Evidence of early impairments in both right and left ventricular inotropic reserves in children with Duchenneʹs muscular dystrophy

In Duchenneʹs muscular dystrophy (DMD), cardiac function deteriorates with time and heart failure is one of the major causes of death. The aim of the study was to determine if a decrease in the ventricular inotropic reserves could be an early sign of cardiac dysfunction in these children. Nineteen children with DMD (aged 9 to 18 years, mean age 13.6 ± 2.4) underwent equilibrium radionuclide angiography at rest and during an inotropic stimulation with low-dose dobutamine perfusion (7.5 to 15 μg · kg−1 · min−1). In all patients, this investigation was short (<30 minutes), successful, and uncomplicated. At rest, left ventricular (LV) ejection fraction (EF) was normal (>0.50) in 79% of patients, and right ventricular (RV) EF was normal (>0.45) in 95%. There was a trend toward a decrease with age for rest LVEF (p = 0.051) but not for rest RVEF (p = 0.8). By contrast, marked declines with age could be documented for the increases (Δ) in LVEF and RVEF during dobutamine perfusion (p = 0.002 for ΔLVEF and p = 0.015 for ΔRVEF). Thus, by multivariate analysis, the sole best indicator of decline in cardiac function with age was LVEF determined with dobutamine. In children with DMD, low-dose dobutamine radionuclide angiography gives evidence of an early decline with age of the inotropic reserves of both ventricles.