TY - EJOU
AU - Asimacopoulos, Eleni P.
AU - Staffa, Steven J.
AU - Laussen, Peter C.
AU - Odegard, Kirsten C.
TI - A 20-Year Follow-up after the Fontan Operation in a Population with Hypoplastic Left Heart Syndrome
T2 - Congenital Heart Disease
PY - 2022
VL - 17
IS - 5
SN - 1747-0803
AB - 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.
KW - Fontan procedure; hypoplastic left heart syndrome; antiplatelet drugs; anticoagulation strategy; thrombosis
DO - 10.32604/chd.2022.020334