Haugaa Kristina H, Basso Cristina, Badano Luigi P, Bucciarelli-Ducci Chiara, Cardim Nuno, Gaemperli Oliver, Galderisi Maurizio, Habib Gilbert, Knuuti Juhani, Lancellotti Patrizio, McKenna William, Neglia Danilo, Popescu Bogdan A, Edvardsen Thor
Department of Cardiology, Center for Cardiological Innovation and Institute for Surgical Research, Oslo University Hospital, Oslo and University of Oslo, Oslo, Norway.
Cardiovascular Pathology, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua Medical School, Padua, Italy.
Eur Heart J Cardiovasc Imaging. 2017 Mar 1;18(3):237-253. doi: 10.1093/ehjci/jew229.
Arrhythmogenic cardiomyopathy (AC) is a progressive disease with high risk of life-threatening ventricular arrhythmias. A genetic mutation is found in up to 50-60% of probands, mostly affecting desmosomal genes. Diagnosis of AC is made by a combination of data from different modalities including imaging, electrocardiogram, Holter monitoring, family history, genetic testing, and tissue properties. Being a progressive cardiomyopathy, repeated cardiac imaging is needed in AC patients. Repeated imaging is important also for risk assessment of ventricular arrhythmias. This expert consensus document gives clinical recommendations for how to use multi-modality imaging in the different aspects of AC disease, including diagnosis, family screening, follow-up, risk assessment, and differential diagnosis.
致心律失常性心肌病（AC）是一种进展性疾病，具有发生危及生命的室性心律失常的高风险。高达50%-60%的先证者存在基因突变，主要影响桥粒基因。AC的诊断需要综合不同检查方式的数据，包括影像学、心电图、动态心电图监测、家族史、基因检测和组织特性。作为一种进展性心肌病，AC患者需要反复进行心脏成像检查。反复成像对于室性心律失常的风险评估也很重要。本专家共识文件给出了关于如何在AC疾病的不同方面，包括诊断、家族筛查、随访、风险评估和鉴别诊断中使用多模态成像的临床建议。
