Tsampras Theodoros, Antonopoulos Alexios S, Kourti Freideriki-Eleni, Tsioufis Konstantinos, Vlachopoulos Charalambos
Cardiogenetics Unit, 1 st Cardiology Department, Hippokration Hospital, National and Kapodistrian University of Athens, 114 Vas. Sofias Avenue, Athens, Greece.
Clin Res Cardiol. 2025 Jul 3. doi: 10.1007/s00392-025-02680-x.
Transthyretin amyloidosis is a significant cause of heart failure with an unfavorable prognosis. In recent years, diagnosing the disease has become easier, with most patients now diagnosed non-invasively, and tissue biopsy being required only in a minority of cases. Although various laboratory findings have been reported as transthyretin amyloidosis red flags, the diagnostic and prognostic value of various electrocardiogram parameters remain largely unknown. In this study, the significance of QTc interval prolongation in transthyretin cardiac amyloidosis patients was investigated. We retrospectively analyzed electrocardiogram data from n = 58 patients with transthyretin amyloid cardiomyopathy and compared them with distinct cohorts of patients diagnosed with other forms of heart muscle disease, i.e., hypertrophic cardiomyopathy and dilated cardiomyopathy. QTc prolongation was found to be a unique feature of transthyretin cardiac amyloidosis, not shared by other cardiomyopathy types. Increased QTc interval in transthyretin amyloidosis patients correlates with disease severity markers, including NYHA class, NAC stage, NT-proBNP, and troponin I levels, suggesting its potential as a unique biomarker for monitoring disease progression.
转甲状腺素蛋白淀粉样变性是导致心力衰竭的一个重要原因，预后不佳。近年来，该病的诊断变得更加容易，现在大多数患者通过非侵入性方法即可确诊，仅少数病例需要进行组织活检。尽管已有各种实验室检查结果被报道为转甲状腺素蛋白淀粉样变性的警示信号，但各种心电图参数的诊断和预后价值在很大程度上仍不明确。在本研究中，我们调查了QTc间期延长在转甲状腺素蛋白心脏淀粉样变性患者中的意义。我们回顾性分析了n = 58例转甲状腺素蛋白淀粉样心肌病患者的心电图数据，并将其与诊断为其他形式心肌病（即肥厚型心肌病和扩张型心肌病）的不同患者队列进行比较。结果发现QTc延长是转甲状腺素蛋白心脏淀粉样变性的一个独特特征，其他类型的心肌病并不具备。转甲状腺素蛋白淀粉样变性患者QTc间期增加与疾病严重程度指标相关，包括纽约心脏协会（NYHA）心功能分级、NAC分期、N末端脑钠肽前体（NT-proBNP）和肌钙蛋白I水平，这表明其有可能作为监测疾病进展的独特生物标志物。
