摘要
Background:Thromboembolic events are a cause of significant morbidity and mortality in the Fontan population.We previously reported on coagulation profile changes in a cohort of patients with hypoplastic left heart syndrome(HLHS)from Stage I through Fontan completion.In this report,we examine their clinical status,anticoagulation and incidence of thromboembolic events up to 20 years post Fontan.Methods:A retrospective chart review was conducted for twenty(20)surviving patients,from 1998 through December 2020.Patients who underwent orthotopic heart transplantation(OTx)were followed until their transplant.Patients who were found in the original study to have a factor VIII activity level>160%,were examined separately.Results:Most patients had follow-up within the last two years(2018–2020).Two patients underwent OTx and two patients died.Anticoagulation strategy was variable.Most patients were on aspirin monotherapy.There was a total of twelve thrombotic events(63.2%).These included six cerebrovascular accidents(two of which were fatal).Three out of the seven patients with elevated factor VIII activity from the original study had thromboembolic events(42.9%).Fontan complications were variable.Some degree of Fontan Associated Liver Disease was universal.Conclusions:This retrospective review of a group of single-ventricle patients post Fontan,illustrates the variability in anticoagulation therapy that exists in this population.A large proportion of patients suffered a significant thromboembolic event,including the patients with elevated factor VIII.Further investigation into the patients with elevated factor VIII may help determine whether a different antithrombotic strategy post Fontan would be beneficial.
