Twenty-Five Year Outcomes of the Lateral Tunnel Fontan Procedure

The Australia and New Zealand Fontan Registry

Research output: Contribution to journalArticlepeer-review

23 Scopus citations

Abstract

The objective of this study was to characterize late outcomes of the lateral tunnel (LT) Fontan procedure. The outcomes of all patients who underwent an LT Fontan procedure in Australia and in New Zealand were analyzed. Original files were reviewed and outcomes data were obtained through a binational registry. Between 1980 and 2014, a total of 301 patients underwent an LT Fontan procedure across 6 major centers. There were 13 hospital mortalities, 21 late deaths, 8 Fontan conversions and revisions, 8 Fontan takedowns, and 4 heart transplantations. Overall survival at 15 and 25 years was 90% (95% confidence interval [CI]: 86%-93%) and 80% (95% CI: 69%-91%), respectively. Protein-losing enteropathy or plastic bronchitis was observed in 14 patients (5%). Freedom from late failure at 15 and 25 years was 88% (95% CI: 84%-92%) and 82% (95% CI: 76%-87%), respectively. Independent predictors of late Fontan failure were prolonged pleural effusions post Fontan operations (hazard ratio [HR] 3.06, 1.05-8.95, P = 0.041), age >7 years at Fontan (vs 3-5 years, HR 9.7, 2.46-38.21, P = 0.001) and development of supraventricular tachycardia (HR 4.67, 2.07-10.58, P < 0.001). Freedom from tachy- or bradyarrhythmias at 10 and 20 years was 87% (95% CI: 83%-91%) and 72% (95% CI: 66%-79%), respectively. Thromboembolic events occurred in 45 patients (16%, 26 strokes), and freedom from symptomatic thromboembolism at 10 and 20 years was 93% (95% CI: 89%-96%) and 80% (95% CI: 74%-86%), respectively. Over a 25-year period, the LT technique has achieved excellent late survival. As this population ages, it is at an increasing risk of failure and adverse events. We are likely to see an increasing proportion requiring heart transplantation and late reintervention.

Original languageEnglish (US)
Pages (from-to)347-353
Number of pages7
JournalSeminars in thoracic and cardiovascular surgery
Volume29
Issue number3
DOIs
StatePublished - Feb 3 2017

Funding

The authors thank the Murdoch Childrens Research Institute for infrastructure support. The authors also acknowledge the Fontan Registry management and research assistants for their invaluable support in the creation and maintenance of the Registry, as well as in the data gathering for this paper, and Belinda Bortone for administrative support. The authors also acknowledge support provided to the Murdoch Childrens Research Institute by the Victorian Government's Operational Infrastructure Support Program. The Australia and New Zealand Fontan Registry is funded by grants from the National Health and Medical Research Council (NHMRC; Project Grants 1012241, 1047923, and 1065794). Dr William Shi is supported by the Royal Australasian College of Surgeons Foundation for Surgery Peter King/Heart Foundation Research Scholarship in addition to the University of Melbourne Viola Edith Reid and the RG and AU Meade Scholarships. Yves d'Udekem is an NHMRC Clinician Practitioner Fellow (1082186). The Victorian Government's Operational Infrastructure Support Program supported this research project.

Keywords

  • Fontan procedure
  • congenital heart defects
  • follow-up studies
  • heart ventricle abnormalities
  • retrospective studies
  • survival rate

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

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