LEFT-VENTRICULAR FUNCTION AND PERFUSION IN BECKERS MUSCULAR-DYSTROPHY

The aim of this study was to evaluate left ventricular (LV) perfusion and function in patients with Becker muscular dystrophy (BMD). Methods : Fourteen male patients (age range 14-40 yr) with BMD were evaluated by Tl-201 SPECT and radionuclide angiography both at rest and after di pyridamole stress test. Results: All patients showed uptake defect dem onstrated by Tl-201 SPECT (mean 4.1 +/- 2.2 uptake defect/patient). Si gnificant relationships (p < 0.05) were found between the number of up take defects and rest LV ejection fraction (LVEF) (r = -0.54), peak fi lling rate (PFR) (r = -0.57) and dipyridamole LVEF (r = -0.65). Dipyri damole induced reversible uptake defects were found in 7/14 (50%) pati ents with BMD. The 14 patients were divided into two groups on the bas is of the presence (Group A, n = 6) or the absence (Group B, n = 8) of severe irreversible uptake defect (i.e., < 50% Tl-201 uptake). Group A showed lower values of PFR and LVEF when compared to patients of Gro up B. Conclusion: In patients with BMD there is a relatively high inci dence of uptake defects and LV function (both at rest and after dipyri damole) appears to be related to the number of uptake defects. Moreove r, the presence of severe irreversible uptake defects identifies a sub group of patients with BMD characterized by a severely depressed LV fu nction.