Congenital – Original Submission
Twenty-Five Year Outcomes of the Lateral Tunnel Fontan Procedure

https://doi.org/10.1053/j.semtcvs.2017.06.002Get rights and content

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.

Introduction

The efficacy of the Fontan procedure in the treatment of children born with single-ventricle cardiac malformations continues to be unveiled as this population progresses further into adulthood.1 It is believed that refinements in surgical techniques have led to an improvement in outcomes since the original atriopulmonary (AP) Fontan, which has now been abandoned.2, 3 Progressive atrial distension and the development of turbulent flow within the atrial chamber predisposed recipients to tachyarrhythmias, thromboembolic events, and even compression of the pulmonary veins.4 In 1988, the concept of total cavopulmonary circulation was proposed by de Leval in an attempt to avoid turbulent flow within the atrium, and his design of the intra-atrial lateral tunnel (LT) Fontan was widely adopted.5 In the 1990s, the extracardiac conduit (ECC) progressively came into vogue and today is the most frequently performed type of Fontan.6, 7 Many patients have undergone an LT Fontan operation and the specific outcomes of this form of Fontan are unclear. In 1 U.S. multicenter database, more than a third of the Fontan operations performed between 2000 and 2009 were undertaken using the LT technique.7 Locally, a quarter of the patients with a Fontan circulation currently followed up within our region are living with an LT Fontan type.1, 8 Although the implementation of this technique has resulted in improved survival up to early adulthood, the rate of failure of their circulation is still unclear. We sought to characterize the late outcomes of the patients who have undergone an LT Fontan procedure in Australia and in New Zealand.

Section snippets

Methods

Ethical review board approvals were obtained at all participating institutions. The need for specific consent was waived because of the retrospective nature of the study.

The full design, data fields, and administration of the Australia and New Zealand Fontan Registry have been described previously.1, 8 Of the 1462 participants whose data were collected in the Registry database between January 1, 1975, and August 1, 2015, all those who underwent an LT Fontan were identified. Fontan conversions

Results

A total of 301 patients were identified from hospital and surgical databases. Patient characteristics are detailed in Table 1.

Discussion

More than 25 years after its original description, the LT modification of the originally described Fontan procedure has demonstrated its superiority over the former AP Fontan. The 25-year survival of the patients discharged from the hospital with an intact LT circulation has reached a remarkable 85% within our population.

Estimates of late survival from the Mayo Clinic of 84%, 70%, and 39% at 10, 20, and 30 years after the LT Fontan, respectively, are less optimistic than our current series. The

Conclusion

Over a 25-year period, the LT technique has achieved excellent late survival. As this population ages, it faces an increasing risk of failure and adverse events, including arrhythmia and thromboembolism. We are likely to see a growing number of these survivors requiring heart transplantation and late reintervention. Efforts must be made to develop treatment strategies for the increasing number of Fontan patients who will continue to experience attrition over time.

Acknowledgments

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

References (18)

There are more references available in the full text version of this article.

Cited by (22)

  • Coagulation and Anticoagulation in Fontan Patients

    2022, Canadian Journal of Cardiology
    Citation Excerpt :

    Several authors have attempted to report time-adjusted risk of TEs after the Fontan operation, although the results are quite divergent. Reported TE rates varied between 0.74% and 5.2% per patient-year, and cumulative TE incidence at 10 years ranged from 2% to 25% (Table 1, Fig. 1).2-16 TEs are subdivided according to location in the circulation.

  • The optimal Fontan operation: Lateral tunnel or extracardiac conduit?

    2021, Journal of Thoracic and Cardiovascular Surgery
  • Atrial pacing in Fontan patients: The effect of transvenous lead on clot burden

    2021, Heart Rhythm
    Citation Excerpt :

    Consistent with our findings, previous studies demonstrated that type of Fontan and thromboprophylaxis agents but not atrial arrhythmia were the main predictors of clots.14 In contrast, history of atrial arrhythmia was found to incur a 2–3 times higher risk of thromboembolic events in previous observational studies.12,18,19 It is important to point out that in 2 of these studies, the investigators did not adjust for anticoagulation or antiplatelet therapy in their multivariable model.

  • Hypoplastic left heart syndrome: From bedside to bench and back

    2019, Journal of Molecular and Cellular Cardiology
View all citing articles on Scopus

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.

Yves d'Udekem is a consultant for MSD and Actelion. Andrew Bullock reports receiving consulting fees from Actelion. All the other authors have no commercial interests to disclose.

View full text