Fianza Pandji I, Pramono Alvinsyah A, Ghozali Mohammad, Sihite Teddy A, Setiabudi Djatnika, Syamsunarno Mas R A A, Fucharoen Suthat, Panigoro Ramdan
Department of Internal Medicine, Division of Hematology and Medical Oncology, Faculty of Medicine, Universitas Padjadjaran/Hasan Sadikin General Hospital, 40161 Bandung, West Java, Indonesia.
Research Center of Medical Genetics, Faculty of Medicine, Universitas Padjadjaran, 40161 Bandung, West Java, Indonesia.
Rev Cardiovasc Med. 2022 Jul 22;23(8):267. doi: 10.31083/j.rcm2308267. eCollection 2022 Aug.
Thalassemia major is the most common monogenetic disorder worldwide, manifested as chronic hemolytic anemia. This condition leads to the need for chronic blood transfusion to be monitored for an iron overload that may be stored in several tissues and organs, including cardiomyocytes, that might cause a broad spectrum of cardiac iron toxicities such as heart failure conduction delays, myocarditis, and arrhythmias. Non-invasive imaging modalities have their benefits and limitations. Each modality complements and generates a comprehensive diagnostic and monitoring of cardiac siderosis in thalassemia major patients.
重型地中海贫血是全球最常见的单基因疾病，表现为慢性溶血性贫血。这种情况导致需要长期输血并监测可能储存在包括心肌细胞在内的多个组织和器官中的铁过载，这可能会引起广泛的心脏铁毒性，如心力衰竭、传导延迟、心肌炎和心律失常。非侵入性成像方式有其优点和局限性。每种方式相互补充，有助于对重型地中海贫血患者的心脏铁沉着症进行全面的诊断和监测。
