title: Reduced inotropic reserve is predictive of  further degradation in left ventricular ejection  fraction in patients with Duchenne muscular  dystrophy.
abstract: OBJECTIVE
  Duchenne muscular dystrophy (DMD), an inherited X-linked muscular disease, is associated with dilated cardiomyopathy that is responsible for death in 40% of patients. Our objective was to determine whether inotropic reserve is predictive of LV trend over time.
  
  
  RESULTS
  A total of 69 DMD patients (age 12.2±2.3 years) were investigated. At baseline, LVEF and the presence of inotropic reserve (defined as an increase in LVEF >10% during dobutamine infusion) were investigated using radionuclide ventriculography. During follow-up (FU), LVEF was remeasured after a mean 29±19 months delay. In the whole population, mean LVEF was 58±8% at baseline and declined to 54±11% during FU (P =0.004). At baseline, 21 patients (30.4%) had LVEF <55% and 38 had no LV inotropic reserve. LVEF declined in the 38 patients (55.1%) without LV inotropic reserve (58±8% to 52±10%, P =0.001), and not in the other patients (58±8% to 57±11%, P =0.516) (P =0.042 for trends in LVEF between groups after adjustment for age, FU duration, and baseline LVEF). Fewer patients with vs. without inotropropic reserve at baseline show a depressed LVEF <55% during follow-up(35.5% vs. 63.2%, respectively, P =0.030). Similar findings were observed in the subgroups of patients with LVEF >45% or 55% at baseline.
  
  
  CONCLUSIONS
  Inotropic reserve assessment allows the distinction of DMD patients who will vs. those who will not show a deterioration in LVEF, thus offering a sensitive approach for delineating the presence and progression of cardiovascular disease in these patients.
authors.forename: François-Xavier
authors.initials: FX
authors.lastname: Goudot
authors.forename: Karim
authors.initials: K
authors.lastname: Wahbi
authors.forename: Linda
authors.initials: L
authors.lastname: Aïssou
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Sorbets
authors.forename: Valérie
authors.initials: V
authors.lastname: Siam-Tsieu
authors.forename: Bruno
authors.initials: B
authors.lastname: Eymard
authors.forename: Christine
authors.initials: C
authors.lastname: Themar Noel
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Devaux
authors.forename: Odile
authors.initials: O
authors.lastname: Dessault
authors.forename: Denis
authors.initials: D
authors.lastname: Duboc
authors.forename: Christophe
authors.initials: C
authors.lastname: Meune
mesh_terms: D000293:Adolescent; D002648:Child; D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000000981:diagnostic imaging / Q000503:physiopathology*; D009200:Myocardial Contraction / Q000502:physiology*; D015635:Radionuclide Ventriculography / Q000379:methods; D013318:Stroke Volume / Q000502:physiology*; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000503:physiopathology*; D016277:Ventricular Function, Left / Q000502:physiology