Anticoagulation in cardiomyopathy: unravelling the hidden threat and challenging the threat individually

Cardiomyopathy comprises a heterogeneous group of myocardial abnormalities, structural or functional in nature, in the absence of coronary artery disease and other abnormal loading conditions. These myocardial pathologies can result in premature death or disability from progressive heart failure, arrhythmia, stroke, or other embolic events. The European Cardiomyopathy Registry reports a high stroke risk in cardiomyopathy patients ranging from 2.1% to 4.5%, as well as high prevalence of atrial fibrillation ranging from 14.0% to 48.5%. There is a growing interest in evaluating the risk of thromboembolism depending on the type of cardiomyopathy, as well as if anticoagulation is indicated in patients with cardiomyopathy without atrial fibrillation. Data available do not unequivocally support anticoagulation therapy in all of these patients; the management of these patients remains challenging. Many published reports pertaining to the risk of thromboembolism and consecutive treatment strategies mainly focus on single cardiomyopathy subtype. We summarize essential pathophysiological knowledge and review current literature associated with thromboembolism in various cardiomyopathy subtypes, providing recommendations for the diagnostic evaluation as well as clinical management strategies in this field. Certain cardiomyopathy subtypes require anticoagulation independent of atrial fibrillation or CHA₂ DS₂ -VASc score. Despite the scarcity of evidence regarding the choice of anticoagulation regimen (vitamin K antagonist vs. non-vitamin K oral anticoagulants) in cardiomyopathy, it is discussed and reviewed in this article. Each patient should receive a tailored strategy based on thorough clinical evaluation, published evidence, and clinical experience, due to the current recommendations mostly developed on small-sample studies or empirical evidence. The future research priorities in this area are also addressed in this article.