Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano Isontina, Trieste, Italy.
Department of Cardiac Imaging, North West Heart Centre, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, United Kingdom.
Can J Cardiol. 2020 Nov;36(11):1832.e1-1832.e4. doi: 10.1016/j.cjca.2020.04.042. Epub 2020 May 12.
Late-onset cardiac Fabry disease is not uncommon among patients with unexplained left ventricular hypertrophy. Despite a less severe phenotype, life-threatening complications are possible in late-onset cardiac Fabry and may be the first presentation of the disease. Classical imaging features support the diagnosis; however, the presence of less common findings, such as ischemic features, should not lead to overlooking the diagnosis. Indeed, the coexistence of Fabry and ischemic heart disease is possible, even in the absence of obstructive coronary artery disease. Therefore, a high level of suspicion should be maintained, even in the presence of atypical presentations.
迟发性心脏法布瑞病在不明原因左心室肥厚患者中并不少见。尽管表型较轻，但迟发性心脏法布瑞病仍可能发生危及生命的并发症，且其可能为该病的首发表现。典型影像学特征有助于诊断；然而，存在不常见表现（如缺血特征）不应导致漏诊。事实上，即使不存在阻塞性冠状动脉疾病，法布瑞病和缺血性心脏病也可能同时存在。因此，即使表现不典型，也应保持高度怀疑，即使表现不典型。
