Józef Struś Hospital, Poznań, Poland: Department of Internal Medicine.
Józef Struś Hospital, Poznań, Poland: Pathomorphology Unit.
Pol Merkur Lekarski. 2022 Dec 22;50(300):378-383.
Brugada syndrome (BrS) is an inherited channelopathy characterized on ECG by coved (type 1) or saddle-back (type 2) ST-segment elevation (STE) of 2 or more mm in the right precordial leads and is associated with an increased risk of malignant ventricular arrhythmias. The term Brugada phenocopy (BrPh) indicates conditions that may reversibly induce Brugada-like ECG pattern in patients without true BrS; e.g.: metabolic abnormalities, mechanical heart compression, ischemia, myocarditis/pericarditis, and pulmonary embolism (PE). Only 9 cases of BPh associated with PE have been described so far. The authors present another case of a 41-year-old-male and analyze the clinical data of all 10 subjects (7 males and 3 females). Type 1 of ECG Brugada pattern was present in 7 patients (including ours), type 2 was found in 2 persons; in 1 case ECG pattern was not defined. In 7 patients STE was prominent (5 mm or more in at least 1 lead). STE was limited to V1-V2 leads in 4 persons, extended to V3 in 3 patients and even to V4 in 3 other patients, which correlated with the significant right ventricular (RV) dilatation. Concomitant left ventricular (LV) systolic dysfunction was reported only in 1 patient, which suggested that paradoxical embolization of coronary artery was not the mechanism of BrS-like STE. Clinical course of PE was usually severe (5 individuals were treated with thrombolysis) and in 3 cases it ended with death. The autopsy was only performed on our patient. It showed diffuse (ischemic) injury of RV and LV secondary to RV overload, decreased cardiac output and severe oxygen deficiency in myocardium, which could have led to BrS pattern in ECG.
Brugada 综合征(BrS)是一种遗传性离子通道病,心电图表现为右胸导联(V1-V3)呈 coved(1 型)或 saddle-back(2 型)型抬高,抬高幅度≥2mm,伴有恶性室性心律失常风险增加。 Brugada 表型(BrPh)是指可能导致无真正 BrS 患者心电图出现 Brugada 样改变的可逆性疾病;例如:代谢异常、机械性心脏压迫、缺血、心肌炎/心包炎和肺栓塞(PE)。迄今为止,仅有 9 例与 PE 相关的 BrPh 病例报道。作者报告了另 1 例 41 岁男性病例,并分析了 10 例患者(7 名男性和 3 名女性)的临床资料。7 例患者(包括我们的患者)存在 1 型心电图 Brugada 样改变,2 例患者存在 2 型改变,1 例患者心电图改变未明确;7 例患者 STE 明显(至少 1 个导联≥5mm),4 例患者 STE 局限于 V1-V2 导联,3 例患者延伸至 V3 导联,3 例患者甚至延伸至 V4 导联,与显著的右心室(RV)扩张相关。仅 1 例患者报告左心室(LV)收缩功能障碍,提示冠状动脉反常栓塞不是导致 Brugada 样 STE 的机制。PE 的临床病程通常较为严重(5 例患者接受溶栓治疗),其中 3 例患者死亡。仅对我们的患者进行了尸检,发现 RV 和 LV 弥漫性(缺血性)损伤,继发于 RV 负荷过重、心输出量减少和心肌严重缺氧,这可能导致心电图出现 BrS 样改变。