Research Article

Echocardiographic Features of Cardiomyopathy in Emery-Dreifuss Muscular Dystrophy

Table 1

Baseline characteristics of the study population.

EDMD patients, n = 41Healthy controls, n = 25 value

Age (years)43 (31–55)37 (36–44)0.17
Female (%)36.5 15/4148.0; 12/250.81
BMI (kg/m2)20.8 (19.3–25.3)22.6 (20.4–24.1)0.37
BSA (m2)1.72 (1.55–1.83)1.77 (1.62–1.93)0.10
EMD (sporadic/familial)12/17
LMNA (sporadic/familial)7/5
Muscular involvement (mild/moderate/severe)18/19/4
NT-proBNP (pg/ml)74.6 (49.2–168.6)31.0 (15.6–66.2)<0.001
NT-proANP (pg/ml)1.0 (0.8–1.9)0.8 (0.6–0.8)<0.001

BMI: body mass index; BSA: body surface area; EMD: mutation in EMD gen; LMNA: mutation in LMNA gene; NTpro-BNP: N-terminal prohormone B-type natriuretic peptide; NT-proANP: N-terminal prohormone A-type natriuretic peptide.