Atrial Fibrillation in β-Thalassemia: Overview of Mechanism, Significance and Clinical Management

Biology

Paolo Sirugo¹  Federico Marchini¹  Claudio Rapezzi¹  Michele Malagù¹  Alessio Fiorio¹  Stefano Clò¹  Matteo Bertini¹  Elisa Mari²  Maria Rita Gamberini² 

[1] Cardiology Unit, Azienda Ospedaliero-Universitaria di Ferrara, 44124 Ferrara, Italy;Day Hospital Thalassemia and Hemoglobinopathies, Azienda Ospedaliero-Universitaria di Ferrara, 44124 Ferrara, Italy;

关键词: thalassemia;    hemoglobinopathy;    arrhythmias;    supraventricular;    atrial fibrillation;    iron;   

DOI  :  10.3390/biology11010148

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown