Research Article
Echocardiographic Features of Cardiomyopathy in Emery-Dreifuss Muscular Dystrophy
Table 3
Evaluation of systolic and diastolic function in EDMD and healthy controls.
| | | EDMD1 patients, n = 29 | EDMD2 patients, n = 12 | Healthy controls, n = 25 | value, EDMD1 versus EDMD2 | value, EDMD versus control |
| | LVEF (%) | 52.2 (46.4–58.7) | 54.0 (43.0–59.0) | 64.6 (62.1–67.3) | 0.94 | <0.001 | | s’ (cm/s) | 8.0 (7.0–10.1) | 8.3 (6.8–9.1) | 8.7 (8.4–9.1) | 0.90 | 0.02 | | TAPSE (mm) | 24.0 (18.5–25.5) | 22.0 (18.3–24.0) | 28.0 (24.0–30.0) | 0.56 | <0.001 | | TRPG (mmHg) | 20.0 (16.5–31.0) | 19.5 (0.0–30.0) | 10.0 (10.0–16.0) | 0.62 | <0.0001 | | e’ (cm/s) | 9.8 (8.1–11.3) | 8.0 (6.7–10.5) | 11.6 (11.0–12.5) | 0.27 | <0.001 | | a’ (cm/s) | 6.7 (5.9–7.4) | 6.5 (6.2–7.6) | 8.4 (7.6–9.7) | 1.00 | 1.00 | | E/e’ | 7.3 (5.4–10.2) | 6.5 (5.8–13.1) | 5.8 (5.5–6.4) | 0.74 | 0.01 | | LAVi (ml/m2) | 34.6 (26.9–39.6) | 35.9 (24.9–39.2) | 24.4 (20.7–26.4) | 0.81 | <0.001 | | Vprop (cm/s) | 61.8 (50.6–79.4) | 68.6 (40.9–73.8) | 71.0 (62.9–90.4) | 0.86 | 0.03 | | IVRT (ms) | 85.0 (72.5–95.5) | 69.0 (61.0–79.5) | 81.0 (66.5–91.5) | 0.02 | 0.84 |
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a’: late diastolic annulus velocity; e’: early diastolic annulus velocity; E/e’: mitral early peak velocity/annular velocity; IVRT: isovolumetric relaxation time; LVEF: left ventricular ejection fraction; LAVi: left atrial volume index; s’: systolic annulus velocity; TAPSE: tricuspid annular plane systolic excursion; TRPG: tricuspid regurgitation pressure gradient; Vprop: inflow propagation velocity.
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