P442Global function index: a useful method to suspect cardiac involment in patients with Fabry disease without hypertrophy.


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Abstract

Background: Fabry cardiomyopathy is characterized by reduced myocardial contraction and relaxation tissue Doppler velocities detectable even before development of LVH. Previous studies have reported the usefulness of a new global function index [GFI = (E/E') / S'] in the differential diagnosis of left ventricular hypertrophy (LVH) secondary to hypertension (HT) and hypertrophic cardiomyopathy (HCM).Aim: To evaluate the usefulness of GFI in the identification cardiac involvement in Fabry patients without LVHMethods: We analyzed 87 patients with a confirmed diagnosis of Fabry disease. Our population was divided in two groups:“ A”:64 patients without LVH and "B": 23 patients with basal LVH. Echocardiography and tissue Doppler imaging (TDI) was performed at baseline and periodically throughout the study: thickness of interventricular septum (IVS) and posterior wall (PW); left ventricular (LV) end-diastolic diameter; diastolic velocities obtained by pulsed Doppler of the mitral valve; diastolic TDI velocities (E' and A'), systolic TDI velocity (S') and the resultant GFI obtained at septal(GFIs) and lateral (GFIl) sites of the mitral annulus.Results: At baseline, the GFI was significantly higher in the group "B"(GFIs 2.24±1.04 and GFI-l 1.58±1.41) than the group "A" GFIs 1.07±0.40 and GFIl: 0.59±0.46 ) p<0.05. On the follow up in the group "A" there was an increasing of GFIs (0.05±0.01; p <0.001) per year. The patients who developed hypertrophy the GFIs increased 0.17±0.06; p= 0.01.Conclusions: GFI could be a useful tool to early diagnosis and follow up of patients with Fabry disease before hypertrophy is present. In Fabry patients, GFI could be a useful tool for early diagnosis of cardiac involvement, even before the development of LVH. This may lead to an early treatment and improve prognosis of such patients

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