Gonano Nicola, Nuzzi Vincenzo, Pavan Daniela, Piazza Rita, Pecoraro Rosa, Altinier Alessandro, Bussani Rossana, Sinagra Gianfranco, Merlo Marco
Department of Cardiothoracovascular, Azienda Sanitaria Universitaria Integrata Giuliana Isontina (ASUGI), University of Trieste, Trieste, Italy.
Department of Cardiology, Santa Maria degli Angeli Hospital, Azienda Sanitaria Friuli Orientale (ASFO), Pordenone, Italy.
ESC Heart Fail. 2024 Dec;11(6):4407-4412. doi: 10.1002/ehf2.14822. Epub 2024 Jul 12.
Non-dilated left ventricular cardiomyopathy (NDLVC) is a newly categorized cardiomyopathy phenotype includingseveral aetiologies with a linking characteristic represented by the normal left ventricular volume. Inflammatory heart disease (InHD) is a heterogeneous process with variegate clinical manifestations, sometimes in overlap with NDLVC. A 26-year-old woman was admitted forcomplete heart block (CHB) and persistently raised troponin. Echocardiography and coronary angiography were normal. Extensive oedema and late gadolinium enhancement was found at cardiac magnetic resonance. Endomyocardial biopsy showed no signs of active myocarditis. Steroid therapy was started with restoration of atrioventricular conduction but subsequently the patient experienced a mild recurrence with a new troponin relapse. Genetic test was negative for mutations related with the clinical scenario. In this case of NDLVC with InHD the precise diagnostic work-up, including genetic test, was crucial for diagnostic, prognostic andtherapeutic purposes. Multimodality approach is crucial to detect and treat possible recurrences.
非扩张型左心室心肌病(NDLVC)是一种新分类的心肌病表型,包括多种病因,其共同特征是左心室容积正常。炎症性心脏病(InHD)是一个异质性过程,临床表现多样,有时与NDLVC重叠。一名26岁女性因完全性心脏传导阻滞(CHB)和肌钙蛋白持续升高入院。超声心动图和冠状动脉造影正常。心脏磁共振成像发现广泛水肿和延迟钆增强。心内膜活检未显示活动性心肌炎迹象。开始使用类固醇治疗,房室传导恢复,但随后患者出现轻度复发,肌钙蛋白再次升高。基因检测未发现与临床情况相关的突变。在这个NDLVC合并InHD的病例中,包括基因检测在内的精确诊断检查对于诊断、预后和治疗目的至关重要。多模式方法对于检测和治疗可能的复发至关重要。