Abstract
Simple SummaryAtrial fibrillation in patients with β-thalassemia has a higher incidence compared to the general population. Its pathophysiology is peculiar and strictly related to anemia, iron overload, hemolysis, inflammation, atrial dilatation, fibrosis, and remodeling. The clinical presentation is that of a highly symptomatic disease with frequent recurrencies, significantly impacting over quality of life and prognosis. Furthermore, the risk of thromboembolic events and stroke is considerable. The available treatments are drug therapy and transcatheter ablation. Moreover, particular attention should be paid to anticoagulant therapy for thromboembolic prophylaxis.Thalassemia is an inherited blood disorder with worldwide distribution. Transfusion and chelation therapy have radically improved the prognosis of β-thalassemic patients in the developed world, but this has led to the development of new chronic cardiac complications like atrial fibrillation (AF). Prevalence of AF in patients with β-thalassemia is higher than in the general population, ranging from 2 to 33%. Studies are lacking, and the little evidence available comes from a small number of observational studies. The pathophysiology is not well understood but, while iron overload seems to be the principal mechanism, AF could develop even in the absence of iron deposition. Furthermore, the clinical presentation is mainly paroxysmal, and patients are highly symptomatic. The underlying disease, the pathophysiology, and the clinical presentation require a different management of AF in β-thalassemia than in the general population. Rhythm control should be preferred over rate control, and the most important antiarrhythmic therapy is represented by chelation drugs. Thromboembolic risk is high, but the available risk scores are not validated in β-thalassemia, and the choice of anticoagulation therapy should be considered early. The main purpose of this review is to summarize the actual knowledge about AF in β-thalassemia, with a specific focus on the clinical management of these complex patients.
Highlights
Since the introduction of transfusion and chelation therapy, the natural history of β-thalassemia has changed
While for millennia, patients died in childhood, and 50 years ago, following the introduction of transfusion therapy, they died young of severe heart failure and hemochromatosis secondary to iron overload, at the present time, with optimal care, most thalassemia patients have a longer life expectancy and survive into their 60s or later
This review aims to analyze the various aspects of the problem and to explore possible management strategies based on the available evidence
Summary
Since the introduction of transfusion and chelation therapy, the natural history of β-thalassemia has changed. While for millennia, patients died in childhood, and 50 years ago, following the introduction of transfusion therapy, they died young of severe heart failure and hemochromatosis secondary to iron overload, at the present time, with optimal care, most thalassemia patients have a longer life expectancy and survive into their 60s or later. An emerging problem in the natural history of thalassemia is the development of atrial fibrillation (AF). The mechanisms leading to AF in thalassemic patients are different from those in the general population, and the clinical features are peculiar. Many points are still obscure, and specific studies are few, but the management of thalassemic patients with AF has entered daily clinical practice. This review aims to analyze the various aspects of the problem and to explore possible management strategies based on the available evidence
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