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Reduced inotropic reserve is predictive of further degradation in left ventricular ejection fraction in patients with Duchenne muscular dystrophy
Author(s) -
Goudot FrançoisXavier,
Wahbi Karim,
Aïssou Linda,
Sorbets Emmanuel,
SiamTsieu Valérie,
Eymard Bruno,
Themar Noel Christine,
Devaux JeanYves,
Dessault Odile,
Duboc Denis,
Meune Christophe
Publication year - 2015
Publication title -
european journal of heart failure
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.149
H-Index - 133
eISSN - 1879-0844
pISSN - 1388-9842
DOI - 10.1002/ejhf.213
Subject(s) - ejection fraction , medicine , inotrope , cardiology , duchenne muscular dystrophy , radionuclide ventriculography , dobutamine , heart failure , muscular dystrophy , hemodynamics
Aims 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. Methods and 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. Conclusion 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.