Late-onset Fabry disease presenting with ventricular tachycardia originating from typical inferolateral scar.

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.