Atrial Fibrillation in β-Thalassemia: Overview of Mechanism, Significance and Clinical Management
by
, ,
Michele Malagù
1,*
,
Federico Marchini
1
,
Alessio Fiorio
1,
Paolo Sirugo
1,
Stefano Clò
1,
Elisa Mari
2,
Maria Rita Gamberini
2,
Claudio Rapezzi
1 and
Matteo Bertini
1
1
Cardiology Unit, Azienda Ospedaliero-Universitaria di Ferrara, 44124 Ferrara, Italy
2
Day Hospital Thalassemia and Hemoglobinopathies, Azienda Ospedaliero-Universitaria di Ferrara, 44124 Ferrara, Italy
*
Author to whom correspondence should be addressed.
Biology 2022, 11(1), 148; https://doi.org/10.3390/biology11010148
Submission received: 27 December 2021
/
Revised: 13 January 2022
/
Accepted: 14 January 2022
/
Published: 17 January 2022
(This article belongs to the Special Issue Biophysics Arrhythmias and Pacing)
Simple Summary
Atrial 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.
Abstract
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.
Keywords:
thalassemia; hemoglobinopathy; arrhythmias; supraventricular; atrial fibrillation; iron; ablation; chelation; heart
