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Left Ventricular Function and Perfusion in Becker's Muscular Dystrophy

Radiology Institute-Nuclear Medicine and Cardiomiology Center; II University of Naples, Naples; Department of Nuclear Medicine, University of Federico II, Naples; Nuclear Medicine Center of Naples, Naples, Italy

Correspondence: For correspondence or reprints contact: Luigi Mansi, MD, Medicina Nucleare-Istituto di Scienze Radiologiche, Seconda Università di Napoli, Piazza Miraglia 2, 80138 Napoli, Italy.

ABSTRACT

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 ²⁰¹Tl SPECT and radionuclide angiography both at rest and after dipyridamole stress test. Results: All patients showed uptake defect demonstrated by ²⁰¹Tl SPECT (mean 4.1 ± 2.2 uptake defect/patient). Significant relationships (p < 0.05) were found between the number of uptake defects and rest LV ejection fraction (LVEF)(r = –0.54), peak filling rate (PFR)(r = –0.57) and dipyridamole LVEF (r = –0.65). Dipyridamole induced reversible uptake defects were found in 7/14 (50%) patients with BMD. The 14 patients were divided into two groups on the basis of the presence (Group A, n = 6) or the absence (Group B, n = 8) of severe irreversible uptake defect (i.e., < 50%²⁰¹Tl uptake). Group A showed lower values of PFR and LVEF when compared to patients of Group B. Conclusion: In patients with BMD there is a relatively high incidence of uptake defects and LV function (both at rest and after dipyridamole) appears to be related to the number of uptake defects. Moreover, the presence of severe irreversible uptake defects identifies a subgroup of patients with BMD characterized by a severely depressed LV function.

Key Words: thallium-201-SPECT • Becker muscular dystrophy • radionuclide angiography