Non-vitamin K antagonist oral anticoagulants in adults with a Fontan circulation: are they safe

Abstract

BackgroundIn Fontan patients with atrial arrhythmias (AA), non-vitamin K antagonist oral anticoagulants(NOACs) have a class III recommendation according to the Pediatric & Congenital Electrophysiology Society (PACES)/Heart Rhythm Society (HRS) guideline in 2014, due to lack of data on outcomes as opposed to evidence of harm. To address this gap in data, we investigated the safety and efficacy of NOACs in adults with a Fontan circulation in a worldwide study.MethodsThis is an international multicentre prospective cohort study, using data from the NOTE (non-vitamin K antagonist oral anticoagulants for thromboembolic prevention in patients with congenital heart disease) registry. The study population comprised consecutive adults with a Fontan circulation using NOACs. Follow-up took place at 6 months and yearly thereafter. The primary endpoints were thromboembolism and major bleeding. Secondary endpoint was minor bleeding.ResultsFrom April 2014 onward, 74 patients (mean age 32±10 years (range 18–68), 54% male) with a Fontan circulation using NOACs were included. During a median follow-up of 1.2 (IQR 0.8–2.0) years, three thromboembolic events (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) and three major bleedings (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) occurred in five atriopulmonary Fontan and one total cavopulmonary connection Fontan patients with AA. Fifteen patients experienced minor bleeding episodes (15.8 per 100 patient-years (95% CI 9.1 to 25.2)). In patients (n=37) using vitamin K antagonists (VKAs) prior to the initiation of NOAC, annual incidence of historical thromboembolic events and major bleeding were 2.4% (95% CI 0.4% to 7.4%) (n = 2) and 1.2% (95% CI 0.7% to 5.1%) (n = 1), respectively.ConclusionsIn this review of the largest Fontan cohort using NOACs with prospective follow-up, NOACs appear to be well tolerated and their efficacy and safety during short-term follow-up seem comparable to VKAs. Longer term data are required to confirm these promising short-term results.

Recurrent Episodes of ST-Elevation Myocardial Infarction in a 19-Year-Old Male with Fontan Circulation

“…The guidelines however do not specifically recommend the use of DOAC at present time given the scarcity of evidence in this area. Despite that, the published literature supports the safety and non-inferiority of DOAC when compared to warfarin in Fontan circulation patients [7][8][9][10][11].…”

Managing the Adult Congenital Heart Disease Patient in the COVID-19 Pandemic—A New York Perspective

“…The ACHD guidelines recommend anticoagulation with vitamin K antagonists (VKA) in adults with Fontan palliation with known or suspected thrombus, thromboembolic events, or prior atrial arrhythmia, and no contraindications to anticoagulation [3]. Given the challenges of VKA monitoring during the pandemic, and based on a recent large international multi-center prospective study demonstrating safety and efficacy of non-vitamin K antagonist oral anticoagulants (NOACs) in Fontan patients, NOACs may be considered instead of VKAs on a case by case basis [16].…”

Non-vitamin K Antagonist Oral Anticoagulants in Adult Congenital Heart Disease: A Single Center Study

“…On other hand, while major bleeding events are not dependent on ACHD severity [7], the HAS-BLED has shown a strong correlation with major bleeding. Notably, patients with Fontan circulation appear to be at higher risk of both thromboembolic and bleeding events, including patients with total cavopulmonary connection (TCPC) [5][6][7][8].…”