Table 1.
Clinical characteristics of subjects
| MYH7 | R92WTNNT2 | Controls | |
|---|---|---|---|
| N | 51 | 30 | 68 |
| Males, % (n) | 35 (69) | 12 (41) | 31 (46) |
| Age, years | 38 (17–72) | 34 (14–66) | 34 (17–63) |
| Body surface area, kg/m2 | 1.9 (1.3–2.5) | 1.7 (1.3–2.1) | 1.8 (1.3–2.3) |
| LVM, g | 182 (71–477) | 126 (48–290) | 122 (58–272) |
| maxLVWT, mm | 13.8 (7.7–38.2) | 11.6 (6.7–30.0) | 10.1 (7.1–27.0) |
| Left atrial diameter, mm | 35.8 (20.0–65.0) | 32.9 (21.0–49.0) | 30.6 (22.0–45.0) |
| LV end-diastolic diameter, mm | 44.0 (32.0–52.0) | 40.5 (33.0–48.0) | 44.0 (37.0–60.0) |
| LV end-systolic diameter, mm | 26.8 (17.0–38.0) | 22.7 (16.1–33.2) | 28.0 (21.0–44.0) |
| Ejection fraction, % | 70 (57–81) | 71 (61–79) | 65 (52–75) |
| SV, mL | 83 (33–124) | 78 (43–103) | 80 (48–133) |
| Cardiac output at rest | 6.2 (2.0–11.3) | 5.3 (3.3–8.9) | 6.0 (4.2–10.9) |
| Resting HR, bpm | 75 (60–145) | 72 (55–118) | 78 (48–123) |
| SBPresting, mmHg | 120 (80–140) | 110 (90–130) | 115 (90–160) |
| DBPresting, mmHg | 80 (60–100) | 70 (60–90) | 80 (60–100) |
| Outflow tract gradient >30 mmHg | 0 (0) | 0 (0) | 0 (0) |
| Systolic anterior motion of the mitral valve | 4 (8) | 0 (0) | 0 (0) |
| MR = 1 | 18 (36) | 4 (15) | 4 (7) |
| MR = 2 | 1 (2) | 1 (4) | 1 (2) |
| NYHA 2 | 8 (16) | 3 (11) | 2 (3) |
| Atrial fibrillation | 2 (4) | 2 (7) | 0 (0) |
| Hypertension diagnosis | 7 (14) | 0 (0) | 2 (3) |
| maxLVWT ≥30 mm | 4 (8) | 1 (3) | 0 (0) |
| Syncope | 4 (8) | 3 (10) | 2 (3) |
| % Families with SCD | 39 | 57 | NA |
Note: Values are given as median (range) for continuous variables and as numbers (percentage) for categorical variables. No individuals were above New York Heart Association (NYHA) class 2 or mitral regurgitation (MR) score 2 (according to Zhogbi et al.33 2003). NA = not applicable.