Table 1.
Baseline characteristics of male and female EMD variant-carriers
| Missing | Female | Male | |
|---|---|---|---|
| n | 21 | 38 | |
| Proband, n (%) | 0 | 0 | 26 (68.4) |
| Presence of cardiac phenotype, n (%) | 0 | 3 (14.3) | 33 (86.8) |
| Age at first evaluation, mean (SD) | 0 | 43.3 (16.8) | 33.4 (13.3) |
| White ethnicity, n (%) | 1 | 21 (100.0) | 35 (94.6) |
| MVA prior to baseline, n (%) | 0 | 0 | 2 (5.3) |
| Prior history of heart failure, n (%) | 0 | 0 | 4 (10.5) |
| Hypertension, n (%) | 0 | 5 (23.8) | 2 (5.3) |
| Diabetes mellitus, n (%) | 0 | 0 | 2 (5.3) |
| Prior conduction disease, n (%) | 0 | 1 (4.8) | 16 (42.1) |
| Device implanted by baseline, n (%) | 0 | 1 (4.8) | 17 (44.7) |
| ICD implanted by baseline, n (%) | 0 | 0 | 8 (21.1) |
| Prior atrial arrhythmia, n (%) | 0 | 0 | 16 (42.1) |
| Previous CVA, n (%) | 0 | 0 | 1 (2.6) |
| Neuromuscular involvement, n (%) | 0 | 0 | 28 (73.7) |
| Cardiac symptoms at baseline assessment, n (%) | 0 | 7 (33.3) | 16 (42.1) |
| Shortness of breath, n (%) | 0 | 2 (9.5) | 9 (23.7) |
| NYHA Class III or IV, n (%) | 0 | 0 | 2 (5.3) |
| Syncope, n (%) | 0 | 0 | 2 (5.3) |
| Palpitations, n (%) | 0 | 6 (28.6) | 5 (13.2) |
| On cardiac medications at baseline, n (%) | 1 | 6 (28.6) | 20 (54.1) |
| Sinus rhythm, n (%) | 1 | 19 (95.0) | 17 (44.7) |
| Baseline ECG paced, n (%) | 0 | 1 (4.8) | 11 (28.9) |
| PR interval (ms), median (Q1–Q3) | 30 | 162.5 (136.5–178.8) | 192.0 (154.0–224.5) |
| QRS duration (ms), median (Q1–Q3) | 17 | 85.0 (84.5–102.5) | 106.0 (96.0–115.0) |
| LBBB, n (%) | 19 | 0 | 2 (7.4) |
| LV internal diameter (diastole) (mm), median (Q1–Q3) | 7 | 46.0 (43.5–49.0) | 53.0 (48.0–58.0) |
| LV ejection fraction (%), median (Q1–Q3) | 1 | 62.0 (57.5–65.0) | 57.5 (45.0–64.0) |
| LA diameter (mm), median (Q1–Q3) | 23 | 32.0 (30.0–35.0) | 40.0 (35.0–48.8) |
| RV dilatation, n (%) | 0 | 0 | 3 (8.8) |
| PVCs/24 h on first Holter, median (Q1–Q3) | 30 | 40.0 (6.8–185.2) | 80.0 (1.5–1083.0) |
CVA, cerebrovascular accident; ECG, electrocardiogram; ICD, implantable cardioverter defibrillator; LA, left atrium; LBBB, left bundle branch block; LV, left ventricular; MVA, malignant ventricular arrhythmia; NYHA, New York Heart Association; PVC, premature ventricular complex; RV, right ventricular.