Premawardhena Anuja, De Silva Shamila, Rajapaksha Megha, Ratnamalala Vishaka, Nallarajah Jemimah, Galappatthy Gamini
Department of Medicine: Faculty of Medicine, University of Kelaniya, Kelaniya, Sri Lanka.
Adolescent & Adult Thalassaemia Unit, North Colombo (Teaching) Hospital, Ragama, Sri Lanka.
Int J Emerg Med. 2023 Mar 9;16(1):16. doi: 10.1186/s12245-023-00495-z.
Cardiac disease remains a dominant if not the most important cause of morbidity and mortality in patients with thalassaemia, particularly in those with thalassaemia major. Myocardial infarction and coronary artery disease however are rarely reported.
Three older patients with three distinct thalassaemia syndromes presented with acute coronary syndrome. Two were heavily transfused whilst the other was a minimally transfused patient. Both heavily transfused patients had ST-elevation myocardial infarctions (STEMI) while the minimally transfused patient had unstable angina. Coronary angiogram (CA) was normal in two patients. One patient who developed a STEMI had a 50% plaque. All three were managed as standard ACS, although the aetiology appeared non-atherogenic.
The exact etiology of the presentation, remains a mystery and therefore the rational use of thrombolytic therapy, carrying out angiogram in the primary setting, using and continuing antiplatelet and high dose statins all remains unclear in this sub group of patients.
心脏病仍然是地中海贫血患者发病和死亡的主要原因，即使不是最重要的原因，尤其是在重型地中海贫血患者中。然而，心肌梗死和冠状动脉疾病很少被报道。
三名患有三种不同地中海贫血综合征的老年患者出现急性冠状动脉综合征。两名患者大量输血，另一名患者少量输血。两名大量输血的患者均发生ST段抬高型心肌梗死（STEMI），而少量输血的患者患有不稳定型心绞痛。两名患者的冠状动脉造影（CA）正常。一名发生STEMI的患者有50%的斑块。尽管病因似乎非动脉粥样硬化性，但所有三名患者均按照标准急性冠状动脉综合征进行治疗。
该病症的确切病因仍是个谜，因此在这一亚组患者中，溶栓治疗的合理使用、在初始阶段进行血管造影、使用和持续使用抗血小板药物及高剂量他汀类药物均仍不明确。
