V. Panichi et al., Echocardiographic ultrasonic tissue characterization in a case of Fabry's disease following renal transplantation, CLIN TRANSP, 15(3), 2001, pp. 214-217
We report a case of Fabry's disease where stabilization of progressive card
iac involvement was recorded in a 29-yr-old Caucasian man, to our knowledge
, for the first time by ultrasonic tissue characterization echocardiography
after 1 yr of successful renal transplantation. Three echocardiographic ev
aluations have been made: the first 3 months before, the second 6 months af
ter, and the third 1 yr after kidney transplantation.
The myocardial structural damage - evaluated by integrated backscatter inde
x - shows a persistence of the impairment of intrinsic myocardial contracti
lity at septum level, probably due to coexistent hypertensive status, which
is able to induce per se alterations of myocardial textural parameters. On
the other hand, the cyclic variation index at posterior free wall, which i
s less dependent on strictly hemodynamic factors than the septum, appears q
uite normal at the third observation.
These data could reflect the improvement of the ultrastuctural myocardial f
indings in relation to renal transplantation, which could correct not only
renal failure but also the enzymatic deficiency by replacement of alpha -ga
lactosidase A through the transplanted kidney.