Table 1.
Study group characteristics and comparison of clinical and instrumental findings between individuals with Danon disease and other aetiologies of non‐ischemic heart failure
| Characteristic | Danon disease (n = 7) | Non‐Danon disease (n = 53) | Overall (n = 60) | P value |
|---|---|---|---|---|
| Age at first symptoms (year) | 16 (15–24) | 24 (12–32) | 22 (12–31) | 0.398 |
| Age at progression (year) | 25 (21–28) | 30 (19–36) | 28 (20–35) | 0.228 |
| Cardiomypathy type | 0.022 * | |||
| Hypertrophic | 4 (57%) | 5 (9%) | 9 (15%) | |
| Dilated | 3 (43%) | 43 (81%) | 46 (77%) | |
| Restrictive | 0 (0%) | 4 (8%) | 4 (6%) | |
| Left‐ventricular non‐compaction | 0 (0%) | 1 (2%) | 1 (2%) | |
| NYHA functional class | (n = 7) | (n = 45) | (n = 52) | 0.754 |
| I | 1 (14%) | 3 (7%) | 4 (8%) | |
| II | 1 (14%) | 10 (22%) | 11 (21%) | |
| III | 4 (58%) | 21 (47%) | 25 (48%) | |
| IV | 1 (14%) | 11 (24%) | 12 (23%) | |
| Arrhythmia | 0.052 | |||
| Atrial fibrillation | 3 (43%) | 4 (8%) | 7 (12%) | |
| Ventricular tachyarrhythmia | 0 (0%) | 5 (10%) | 5 (8%) | |
| Electrocardiogram | (n = 7) | (n = 42) | (n = 49) | |
| PR duration (ms) | 154 (152–160) | 166 (159–184) | 162 (154–184) | 0.268 |
| QRS duration (ms) | 144 (137–187) | 96 (80–110) | 102 (83–121) | 0.001 ** |
| Delta‐waves | 3 (43%) | 0 (0%) | 3 (6%) | 0.002 ** |
| LBBB | 3 (43%) | 5 (12%) | 8 (16%) | 0.068 |
| Echocardiography | (n = 7) | (n = 46) | (n = 53) | |
| LVEDD (mm) | 62 (49–67) | 61 (56–69) | 62 (56–68) | 0.537 |
| LVEF (mm) | 20 (20–39) | 25 (20–30) | 24 (20–30) | 0.749 |
| Interventricular septum (mm) | 14 (9–14) | 8 (7–9) | 8 (7–9) | 0.001 ** |
| Posterior wall (mm) | 12 (10–14) | 8 (7–9) | 8 (7–9) | 0.002 ** |
| Mitral regurgitation | (n = 7) | (n = 50) | (n = 57) | 0.899 |
| None or trace | 4 (58%) | 20 (40%) | 24 (42%) | |
| Mild | 1 (14%) | 10 (20%) | 11 (19%) | |
| Moderate | 1 (14%) | 12 (24%) | 13 (23%) | |
| Severe | 1 (14%) | 8 (16%) | 9 (16%) |
LBBB, left bundle branch block; LVEDD, left ventricular end‐diastolic diameter; LVEF, left ventricular ejection fraction; NYHA, New York Heart Association.
*
P ˂ 0.05.
**
P ˂ 0.01.