Table 1.
Baseline characteristics of participants in VANISH by cohort
| Total (n=212) | Overt HCM – Primary Analysis Cohort (n=178) | Preclinical HCM- Exploratory Cohort (n=34) | |
|---|---|---|---|
| Age, years | 22.2 (9.7) | 23.3 (10.1) | 16.5 (4.9) |
| Female gender (%) | 86 (40.6) | 69 (38.8) | 17 (50.0) |
| Race (%) | |||
| White | 203 (95.8) | 169 (94.9) | 34 (100.0) |
| Black | 3 (1.4) | 3 (1.7) | 0 (0.0) |
| Other | 6 (2.8) | 6 (3.4) | 0 (0.0) |
| NYHA class (%) | |||
| I | 198 (93.4) | 164 (92.1) | 34 (100.0) |
| II | 14 (6.6) | 14 (7.9) | 0 (0.0) |
| Cardiac medications (%) | |||
| Beta-blocker | 36 (17.0) | 34 (19.1) | 2 (5.9) |
| Calcium channel blocker | 5 (2.4) | 5 (2.8) | 0 (0.0) |
| Family history (%) | 180 (85) | 146 (82) | 34 (100) |
| No. of affected relatives (median, q1, q3) | 2 (1, 4) | 2 (1, 3) | 3 (2, 4) |
| Heart rate, bpm | 70 (14) | 70 (14) | 74 (13) |
| Blood pressure, mmHg | |||
| systolic | 117 (12) | 118 (11) | 114 (12) |
| diastolic | 68 (10) | 69 (10) | 66 (8) |
| Echocardiographic findings | |||
| Max. LV Wall Thickness, mm | 15 (4) | 16 (4) | 10 (1) |
| Max LV Wall Thickness Z-Score | 6.4 (4.2) | 7.3 (4.0) | 2.1 (0.6) |
| e’ lateral Z-Score | −1.7 (1.5) | −2.0 (1.4) | −0.4 (1.0) |
| e’ septum Z-Score | −1.6 (1.4) | −1.9 (1.3) | −0.4 (1.0) |
| Average e’, cm/s | 12.0 (3.6) | 11.3 (3.5) | 15.4 (2.0) |
| E/e’ average | 7.3 (2.4) | 7.6 (2.5) | 6.0 (1.2) |
| LVEF, % | 66 (8) | 66 (8) | 64 (4) |
| LA Diameter, cm | 3.4 (0.6) | 3.4 (0.7) | 3.1 (0.4) |
| Peak left ventricular outflow tract gradient at rest, mmHg | 7 (3) | 7 (4) | 6 (2) |
| Peak left ventricular outflow tract gradient during Valsalva maneuver, mmHg | 7 (4) | 7 (4) | 6 (2) |
| Cardiopulmonary exercise test | |||
| Peak VO2, ml/kg/min | 33.0 (9.2) | 31.7 (8.9) | 39.0 (8.1) |
| % predicted maximal VO2 | 82 (20) | 81 (21) | 87(17) |
| CMR findings | |||
| LGE present (%) | 29 (15.3) | 29 (18.6) | 0 (0.0) |
| LGE mass, g | 19 (18) | 19 (18) | - |
| LGE mass, % of LV | 13 (13) | 13 (13) | - |
| LVEDV, ml | 108 (31) | 108 (31) | 108 (27) |
| LVESV, ml | 37 (14) | 37 (14) | 40 (12) |
| LV mass, g | 116 (42) | 122 (43) | 89 (27) |
| LV mass indexed to BSA, g/m2 | 63 (17) | 65 (18) | 53 (9) |
| LA volume diastolic, ml | 29 (19) | 31 (20) | 19 (6) |
| LA volume systolic, ml | 66 (30) | 70 (31) | 51 (17) |
| Max LV wall thickness, mm* | 12 (4) | 12 (4) | 8 (1) |
Results are presented as % or as mean (SD) or median (IQR). CMR indicates, cardiac magnetic resonance imaging; E, peak early mitral inflow velocity; e’, peak early mitral annular relaxation velocity; LA, left atrial; LGE, late gadolinium enhancement; LV, left ventricular; LVEDV, left ventricular end-diastolic volume; LVEF, left ventricular ejection fraction; LVESV, left ventricular end-systolic volume; NYHA, New York Heart Association Class; VO2, peak oxygen uptake. Difference between groups assessed by Chi-Square or Fisher’s exact test or two-sample t test depending on number and type of variable.
The maximum LV wall thickness from CMR is the maximum value across the 16 American Heart Association segments.