An Exhaustive Echocardiographic Analysis of Left Ventricular Structure and Functions in Children with Duchenne Muscular Dystrophy

作者：Bornaun Helen^*; Yilmazer Murat Muhtar; Dedeoglu Savas; Dedeoglu Reyhan

来源：Iranian Journal of Pediatrics, 2018, 28(4): e62204.

DOI：10.5812/ijp.62204

• Muscular Dystrophy
• Duchenne
• Cardiac
• Echocardiography
• Diastole

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.

• 出版日期2018-8