Abstract
Objective
Fontan takedown remains an option for the management of Fontan failure. We sought
to evaluate early and late outcomes after Fontan takedown.
Methods
The Australia and New Zealand Fontan Registry was interrogated to identify all patients
who had a Fontan takedown.
Results
Over a 43-year study period (1975-2018), 36 of 1540 (2.3%) had a Fontan takedown.
The median age at takedown was 5.1 years (interquartile range [IQR], 3.7, 7.0). Nine
(25%) patients had a takedown within 48 hours, 6 (16%) between 2 days and 3 weeks,
14 (39%) between 3 weeks and 6 months, whereas 7 (19%) had a late takedown (>6 months).
Median interval to takedown was 26 days (IQR, 1.5, 127.5). Sixteen (44%) patients
died at a median of 57.5 days (IQR, 21.8, 76.8). The greatest mortality occurred between
3 weeks and 6 months (<2 days: 1/9, 11%; 2 days to 3 weeks: 2/6, 33%; 3 weeks to 6 months:
11/14, 79%; >6 months: 2/7, 28%; P = .007). At median follow-up of 9.4 years (IQR, 4.5, 15.3), 11 (31%) patients were
alive with an intermediate circulation (10 in New York Heart Association class I/II).
Five (14%) patients underwent a successful second Fontan. Freedom from death/transplant
after Fontan takedown was 59%, 56%, and 52% at 1, 5, and 10 years, respectively.
Conclusions
The incidence of Fontan takedown is low, but mortality is high. The majority of takedowns
occurred within 6 months. Mortality was lowest when takedown occurred <2 days and
highest between 3 weeks and 6 months. A second Fontan is possible in a small proportion
of survivors.
Graphical abstract
Graphical Abstract
Key Words
Abbreviations and Acronyms:
BCPS (bidirectional cavopulmonary shunt), CI (confidence interval), DORV (double outlet right ventricle), IQR (interquartile range), NYHA (New York Heart Association), PLE (protein-losing enteropathy)To read this article in full you will need to make a payment
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References
- 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: S32-S38
- 40-year follow-up after the Fontan operation long-term outcomes of 1,052 patients.J Am Coll Cardiol. 2015; 66: 1700-1710
- Results of the Fontan operation with no early mortality in 248 consecutive patients.Kardiol Pol. 2017; 75: 255-260
- The relentless effects of the Fontan paradox.Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2016; 19: 37-43
- Management of early Fontan failure: a single-institution experience.Eur J Cardiothorac Surg. 2014; 46: 458-464
- Chylothorax and pleural effusion in contemporary extracardiac fenestrated Fontan completion.J Thorac Cardiovasc Surg. 2018; 155: 2069-2077
- Life after surviving Fontan surgery: a meta-analysis of the incidence and predictors of late death.Hear Lung Circ. 2018; 27: 552-559
- The option of taking down the Fontan circulation: the Melbourne experience.J Thorac Cardiovasc Surg. 2010; 139: 1346-1348
- The Australia and New Zealand Fontan registry: description and initial results from the first population-based fontan registry.Intern Med J. 2014; 44: 148-155
- Outcome after Fontan failure and takedown to an intermediate palliative circulation.Ann Thorac Surg. 2007; 84: 880-887
- Long-term outcomes after extracardiac Fontan takedown to an intermediate palliative circulation.Ann Thorac Surg. 2018; 105: 599-605
- The modified Fontan operation. An analysis of risk factors for early postoperative death or takedown in 702 consecutive patients from one institution.J Thorac Cardiovasc Surg. 1995; 109: 1237-1243
- Fontan operation in five hundred consecutive patients: factors influencing early and late outcome.J Thorac Cardiovasc Surg. 1997; 114: 376-391
- Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology.Heart. 2000; 83: 51-57
- Outcome of the Glenn procedure as definitive palliation in single ventricle patients.Int J Cardiol. 2020; 303: 30-35
- Catheter takedown in the management of the acutely failing Fontan circulation.Ann Thorac Surg. 2011; 92: 346-348
- Transcatheter fontan takedown.Catheter Cardiovasc Interv. 2015; 86: 849-854
- Rescue cardiac transplantation for early failure of the Fontan-type circulation in children.J Thorac Cardiovasc Surg. 2005; 129: 416-422
Article info
Publication history
Published online: October 05, 2020
Accepted:
September 19,
2020
Received in revised form:
August 28,
2020
Received:
June 9,
2020
Footnotes
The Australia & New Zealand Fontan Registry is partly funded by a National Health and Medical Research Council partnership grant (1076849). Professor D'Udekem is a Clinician Practitioner Fellow of the NHMRC (1082186).
Identification
Copyright
Crown Copyright © 2020 Published by Elsevier Inc. on behalf of The American Association for Thoracic Surgery
ScienceDirect
Access this article on ScienceDirectLinked Article
- Commentary: Fontan takedown: The journey off the beaten pathThe Journal of Thoracic and Cardiovascular SurgeryVol. 161Issue 3
- Commentary: Fontan challenges: Critical early surgical decisions maybe keyThe Journal of Thoracic and Cardiovascular SurgeryVol. 161Issue 3
- PreviewThe Fontan physiology since its inception was never meant to be ideal for the human heart. Nevertheless, it has become the final step in the path of palliating many patients with single-ventricle disease.1 Currently, the total cavopulmonary connection or Fontan procedure places the pulmonary circulation like a dam between the systemic venous return and the systemic ventricle, creating, like any dam, upstream congestion and downstream decreased flow. These 2 features are the root cause of all early and late Fontan complications.
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