One of the major causes of morbidity and mortality after the Fontan operation is thromboembolic events (TE). To assess the current knowledge of TE after Fontan surgery, a comprehensive MEDLINE search of the English literature from 1971 to 2000 was conducted using the key words Fontan, univentricular heart, children, thrombosis, congenital heart disease, cavopulmonary, and palliation. Other relevant publications were identified from bibliographies of the literature retrieved. Fifty-one studies were identified and analyzed for incidence, potential morbidity and mortality, risk factors, prophylactic options, and risk/benefit ratio of prophylactic anticoagulation as relates to TE after Fontan surgery. There were 23 case reports, 13 retrospective cohort studies that included some details about TE among other reported outcomes after Fontan procedures, eight retrospective cohort studies in which TE was the primary outcome measure, and seven articles reporting cross-sectional point surveys, only three of which directly surveyed TE as an outcome. Based on an analysis of the current literature, there is insufficient evidence to make clear recommendations about optimal anticoagulant prophylaxis at this time. Multicenter randomized controlled trials comparing prophylactic antiplatelet with anticoagulation therapies are needed to provide rational scientific guidelines for future management of Fontan patients.
Copyright 2002 by W.B. Saunders Company