Research Article

Echocardiographic Features of Cardiomyopathy in Emery-Dreifuss Muscular Dystrophy

Table 4

Percentage of EDMD patients meeting the following criteria.

EDMD1EDMD2 value

LVEDV >150/106 ml (M/F) (%)41.4; 12/2941.7; 5/121.00
LVEDVi > 74/61 ml/m2 (M/F) (%)44.8; 13/2941.7; 5/121.00
LAVi > 34 ml/m2 (%)48.3; 14/2958.3; 7/120.74
RAVi > 32/27 ml/m2 (M/F) (%)69.0; 20/2975.0; 9/121.00
RV > 30 mm (%)6.9; 2/2925.0; 3/120.14
LVEF < 52/54 % (M/F) (%)41.4; 12/2941.7; 5/121.00
TAPSE < 17 mm (%)6.9; 2/2916.7; 2/120.57
E/e’ > 14 (%)16.0; 4/2516.7; 2/121.00
Vprop < 45 cm/s (%)12.5; 3/2433.3; 4/120.19

E/e’: mitral early peak velocity/annular velocity; LAVi: left atrial volume index; LVEDV: left ventricular end-diastolic volume; LVEDVi: left ventricular end-diastolic volume index; LVEF: left ventricular ejection fraction; RAVi: right atrial volume index; RV: right ventricle; TAPSE: tricuspid annular plane systolic excursion; Vprop: inflow propagation velocity.