Anticoagulation management in adult patients with congenital heart disease: a narrative review

Abstract

With improvements in treatment of congenital heart disease more paediatric patients are surviving with palliative or corrective interventions during childhood, thus becoming adults with congenital heart disease (ACHD). Overall, the ACHD population is at a higher risk of arrhythmias and stroke. The abnormal structure and function of their corrected hearts suggests that in addition to established stroke risk factors, such as prior stroke or older age, additional stroke risk factors need to be considered to determine the risk and establish the indication for oral anticoagulation (OAC) in ACHD patients. In structurally normal hearts non-vitamin-K oral anticoagulants (NOACs) offer at least equal stroke prevention with a better safety profile compared to vitamin K antagonists (VKA) in patients with atrial fibrillation (AF) or pulmonary embolism. Current guidelines recommend NOACs in ACHD patients with simple lesions and indication for OAC, while there is less certainty about their safety in ACHD patients with moderate or complex congenital heart disease such as patients with transposition of the great arteries (TGA) after atrial switch operation (Senning or Mustard operation), Fontan circulation or congenital corrected transposition of the great arteries (ccTGA). This review summarises the available evidence characterising stroke risk in patients with ACHD and the use of anticoagulants and interventional therapies to reduce that risk.

Successful Intravenous Thrombolytic Therapy in a Patient with Tetralogy of Fallot Presenting with Stroke

“…Treatment of paradoxical emboli should consider both clot prevention and shunt correction [6]. It is well described in the literature that vitamin K antagonists would be preferred in cyanotic heart disease as there is insufficient safety and efficacy data to support routine non-vitamin K antagonist oral anticoagulant [7,8]. Treatment with intravenous recombinant tissue plasminogen (tPA) should also be performed in suitable patients who present later (6-12 hours).…”

Thromboembolic Complications in Adult Patients Following Fontan Procedure—A Multicenter Study

“…Therefore, there is a need to search for high-risk TEC patients in the FO population actively. The CHA 2 DS 2 -VASc score, as a simple and easy-to-use tool, is used in daily practice to establish TEC risk and, eventually, the implementation of therapy in patients with atrial fibrillation diagnosis; however, its usefulness is unclear in the adult congenital heart disease population [33]. Of note, FO subjects are younger compared to the typical population of patients with atrial fibrillation and have a higher TEC risk due to FO circulation.…”

Case Report: Residual Atrial Shunt Lesions in Aging Adults With Congenital Heart Disease: An Underestimated Risk of Stroke?

“…The majority of events occur in patients with cyanotic defects and in those without sinus rhythm ( 4 ). Arrhythmias are common in ACHD patients ( 5 ), in particular there is a high prevalence of atrial arrhythmias ( 6 , 7 ). While paradoxical embolism via a patent foramen ovale (PFO) is associated with cryptogenic stroke in the general population and PFO closure reduces the rate of subsequent events in selected patients ( 8 – 10 ), up to now cryptogenic stroke has not been investigated systematically in ACHD patients.…”