Table 5.
Baseline Characteristics of Carriers of Multiple Variants and a Single Variant Among Adult Patients
| Characteristic | Adult Patients (n=83) | ||
|---|---|---|---|
| Single Variant (n=26) | Multiple Variants (n=6) | P Value | |
| Age at enrollment, y | 42.5 (35.0–46.5) | 55.0 (39.8–63.3) | 0.069 |
| Age at onset, y | 38.5 (29.5–44.0) | 52.5 (39.5–61.3) | 0.033 |
| Male sex, n (%) | 18 (69.2) | 4 (66.7) | 1.000 |
| Family history of cardiomyopathy, n (%) | 8 (30.8) | 1 (16.7) | 0.648 |
| NYHA class III/IV, n (%) | 14 (53.8) | 3 (50.0) | 1.000 |
| Comorbidities, n (%) | |||
| Coronary artery disease | 1 (3.8) | 0 (0.0) | 1.000 |
| Hypertension | 0 (0.0) | 0 (0.0) | |
| Diabetes mellitus | 2 (7.7) | 1 (16.7) | 0.476 |
| Hyperlipidemia | 4 (15.4) | 2 (33.3) | 0.310 |
| Other cardiomyopathies | 9 (34.6) | 2 (33.3) | 1.000 |
| Atrial fibrillation | 9 (34.6) | 2 (33.3) | 1.000 |
| Echocardiography | |||
| LVEDD, mm | 66.5 (56.3–72.0) | 58.5 (47.8–63.5) | 0.119 |
| LAD, mm | 43.5 (38.0–50.3) | 45.0 (35.3–50.3) | 0.981 |
| LVEF, % | 30.5 (24.8–41.8) | 41.5 (34.8–50.8) | 0.087 |
| Primary end point | |||
| Death and heart transplantation | 13 (50.0) | 3 (50.0) | 1.000 |
| Secondary end point | |||
| All‐cause death | 9 (34.6) | 3 (50.0) | 0.647 |
| Sudden cardiac death | 1 (3.8) | 0 (0.0) | 1.000 |
| Heart failure–related death | 8 (30.8) | 3 (50.0) | 0.390 |
| Cardiovascular death | 9 (34.6) | 3 (50.0) | 0.647 |
| Heart transplantation | 4 (15.4) | 0 (0.0) | 0.566 |
Data are given as median (interquartile range) unless otherwise indicated. LAD indicates left atrial diameter; LVEDD, left ventricular end‐diastolic dimension; LVEF, left ventricular ejection fraction; NYHA, New York Heart Association.