21. Brown KL, Ridout DA, Goldman AP, Hoskote A, Penny DJ (2003) Risk factors for long intensive care unit stay after cardiopulmonary bypass in children. Crit Care Med 31:28–33

    Article  PubMed  Google Scholar 

  22. Pagowska-Klimek I, Pychynska-Pokorska M, Krajewski W, Moll JJ (2011) Predictors of long intensive care unit stay following cardiac surgery in children. Eur J Cardio-Thorac Surg 40:179–184

    Article  Google Scholar 

  23. Marcin JP, Slonim AD, Pollack MM, Ruttimann UE (2001) Long-stay patients in the pediatric intensive care unit. Crit Care Med 29:652–657

    Article  CAS  PubMed  Google Scholar 

  24. Pasquali SK, He X, Jacobs ML, Shah SS, Peterson ED, Gaies MG, Hall M, Gaynor JW, Hill KD, Mayer JE, Li JS, Jacobs JP (2014) Excess costs associated with complications and prolonged length of stay after congenital heart surgery. Ann Thorac Surg 98:1660–1666

    Article  PubMed  PubMed Central  Google Scholar 

  25. Newburger JW, Wypij D, Bellinger DC, du Plessis AJ, Kuban KC, Rappaport LA, Almirall D, Wessel DL, Jonas RA, Wernovsky G (2003) Length of stay after infant heart surgery is related to cognitive outcome at age 8 years. J Pediatr 143:67–73

    Article  PubMed  Google Scholar 

  26. Marino BS (2013) New concepts in predicting, evaluating, and managing neurodevelopmental outcomes in children with congenital heart disease. Curr Opin Pediatr 25:574–584

    Article  PubMed  Google Scholar 

  27. Connolly D, McClowry S, Hayman L, Mahony L, Artman M (2004) Posttraumatic stress disorder in children after cardiac surgery. J Pediatr 144:480–484

    Article  PubMed  Google Scholar 

  28. Helfricht S, Latal B, Fischer JE, Tomaske M, Landolt MA (2008) Surgery-related posttraumatic stress disorder in parents of children undergoing cardiopulmonary bypass surgery: a prospective cohort study. Pediatr Crit Care Med 9:217–223

    Article  PubMed  Google Scholar 

  29. Meadows J, Gauvreau K, Jenkins K (2008) Lymphatic obstruction and protein-losing enteropathy in patients with congenital heart disease. Congenit Heart Dis 3:269–276

    Article  PubMed  Google Scholar 

  30. Loomba RS (2015) Arterial desaturation due to pulmonary arteriovenous malformations after the Kawashima. Ann Pediatr Cardiol 9:1–4

    Google Scholar 

  31. Loomba RS (2016) Finding harmony between science and art in pediatric cardiology: acknowledging when being “objective” may not truly be objective. Children (Basel). https://doi.org/10.3390/children3040037

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Rohit S. Loomba.

Ethics declarations

Conflict of interest

None of the authors have any conflicts of interest to disclose.

Ethical Approval

All procedures performed in this study were in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The proposed methodology was approved by the Institutional Review Board at the Children’s Hospital of Wisconsin.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Loomba, R.S., Frommelt, P.C., Anderson, R.H. et al. Timing of Fontan Completion in Children with Functionally Univentricular Hearts and Isomerism: The Impact of Age, Weight, and Pre-Fontan Arterial Oxygen Saturation. Pediatr Cardiol 40, 753–761 (2019). https://doi.org/10.1007/s00246-019-02060-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-019-02060-z

Keywords

Navigation