[Heavy metal! A case of severe iron overload and supraventricular arrhythmias in a thalassemia major patient]. / Heavy metal! Un caso di severo accumulo di ferro e aritmie sopraventricolari in paziente con talassemia major.

Cardiovascular complications are among the main causes of mortality and morbidity in patients with thalassemia major. Iron-chelation therapy is essential to prevent the chronic iron overload linked to the need for transfusions and the consequent cardiac hemosiderosis. Despite the wide use of iron-chelation drugs, today it is still possible to find cases of severe iron accumulation. Furthermore, even regardless of iron overload and cardiac dysfunction, the thalassemic patient has a high arrhythmic burden, especially for supraventricular arrhythmias. There are still many doubts and open questions about the management of such patients, especially regarding the correct use of anticoagulant therapy and the best utilization of therapeutic strategies available for rhythm control. The case presented shows how the interventional approach with catheter ablation can be useful also in the acute phase when antiarrhythmic drugs are ineffective and it is not possible to wait for the iron-chelation therapy to take effect.

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

Thalassemia is an inherited blood disorder with worldwide distribution. Transfusion and chelation therapy have radically improved the prognosis of ß-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 ß-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 ß-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 ß-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 ß-thalassemia, with a specific focus on the clinical management of these complex patients.