Rato J; Pediatric Cardiology Department, Hospital de Santa Cruz
– Unidade Local de Saude Lisboa
Cordeiro S; Anjos R

Cardiology in the Young. 36(2):332-337, 2026 Feb.

INTRODUCTION: The Fontan procedure enables survival in individuals with
univentricular physiology but is associated with progressive circulatory
failure. Identifying predictors of adverse outcomes is essential to
improve long-term management. This study evaluated five-year outcomes and
baseline predictors of major events in a previously characterised
paediatric and young adult Fontan cohort.

METHODS: This retrospective longitudinal study included 51 patients
(median age 18 years, interquartile range 11) who underwent comprehensive
evaluation between 2018 and 2019, including echocardiography with atrial
strain analysis and cardiopulmonary exercise testing. The composite
outcome comprised death, heart transplantation listing, hospitalisation
for heart failure, or conduit thrombosis. Univariable and multivariable
logistic regression identified predictors of adverse outcomes.

RESULTS: During a five-year follow-up, 7 patients (14%) met the composite
outcome. Those with events had lower body mass index (19.8 kg/m2 [4.1] vs
16.9 [3.6]; p = 0.007), lower atrial conduit strain (10.95% [8.95] vs 2.8
[6.2]; p = 0.011), reduced peak oxygen uptake, and higher VE/VCO2 slope.
In multivariable analysis, lower body mass index (OR 0.49, 95% CI
0.26-0.93; p = 0.028) and reduced atrial conduit strain (OR 0.70, 95% CI
0.51-0.96; p = 0.026) were independently associated with adverse outcome,
with excellent model discrimination (AUC = 0.95).

DISCUSSION: Both nutritional status and atrial functional parameters
demonstrated a strong association with the outcome. Reduced atrial conduit
strain, reflecting diastolic dysfunction, and lower body mass index,
possibly reflecting myopenia or cachexia, identified higher-risk patients
and potential areas for intervention. Routine assessment of atrial
function and nutritional status should be integrated into clinical
surveillance and risk stratification of the Fontan population.