Pulsed Doppler tissue Imaging in dystrophinopathic cardiomyopathy

Cardiac dysfunction is a primary feature in patients and female carriers of Becker muscular dystrophy (BMD). Conventional echocardiography and pulsed Doppler tissue imaging (DTI) were performed in 28 patients with BMD, in 20 female carriers, and in 38 control participants (20 men and 18 women). Left ventricular ejection fraction (LVEF) was lower in BMD patients (P<.02) and carriers (P<.02) than in normal participants. Two subgroups of BMD patients were identified: A1=LVEF greater than or equal to 55% (n=20) and A2=LVEF < 55% (n=8). The carriers displayed LVEF >= 55%. Compared with control participants the diastolic alterations by conventional echocardiography were lower early filling peak in the subgroup A1 (P<.05) and prolonged isovolumic relaxation time in A1, A2, and in carriers (at least P<.02). Furthermore, pulsed DTI showed lower early diastolic wave peak at basal septum in A2 (P<.05) and in carriers (P<.0001); at lateral mitral annulus and at basal inferior wall in A1, A2, and in carriers (at least P<.05); lower early/late diastolic wave ratio at basal septum and lateral mitral annulus (P<.05) in carriers; and prolonged isovolumic relaxation time in A1, A2, and in carriers (at least P<.02), except at lateral mitral annulus of carriers. Systolic parameters investigated by pulsed DTI detected lower peak systolic wave at basal septum in A1, A2, and carriers (P<.02); at lateral mitral annulus in carriers (P<.02); at basal inferior wall in A1, A2 (P<.02), and in carriers (P<.0001); and lower time-velocity integral of S wave at each segment in A1, A2, and in carriers. in dystrophinopathic cardiomyopathy, pulsed DTI may be a useful technique to assess diastolic dysfunction and appears to be a promising tool in identifying early regional systolic alterations in patients and carriers with normal LVEF.