Anticoagulation for Thromboembolic Risk Reduction in Adults With Congenital Heart Disease

Adults with congenital heart disease (ACHD) represent one of the fastest expanding groups of patients in contemporary practice with unique and often complex management pathways. ACHD have a higher risk of thromboembolism not only from their complex physiology and anatomy but also from substrates such as valvular heart disease, atrial arrhythmias, cyanosis, and ventricular dysfunction, resulting in significant morbidity and mortality. Thromboembolic event rates in ACHD have been shown to be related to Congestive Heart Failure, Hypertension, Age (≥75 years), Diabetes, Stroke/Transient Ischemic Attack, Vascular Disease, Age (65-74 years), Sex (Female) (CHA₂DS₂-VASc) scores (0.75%, 1.24%, and 2.65% per patient-year for CHA₂DS₂-VASc scores of 0, 1, and ≥ 2 respectively), but are likely more strongly associated with disease complexity. However, optimum anticoagulation strategies have not been studied extensively in this group, mostly because of their heterogeneity and complex clinical profiles. Individual comorbidities, disease severity, and the presence of prosthetic material must be taken into consideration when determining the ideal anticoagulation strategy in pregnant patients. With improving survival and expanding treatment options, the requirement of thromboprophylaxis is steadily increasing, however, safety, choice, and duration of anticoagulation strategies in this group has not been well studied. In this review we aim to highlight the current understanding of antithrombotic therapy in the ACHD population, with discussion regarding special considerations in unique clinical situations relevant to ACHD.