Predictive Value of Cardiac MRI in Patients with Fabry Disease.

Abstract

HomeRadiologyVol. 296, No. 2 PreviousNext CommunicationsFree AccessLetters to the EditorPredictive Value of Cardiac MRI in Patients with Fabry DiseaseEkaterina Tao* , Alexey Moiseev†, Elena Mershina†, Irina Bobkova*, Sergey Moiseev*,†Ekaterina Tao* , Alexey Moiseev†, Elena Mershina†, Irina Bobkova*, Sergey Moiseev*,†Author AffiliationsTareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, 11/5 Rossolimo St, Moscow 119021, Russian Federation*Faculty of Medicine, Moscow State University, Moscow, Russian Federation†e-mail: [email protected]Ekaterina Tao* Alexey Moiseev†Elena Mershina†Irina Bobkova*Sergey Moiseev*,†Published Online:May 12 2020https://doi.org/10.1148/radiol.2020200909MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked InEmail Editor:In a retrospective study in the January 2020 issue of Radiology, Dr Hanneman and colleagues reported cardiac MRI findings in 90 patients with Fabry disease (FD) (1). Left ventricular hypertrophy (LVH) and late gadolinium enhancement (LGE) were found in 40% of patients and were independent predictors of the composite end point (ventricular tachycardia, bradycardia requiring device implantation, severe heart failure, and cardiac death), which was reached in 21 (23%) patients within a median of 3.6 years. Patients with extensive LGE demonstrated the highest risk. Nonsustained ventricular tachycardia was the most common cardiac event, occurring in 19 of 21 patients.We performed cardiac MRI in 93 patients with definite FD including 55 men and 38 women (median age, 35 years; interquartile range, 29–48 years). In our cohort, the prevalence of LVH was higher (50.5%) than in the study by Dr Hannemann and colleagues, whereas LGE was a rarer finding (23.7%), probably as a result of the younger age of our patients. Adverse cardiac events occurred in nine patients (9.7%) and included clinically significant arrhythmias (n = 8), heart failure (n = 1), and sudden cardiac death (n = 2). Of note, most patients (n = 8) presented with atrial fibrillation, whereas nonsustained ventricular tachycardia was detected in only one patient. Cardiac events were associated with a higher median left ventricular myocardial mass index (140.0 g/m2 vs 103.5 g/m2 in patients without cardiac events; P = .04), and occurred more frequently in patients with LVH (17% vs 2% in patients with normal left ventricular mass; P = .03). Nevertheless, the difference in the frequency of cardiac events between patients with and without LGE did not reach statistical significance (14% and 7%, respectively; P = .39).In summary, we agree with Dr Hannemann and colleagues that cardiac MRI is a valuable tool to identify patients with FD who are at high risk of cardiac events and to monitor the efficacy of enzyme replacement therapy. Unlike men, not all women with FD require enzyme replacement therapy. Therefore, cardiac MRI is also important for treatment decisions because the presence of LVH and/or LGE in women with FD indicates a need for enzyme replacement therapy (2). In most of our patients, LVH or LGE was not associated with life-threatening arrhythmias such as ventricular tachycardia. Therefore, the predictive value of cardiac MRI may be variable in a different cohort of patients with FD and should be evaluated in cooperative international studies.Disclosures of Conflicts of Interest: E.T. disclosed no relevant relationships. A.M. disclosed no relevant relationships. E.M. disclosed no relevant relationships. I.B. disclosed no relevant relationships. S.M. disclosed no relevant relationships.Supported by Russian Academic Excellence Project 5-100.