Outcome of Early and Late Onset Fontan Operation in Patients with Univentricular Heart Repair

Shahnawaz Sathio; Shakeel Ahmed; Mehtab Hussain; Nimra Zahid; Saad Ullah Siddiqui; Fazal ur Rehman

doi:10.51253/pafmj.v74i4.8006

Authors

Shahnawaz Sathio Department of Pediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan
Shakeel Ahmed Department of Pediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan
Mehtab Hussain Department of Pediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan
Nimra Zahid Department of Pediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan
Saad Ullah Siddiqui Department of Pediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan
Fazal ur Rehman Department of Pediatric Cardiology, Parvez Elahi Institute of Cardiology Bahawalpur

DOI:

https://doi.org/10.51253/pafmj.v74i4.8006

Keywords:

Fontan surgery, mortality, oxygen saturation, univentricular heart.

Abstract

Objective: To determine the outcome of early and late-onset Fontan operation in patients with univentricular heart repair.

Study Design: Quasi-experimental study.

Place and Duration of the Study: Department of Paediatric Cardiology, National Institute of Cardiovascular Diseases, Karachi Pakistan, from Jan 2019 to Dec 2021.

Methodology: During the study period, 121 patients of both genders, up to any age, with functionally univentricular hearts and who planned to undergo Fontan procedures were included. All cases were divided into two groups, Group-A had patients who underwent extra-cardiac Fontan at age ≤6 years, while Group B had patients who underwent extra-cardiac Fontan at age >6 years. Pre-procedure diagnosis, demographic, anatomic and hemodynamic characteristics, post-Fontan procedure morbidity and mortality were noted.

Results: As per inclusion/exclusion criteria, 121 cases underwent Fontan surgery during the study period. There were 61(50.4%) male patients. Overall, the mean age was 7.02±4.6 years. Seventy-six 76(61.2%) cases were in Group-A and 45(38.8%) in Group-B. Mortality (within 30 days post-surgery) was reported in 11(9.1%) patients. Except for the mean duration of chest tube drainage ((0.010), there was no difference in terms of post-Fontan surgery outcomes between both study groups (p>0.05).

Conclusion: The age of the patients at the time of the Fontan procedure did not affect the outcomes.

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References

Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971; 26(3): 240–248. https://doi.org/10.1136/thx.26.3.240

de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg 1988; 96(5): 682-695.

Balaji S, Gewillig M, Bull C, de Leval MR, Deanfield JE.

Arrhythmias after the Fontan procedure. Comparison of total cavopulmonary connection and atriopulmonary connection. Circulation 1991; 84(5 Suppl): III162-67.

Kverneland LS, Kramer P, Ovroutski S. Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation. Congenit Heart Dis 2018; 13(2): 181-193. https://doi.org/10.1111/chd.12570

Caneo LF, Neirotti RA, Turquetto AL, Jatene MB. The Fontan operation is not the end of the road. Arq Bras Cardiol 2016; 106(2): 162-165. https://doi.org/10.5935/abc.20160017

Trusty PM, Slesnick TC, Wei ZA, Rossignac J, Kanter KR, Fogel MA, et al. Fontan Surgical Planning: Previous Accomplishments, Current Challenges, and Future Directions. J Cardiovasc Transl Res 2018; 11(2): 133-144.

https://doi.org/10.1007/s12265-018-9786-0

Fredenburg TB, Johnson TR, Cohen MD. The Fontan procedure: anatomy, complications, and manifestations of failure.

Radiographics 2011; 31(2): 453-463.

https://doi.org/10.1148/rg.312105027

Pizarro C, Mroczek T, Gidding SS, Murphy JD, Norwood WI. Fontan completion in infants. Ann Thorac Surg 2006; 81(6): 2243-2248. https://doi.org/10.1016/j.athoracsur.2006.01.016

Veldtman GR, Nishimoto A, Siu S, Freeman M, Fredriksen PM, Gatzoulis MA, et al. The Fontan procedure in adults, Heart 2001; 86(3): 330–335.

https://doi.org/10.1136/heart.86.3.330

Deraz S, Ismail MF, Jamjoom A. Outcome of early and late onset Fontan operation in patients with univentricular heart repair. Egyptian Heart J 2014; 66(2): 171-176.

https://doi.org/10.1016/j.ehj.2013.06.001

Bezuska L, Lebetkevicius V, Sudikiene R, Liekiene D, Tarutis V. 30-year experience of Fontan surgery: single-centre's data. J Cardiothorac Surg 2017; 12(1): 67.

https://doi.org/10.1186/s13019-017-0634-0

Firdouse M, Agarwal A, Chan AK, Mondal T. Thrombosis and thromboembolic complications in Fontan patients: a literature review. Clin Appl Thromb Hemost 2014; 20(5): 484-492.

https://doi.org/10.1177/1076029613520464

Raj S, Rosenkranz E, Sears B, Swaminathan S. Intermediate-term results after extracardiac conduit Fontan palliation in children and young adults with single ventricle physiology-A single-center experience. Pediatr Cardiol 2016; 37(6): 1111-1118.

https://doi.org/10.1007/s00246-016-1402-y

Cazzaniga M, Fernández Pineda L, Villagrá F, Pérez De León J, Gómez R, Sánchez P, et al. Single-stage Fontan procedure: early and late outcome in 124 patients. Rev Esp Cardiol 2002; 55(4): 391-412. https://doi.org/10.1016/s0300-8932(02)76619-0

Ota N, Fujimoto Y, Murata M, Tosaka Y, Ide Y, Tachi M, et al. Impact of postoperative hemodynamics in patients with

functional single ventricle undergoing Fontan completion before weighing 10 kg. Ann Thorac Surg 2012; 94(5): 1570-1577.

https://doi.org/10.1016/j.athoracsur.2012.06.022

Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, et al. 40-Year Follow-Up After the Fontan Operation: Long-Term Outcomes of 1,052 Patients. J Am Coll Cardiol 2015; 66(15): 1700-1710. https://doi.org/10.1016/j.jacc.2015.07.065

d'Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, et al. Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation 2014; 130(11 Suppl 1): S32-38.

https://doi.org/10.1161/CIRCULATIONAHA.113.007764

Ono M, Kasnar-Samprec J, Hager A, Cleuziou J, Burri M, Langenbach C, et al. Clinical outcome following total

cavopulmonary connection: a 20-year single-centre experience. Eur J Cardiothorac Surg 2016; 50(4): 632-641.

https://doi.org/10.1093/ejcts/ezw091

Driscoll DJ, Offord KP, Feldt RH, Schaff HV, Puga FJ, Danielson GK. Five- to fifteen-year follow-up after Fontan operation. Circulation 1992; 85(2): 469-496.

https://doi.org/10.1161/01.cir.85.2.469

Rychik J. The relentless effects of the Fontan paradox. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2016; 19(1): 37-43. https://doi.org/10.1053/j.pcsu.2015.11.006

Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 2007; 115(2): 163-172. https://doi.org/10.1161/CIRCULATIONAHA.106.627224

Schilling C, Dalziel K, Nunn R, Du Plessis K, Shi WY, Celermajer D, et al. The Fontan epidemic: Population projections from the Australia and New Zealand Fontan Registry. Int J Cardiol 2016; 219: 14-19. https://doi.org/10.1016/j.ijcard.2016.05.035