Abstract:
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.
摘要:
非扩张型左心室心肌病(NDLVC)是一种新分类的心肌病表型,包括几种病因,其连接特征由正常左心室容积表示。炎症性心脏病(InHD)是一个异质性的过程,具有不同的临床表现,有时与NDLVC重叠。一名26岁的妇女因完全心脏传导阻滞(CHB)和持续升高的肌钙蛋白而入院。超声心动图和冠状动脉造影正常。在心脏磁共振中发现了广泛的水肿和钆的晚期增强。心内膜活检显示没有活动性心肌炎的迹象。从恢复房室传导开始类固醇治疗,但随后患者出现轻度复发,并伴有新的肌钙蛋白复发。与临床情况相关的突变基因检测呈阴性。在这种情况下,NDLVC与InHD的精确诊断工作,包括基因测试,对诊断至关重要,预后和治疗目的。多模态方法对于检测和治疗可能的复发至关重要。