Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis

Abstract

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/m2). 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.

Elevated ventricular filling pressures and long‐term survival in adults post‐Fontan

“…As expected given the age group, atriopulmonary connections were common in our cohort; the correlation between PAWP and VEDP might be different in other types of Fontan connections due to the iatrogenic large atrial septal defect/larger left atrial volumes. We acknowledge the pitfalls and challenges in PVR calculation in Fontan patients but, for consistency purposes, we applied the method used in a previous publication by our group . Lastly, we were unable to assess predictors of Fontan‐related deaths due to the number of patients with unknown cause of death.…”

“…We acknowledge the pitfalls and challenges in PVR calculation in Fontan patients but, for consistency purposes, we applied the method used in a previous publication by our group. 21 Lastly, we were unable to assess predictors of Fontan-related deaths due to the number of patients with unknown cause of death. However, we expect the percentage of noncardiac deaths to be small and that predictors of cardiac death would mirror those of overall mortality.…”

Efficacy of phosphodiesterase type 5 inhibitors in univentricular congenital heart disease: the SV‐INHIBITION study design

“…In addition, a TPG above 5 mmHg avoids enrolling patients with isolated ventricular or atrio‐ventricular valve dysfunction. Indeed, diastolic ventricular dysfunction seems to be aggravated by pulmonary vasodilators and may explain discrepancies in the results observed in previous studies . We purposely did not use pulmonary vascular resistance to define patients eligible to the SV‐INHIBITION trial, given the known difficulties to accurately measure pulmonary blood flow in SV physiology.…”

“…Cardiac index corresponds to Qs divided by body surface area. For Qp calculation, the Ao‐Sat will be used or an assumed pulmonary vein saturation of 95% in case of veno‐venous collaterals or fenestration . Indexed pulmonary vascular resistance (PVRi) will be calculated as follows: PVRi = (mPAP − pulmonary capillary wedge pressure (PCWP)) ÷ (body surface area × Qp).…”

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“…The Fontan operation is associated with significant long‐term morbidity and mortality. Miranda et al . divided 84 adult Fontan patients into four different haemodynamic profiles based on Fontan pressures and cardiac index.…”