Wijaya Wilson Saputra, Krisnawati Ika
Faculty of Medicine, Trisakti University, Jakarta, Indonesia.
Department of Cardiology, Tangerang City General Hospital, Tangerang City, Indonesia.
Int Med Case Rep J. 2022 Mar 2;15:75-80. doi: 10.2147/IMCRJ.S353263. eCollection 2022.
Brugada syndrome (BrS) has been described as an inherited cardiac disorder due to gene mutations, which contributes to sudden cardiac death, especially in Southeast Asians. Brugada phenocopy, which has the same pattern caused by etiologies such as metabolic disorder, is potentially reversible and avoids unnecessary implantable medical devices.
We reported a rare case of Brugada phenocopy in a 60-years-old Indonesian male with severe anemia and a history of chronic urinary tract bleeding and hemorrhoid. Brugada phenocopy, widely known as BrS look-alike, has been linked to sodium, potassium, and calcium channel dysfunction. This condition is uncommon and has been related to metabolic conditions such as anemia. Iron deficiency anemia induces ischemia in the myocardium and degrades the calcium channels. During the transfusion, hypocalcemia precipitates temporary arrhythmia with right ventricular outflow tract origin, which reverts after the hypocalcemia has been treated.
Due to the possibility of altered ion channels and how rarely hypocalcemia induces arrhythmia, this makes understanding the pathogenesis of Brugada phenocopy essential. Though caution is necessary, early recognition can improve prognosis as Brugada phenocopy is potentially reversible.
布加综合征(BrS)被描述为一种由基因突变引起的遗传性心脏疾病,它会导致心源性猝死,在东南亚人群中尤为常见。布加综合征拟表型是由代谢紊乱等病因导致的具有相同心电图模式的情况,具有潜在可逆性,可避免不必要的植入式医疗设备。
我们报告了一例罕见的布加综合征拟表型病例,患者为一名60岁的印度尼西亚男性,患有严重贫血,有慢性尿路出血和痔疮病史。布加综合征拟表型,广为人知的类似布加综合征的情况,与钠、钾和钙通道功能障碍有关。这种情况并不常见,且与贫血等代谢状况有关。缺铁性贫血会导致心肌缺血并使钙通道退化。输血过程中,低钙血症会引发起源于右心室流出道的暂时性心律失常,在低钙血症得到治疗后心律失常会恢复。
由于存在离子通道改变的可能性以及低钙血症诱发心律失常的情况极为罕见,这使得了解布加综合征拟表型的发病机制至关重要。尽管需要谨慎,但早期识别可改善预后,因为布加综合征拟表型具有潜在可逆性。