Marchini Federico, Fiorio Alessio, Sirugo Paolo, Gamberini Maria Rita, Mari Elisa, Bertini Matteo, Malagù Michele
U.O. Cardiologia, Azienda Ospedaliero-Universitaria di Ferrara, Ferrara.
U.O. Interdipartimentale di Day Hospital della Talassemia e delle Emoglobinopatie, Azienda Ospedaliero-Universitaria di Ferrara, Ferrara.
G Ital Cardiol (Rome). 2022 Jun;23(6):477-480. doi: 10.1714/3810.37945.
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.
心血管并发症是重型地中海贫血患者死亡和发病的主要原因之一。铁螯合疗法对于预防因输血需求导致的慢性铁过载及随之而来的心脏含铁血黄素沉着至关重要。尽管铁螯合药物被广泛使用,但如今仍能发现严重铁蓄积的病例。此外,即使不考虑铁过载和心脏功能障碍,地中海贫血患者的心律失常负担也很高,尤其是室上性心律失常。对于这类患者的管理仍有许多疑问和未解决的问题,特别是在抗凝治疗的正确使用以及可用于节律控制的治疗策略的最佳利用方面。所呈现的病例表明,在抗心律失常药物无效且无法等待铁螯合疗法起效的急性期,导管消融的介入方法也可能有用。