Diagnosis of occult diastolic dysfunction late after the Fontan procedure using a rapid volume expansion technique

Objectives Diastolic dysfunction (DD), a key driver of long-term Fontan outcomes, may be concealed during standard haemodynamic evaluation. We sought to identify Fontan patients with occult DD using 'ventricular stress testing' with rapid volume expansion (RVE). Methods Cardiac catheterisation with RVE was performed routinely in Fontan patients between 11/2012 and 4/2015. Baseline and post-stress haemodynamic data were compared using t test, Mann-Whitney U test,.2 and Fisher's exact tests. A post-stress ventricular end diastolic pressure (EDP) threshold of 15 mm Hg defined occult DD. Results Forty-six Fontan patients (48% female, median age 14.1 (IQR 9.1 to 21.3) years) were included. The median Fontan duration was 10.8 (IQR 5.1 to 17.8) years and dominant left ventricular morphology was present in 63% of patients. Volume expansion increased mean Fontan pressure (15.2 +/- 2.5 vs 12.4 +/- 2.2 mm Hg, p<0.001), pulmonary capillary wedge pressure (11.3 +/- 2.6 vs 7.9 +/- 2 mm Hg, p<0.001) and EDP (12.7 +/- 3.3 vs 8.5 +/- 2.1 mm Hg, p<0.001). Sixteen patients (35%) had occult DD, demonstrating higher baseline EDP (10.3 +/- 1.9 vs 7.6 +/- 1.5 mm Hg, p<0.001) and greater increase in EDP (6.3 +/- 2.4 vs 3.1 +/- 1.4 mm Hg, p<0.001) compared with patients without DD. Higher baseline EDP, lower baseline cardiac index and longer duration of Fontan circulation were associated with higher post-stress EDP. There were no complications related to RVE. Conclusions Ventricular stress testing by RVE is feasible, safe and identifies a subgroup of Fontan patients with occult DD. Higher baseline EDP and longer duration of Fontan circulation are associated with worse diastolic function. Future work is necessary to better understand the aetiology, associations and clinical implications of occult DD in Fontan survivors.