Objective More patients with Fontan physiology are reaching adulthood. The purpose of this meta-analysis was to evaluate the late outcomes of patients palliated with Fontan procedure and to assess the risk factors for mortality.
Methods PubMed, Embase and Web of Science were queried to retrieve observational studies of survival in patients following the Fontan procedure with ≥5 years of follow-up. A random-effects model was used to determine pooled survival estimates at 5, 10 and 15 years. Meta-regression was used to assess potential moderators for death.
Results Nineteen articles with a total of 5859 patients were included. The weighted mean follow-up time was 8.94±2.64 years with overall 8.3% deaths and 1.5% transplants. Pooled survival estimates at 5, 10 and 15 years were 90.7%, 87.2% and 87.5%, respectively; and 88.4%, 85.7% and 84.1%, respectively, for studies that included all three time intervals (n=4). Earliest surgical year included in the study, proportion of atriopulmonary connections versus extracardiac conduit or lateral tunnel, and older age at Fontan were associated with higher rates of death, but ventricular morphology was not. Protein-losing enteropathy, reoperation and pacemaker insertion were reported in 2.1%, 5.6% and 6.8% patients, respectively.
Conclusions Survival following the Fontan procedure has improved with time and is influenced by Fontan type and age at the time of Fontan. At a mean follow-up of 8.9 years, there was no significant association between survival and ventricular morphology, not taking into account the mortality prior to Fontan.
- single ventricle
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Contributors Each author contributed significantly to the manuscript and met recommendations for authorship as defined by ICJME. IS: principal investigator of the study. Involved with the conception and design of the study. Extraction of the data from studies for meta-analysis and interpretation of the results, drafting the original manuscript, revising it based on the feedback from coauthors. CEM: helped with study design and performing the meta-analysis. Interpreted the results of the meta-analysis. Wrote the statistics section of the methods, critically revised the manuscript and approved the final submission. CJP: coauthor of the study, involved with the study design, interpretation of the data, reviewing the original and final manuscript and providing feedback and approving the final submission. RS: senior author, involved with the conception and design of the study, extraction of the data from studies for meta-analysis and interpretation of the results, revising multiple versions of the manuscript, and finalising and submitting the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Not required.
Ethics approval Institutional Review Board of Children’s Healthcare of Atlanta.
Provenance and peer review Not commissioned; externally peer reviewed.