Key result
Extracardiac Fontan patients face ~2% annual rates of reoperation and arrhythmia alongside low late mortality.
Why the study?
Fontan palliation improves survival but causes lifelong morbidity, and long-term outcomes including conduit changes in extracardiac Fontan patients were not systematically synthesized.
What are the long-term adverse event rates in patients who underwent extracardiac Fontan palliation?
Meta-Analysis
What are the long-term adverse event rates in patients who underwent extracardiac Fontan palliation?
Extracardiac Fontan patients experience significant long-term morbidity, including arrhythmias, reoperations, and conduit obstruction, highlighting the need for standardized evaluation approaches as this population ages.
Necessitates standardized long-term surveillance in aging extracardiac Fontan patients; leaves open optimal risk-stratification strategies.
BACKGROUND: Although Fontan palliation improves survival, it poses a large burden of lifelong morbidity. In extracardiac Fontan (ECF) patients, for example, conduit stenosis developing over time has been associated with Fontan failure and other adverse outcomes. This systematic review, for the first time, synthesized existing data on various long-term outcomes of ECF patients, including change in conduit and associated outcomes. METHODS: We searched the Medline, Embase, and Cochrane indexes from inception to 2023 and included studies reporting separate results for ECF patients with a follow-up time of at least 3 years. Full-text studies were assessed for risk of bias. We summarised information on the study, patient characteristics, and outcomes narratively and with the use of descriptive tables. Meta-analysis was performed to calculate pooled incidence rates of adverse events. RESULTS: We included 61 full-text studies, with most studies published after 2009 (77.0%) and using a retrospective cohort design (76.2%). The pooled incidence rates per 100 person-years were for arrhythmia 1.8 (95% confidence interval [CI] 1.3-2.6), thrombotic events 0.2 (95% CI 0.1-0.4), protein-losing enteropathy 0.7 (95% CI 0.5-1.0), conduit obstruction 0.7 (95% CI 0.3-1.5), reoperation 1.9 (95% CI 1.1-3.4), and late death 0.3 (95% CI 0.2-0.5). The ranges of hemodynamic and exercise parameters and findings from studies reporting changes in conduit size and liver disease were reported. CONCLUSIONS: Although many studies have largely focused on long-term survival, several other adverse outcomes require further research to develop consensus-based definitions and approaches for evaluations, especially as the ECF population ages. (PROSPERO: CRD42024533080).
No takes yet. Share an insight, caveat, or question.
Talla et al. (2025) conducted a meta-analysis in Extracardiac Fontan. Extracardiac Fontan conduit was evaluated on Incidence of adverse events (arrhythmia, thrombotic events, protein-losing enteropathy, conduit obstruction, reoperation, late death). In extracardiac Fontan patients, pooled incidence rates per 100 person-years were 1.9 for reoperation, 1.8 for arrhythmia, 0.7 for conduit obstruction, and 0.3 for late death.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:
More on Congenital heart disease adultsMore from Canadian Journal of Cardiology