Comparison of Cardiac Magnetic Resonance Imaging and Echocardiography in Assessment of Left Ventricular Hypertrophy in Fabry Disease.

BACKGROUND

Cardiac hypertrophy in Fabry disease can be assessed using the left ventricular mass index (LVMI) with either echocardiography (LVMI-ECHO) or magnetic resonance imaging (LVMI-CMR).

METHODS

A retrospective case series of patients with Fabry disease in Alberta involved a cross-sectional analysis of 32 patients and a longitudinal analysis of 14 of these patients with at least 4 serial CMR measurements.

RESULTS

The cross-sectional analysis showed the mean LVMI-ECHO was 97.8 ± 26.0 g/m2, which was higher compared with LVMI-CMR at 81.1 ± 26.9 g/m2 with a mean bias of 16.7 g/m2 (P < 0.001). In the longitudinal analysis, LVMI-ECHO was higher, with an estimated marginal mean of 96.21 ± 6.13 (mean ± standard error of the mean [SEM]) compared with 71.18 ± 5.99 for LVMI-CMR (P < 0.01; generalized estimating equations). There was an association between an increase in LVMI-CMR over time with the presence of cardiac fibrosis, and patients treated with enzyme replacement therapy (ERT) had slower increases than those without therapy. LVMI-ECHO failed to detect these associations owing to the higher variability and tendency to overestimate the LVMI.

CONCLUSIONS

We propose the preferred method for measuring LVMI is CMR in patients with Fabry disease.