Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis

Aims The Fontan operation is associated with significant long-term morbidity and mortality, posing challenges in management. No haemodynamic classification has been established to aid management and prognostication of patients with Fontan palliation. Our aim was to assess haemodynamic profiles for Fontan patients and evaluate their relationships to prognosis. Methods and results Eighty-four adult Fontan patients without obstruction or significant valve disease undergoing cardiac catheterization for Fontan-related co-morbidities were divided into four different haemodynamic profiles based on normal/high Fontan pressures (FP) (< 15 or >= 15 mmHg) and low/normal cardiac index (CI) (< 2.5 or >= 2.5 L/min/m(2)). Fourteen patients had low CI/high FP (17%), 24 low CI/normal FP (29%), 20 normal CI/high FP (24%), and 26 normal CI/normal FP (31%). Demographic and clinical data were similar among groups except for a trend towards a higher prevalence of cirrhosis in the normal CI/high FP (50%) group. Systemic vascular afterload, assessed by systemic vascular resistance and effective arterial elastance, was higher in patients with low CI/high FP and low CI/normal FP as compared to normal CI profiles. Pulmonary vascular resistance indices were higher in patients with low CI/high FP than all other profiles. The normal CI/high FP haemodynamic profile was an independent predictor of mortality (hazard ratio 4.1, 95% confidence interval 1.2-11.5). Heterotaxy and protein-losing enteropathy were also predictors of mortality on the multivariate model. Conclusion The proposed haemodynamic classification provides prognostic information in adults post-Fontan with patients in the normal CI/high FP profile having worse survival. Whether this classification can guide management in these patients deserves further investigation.