Altered myocardial motion pattern in Fabry patients assessed with CMR-tagging.

ABSTRACT

Progressive left ventricular hypertrophy is the hallmark of cardiac manifestations in patients with Fabry disease. Cardiovascular magnetic resonance with tissue tagging allows detailed assessment of the cardiac motion pattern. The aim was to test the hypothesis that not only Fabry patients with severe left ventricular hypertrophy exhibit changes in myocardial motion, but also Fabry patients with normal left ventricular mass. Magnetic resonance tagging using slice following complementary spatial modulation of magnetization (CSPAMM) combined with harmonic phase analysis (HARP) was applied to measure left ventricular shortening and contraction. Additionally, left ventricular rotation and global left ventricular torsion were examined. Twenty-nine Fabry patients grouped in patients with (n = 13) and without (n = 16) left ventricular hypertrophy were compared with 29 age and sex matched healthy volunteers. Long axis shortening and circumferential contraction showed reduced peak values with increasing left ventricular mass and were significantly reduced in Fabry patients with left ventricular hypertrophy (p < 0.001 and p < 0.05, respectively). Torsional deformation and apical rotation were increased both in Fabry patients with left ventricular hypertrophy as well as in patients with normal left ventricular mass (p < 0.001 for torsion) compared with controls. Applying the magnetic resonance tagging acquisition and analysis methods, myocardial motion abnormalities could not only be measured in Fabry patients with left ventricular hypertrophy but also in patients without macroscopic cardiac involvement.