We have a similar case of a 34-year-old woman who had undergone
splenectomy, who presented with hypoxia and a tricuspid incompetence
(TI) gradient of 27 mm Hg. After 3 years on enzyme therapy with
improvement in hypoxemia, clubbing, and pulmonary function tests, the
TI gradient dropped to 20 mm Hg (within the normal range). After 2 more
years of treatment, she developed elevation of TI gradient to 37 mm Hg.
We also documented another patient with Gaucher’s disease, who started
enzyme therapy with a TI gradient within the normal range, which then
rose above 30 mm Hg with treatment with the placental derivative,
alglucerase, and then returned to the normal range after
withdrawal.4 Challenge with the recombinant form,
imiglucerase, resulted in elevation of the TI gradient above 30 mm Hg,
and treatment withdrawal again resulted in return to the normal range.