Altered endothelial function following the Fontan procedure

Abstract

Our data suggest that patients with the Fontan circulation may have endothelial dysfunction, as indicated by raised levels of von Willebrand factor. Fibrinolysis seems to be relatively preserved, as suggested by appropriate response to venous occlusion.

Aspirin resistance in adult patients after Fontan surgery

“…Significant endothelial activation was present in our Fontan patients, which was reflected by increased plasma sVCAM-1, syndecan-1 vWF, and endothelin-1 concentrations, a result that is in agreement with those of previous studies (7,8). FC is characterized by loss of pulsatility in the pulmonary circulation, which may lead to an abnormal shear stress on the vessel walls and consequent endothelial activation (8,33).…”

“…Indeed, changes in plasma activity of coagulation factors and inhibitors, endothelial dysfunction, and enhanced platelet activation have been reported in children after Fontan surgery (5)(6)(7). However, only a few studies of hemostatic abnormalities in adult patients, late after Fontan surgery (8)(9)(10), have been reported.…”

Task Force 6: Pediatric Cardiology Fellowship Training in Adult Congenital Heart Disease

“…Pediatric cardiology fellowship training should include an understanding of the multiple factors that contribute to ventricular dysfunction in adults with CHD, 6,7 including abnormal vascular function, particularly in those with Fontan physiology. 8,9 Training should emphasize ACHD patients who are at significant risk of heart failure, particularly those with systemic right ventricles, including Mustard/Senning repairs of TGA and congenitally corrected TGA, as well as patients with a single ventricle who have undergone a Fontan procedure. 10 They should also understand the neurohormonal abnormalities that frequently accompany heart failure in these adults.…”

Management of the Fontan circulation in the adult patient

“…(86)(87)(88) By adult life the gains in ventricular function are largely lost with around a third of reported patients having moderate or severe ventricular function. (89) There are many plausible theories as to why late ventricular function occurs, with increasing systemic vascular resistance, due to abnormal vascular tone, (90) endothelial dysfunction, (91)(92)(93) and the abnormal resistances encountered by the pulmonary and systemic circulations running in series (6) all probably playing a part. The onset of significant valvular incompetence and the onset of atrial arrhythmias are both poorly tolerated.…”