Author/Authors :
Bornaun, Helen Kanuni Sultan Suleyman Educational and Research Hospital - Istanbul - Turkey , Yilmazer, Murat Muhtar Behcet Uz Children’s Hospital - Izmir - Turkey , Dedeoglu, Savas Uskudar University - Istanbul - Turkey , Dedeoglu, Reyhan Faculty Istanbul University - Istanbul - Turkey
Abstract :
Objectives: We aim to characterize cardiac morphological and functional changes by echocardiography that are present in the
early and late stages of Duchhenne type muscular dystrophy (DMD).
Methods: Sixty-two patients with DMD and 62 healthy children were included in the study. Study group was divided into patients
with systolic dysfunction group Ia and those without systolic dysfunction group Ib. Transthoracic echocardiography was performed
in all individuals in the study. Left ventricular end diastolic diameter (LVEDd), left ventricular end systolic diameter (LVSd), posterior wall thickness (LVPWd), interventricular septal thickness (IVSD), ejection fraction and fractional shortening (FS), LV mass index
(LVmassi), early (E) and late (A) transmitral inflow velocities, the ratio of early-to-late peak velocities (E/A) were recorded. Tissue
Doppler measurements of the peak early (E’) and late diastolic (A’) annular velocities, Doppler measurements of pressure half time
(PHT) and mitral valve area were measured.
Results: DMD patients had significantly higher LVmass, LVmass index and mitral valve area (P < 0.05). Patients showed significantly
lower EF values (P < 0.001), SF (P < 0.001), stroke volume (P < 0.05), cardiac output (P < 0.05), Mitral valve PHT (P < 0.001), Mitral E’
(P < 0.001), Mitral A’ (P < 0.001), E’/A’ ratio (P < 0.001) than the control patients. Group Ia patients had higher age, LVH and LV mass
index, LVEDd (P < 0.05), LVSd (P < 0.05), IVSd (P < 0.05) and LVPWd (P < 0.05) compared to group Ib patients (P < 0.05). There was
a positive linear correlation between age and LVPWd (P < 0.001, r = 0.446).
Conclusions: Our study showed that diastolic functions began to deteriorate in the early phase of DMD and systolic impairment
begins subclinically in early stages.
Keywords :
Muscular Dystrophy , Duchenne , Cardiac , Echocardiography , Diastole