title: Evidence of early impairments in both right and left ventricular inotropic reserves in children with Duchenne's muscular dystrophy.
abstract: In Duchenne's 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 microg. 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 (Delta) in LVEF and RVEF during dobutamine perfusion (p = 0.002 for DeltaLVEF and p = 0.015 for DeltaRVEF). 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.
authors.affiliation: Department of Pediatric Cardiology, Chu-Nancy, France. g.bosser@chu-nancy.fr
authors.forename: Gilles
authors.initials: G
authors.lastname: Bosser
authors.forename: Hugues
authors.initials: H
authors.lastname: Lucron
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Lethor
authors.forename: Guillaume
authors.initials: G
authors.lastname: Burger
authors.forename: Françoise
authors.initials: F
authors.lastname: Beltramo
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Marie
authors.forename: François
authors.initials: F
authors.lastname: Marçon
mesh_terms: D000293:Adolescent; D002648:Child; D004280:Dobutamine; D005260:Female; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology*; D015635:Radionuclide Ventriculography; D012189:Retrospective Studies; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000503:physiopathology*
chemical_list: D004280:Dobutamine