Impaired vascular function in patients with Fontan circulation.

Background: This study was performed to evaluate the endothelial function in Fontan patients, and to investigate the associated factors which influence the endothelial function in these patients.

Methods: Flow mediated dilatation (FMD) and nitroglycerin induced dilatation (NG) of the brachial artery, and intima media thickness (IMT) of common carotid artery (CCA) were measured in nonselected Fontan patients (n=44, age 5 to 29 years, median 14 years, 18 females) by means of high-resolution ultrasound according to the standard protocols. The results were compared to age and sex matched controls (n=25, age 5 to 27 years, median 13 years, 10 females).

Results: Fontan patients presented significantly reduced FMD and NG compared with controls (FMD; 6.5+/-2.4 vs. 11.1+/-1.4%, p<0.001, NG; 13.3+/-5.2 vs. 19.4+/-6.2%; p=0.035). Twenty two percent of Fontan patients presented pure endothelial dysfunction, and 34% of patients presented combined endothelial and smooth muscle dysfunction. Although there was no correlation between FMD and IMT, Fontan patients presented increased carotid IMT (0.44+/-0.07 vs. 0.38+/-0.06 mm, p=0.008). In multivariate analysis, duration of exposure to chronic hypoxia was inversely correlated with FMD (p=0.117, hazards ratio=1.294, 95% confidence interval=0.938-1.786). There was higher FMD in patients receiving angiotensin-converting enzyme inhibitors (ACEi) compared with those not receiving ACEi (7.0+/-2.5 vs. 5.5+/-2.2%, p=0.069).

Conclusions: Endothelial dysfunction is more prevalent in Fontan patients compared with healthy controls, and the previous hypoxia is an independent factor. Although it is not statistically significant, those patients on treatment with ACEi seem to have better endothelial function.