Сomparative analysis of cardiac magnetic resonance imaging data in patients with hypertrophic cardiomyopathy and Anderson-­Fabry disease

Aim. Cardiac involvement in Anderson-Fabry disease (AFD) is often characterized by left ventricular hypertrophy (LVH), which can be mistaken for hypertrophic cardiomyopathy (HCM). The aim was to analyze and compare morphological and functional cardiac changes using multiparametric magnetic resonance imaging (MRI) in patients with AFD, HCM, and healthy volunteers.

Material and methods. The study included 131 patients with HCM, 42 patients with AFD, and 18 healthy volunteers. Multiparametric cardiac MRI was performed using a 3T scanner.

Results. LVH was detected in 55% of patients with AFD. Signs of myocardial fibrosis were more common in HCM than in AFD+LVH (86% vs 35%, p<0,05). Native T1 and T2 mapping and extracellular volume  fraction values were higher in the HCM group than in the AFD+LVH group as follows: 1269,4±52,7 vs 1144,5±30,6 ms, 41,6±3,9 vs 40,0±1,4 ms, 0,33±0,06 vs 0,27±0,02 ms, respectively (p<0,05). Native T1 mapping values in AFD patients without LVH were significantly lower than in healthy volunteers as follows: 1124,6±45,0 vs 1207,7±40,0 ms, respectively (p<0,05).

Conclusion. Native T1 mapping values in AFD, regardless of LVH, are lower than those in patients with HCM and healthy volunteers and can be used in the early diagnosis of myocardial involvement in AFD. 

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