Comparisons of clinical and electrocardiogram and echocardiogram characteristics in symptomatic subjects with cardiac or cardiac + neurologic phenotype
| . | N (total) . | Total (N = 508) . | Cardiac (N = 210) . | Cardiac + neurologic (N = 298) . | P-value . |
|---|---|---|---|---|---|
| Age at onset (years), mean (SD) | 508 | 53.1 (16.9) | 60.8 (14.5) | 47.6 (16.3) | <0.0001 |
| Age at inclusion (years), mean (SD) | 508 | 61.3 (15.5) | 67.3 (13.5) | 57.0 (15.5) | <0.0001 |
| Sex, n (%) | 508 | <0.0001 | |||
| Male | 335 (65.9) | 164 (78.1) | 171 (57.4) | ||
| Female | 173 (34.1) | 46 (21.9) | 127 (42.6) | ||
| NYHA | 501 | <0.0001 | |||
| No HF/I | 265 (52.9) | 68 (32.5) | 197 (67.5) | ||
| II–IV | 236 (47.1) | 141 (67.5) | 95 (32.5) | ||
| Diastolic interventricular septum thickness (mm), median (Q1–Q3) | 270 | 17.0 (14.0–20.0) | 18.0 (16.0–21.0) | 16.0 (13.0–20.0) | 0.0006 |
| Diastolic LV posterior wall thickness (mm), median (Q1–Q3) | 248 | 15.0 (12.0–18.0) | 16.0 (13.0–18.0) | 13.0 (10.0–17.0) | 0.0001 |
| Left atrial diameter (mm), median (Q1–Q3) | 221 | 43.0 (39.0–48.0) | 46.0 (42.0–50.0) | 41.0 (36.0–46.0) | <0.0001 |
| LV ejection fraction (%), median (Q1–Q3) | 216 | 60.9 (48.6–69.8) | 55.5 (46.4–68.0) | 65.3 (57.4–70.8) | 0.0002 |
| LV ejection fraction <40%, n (%) | 216 | 20 (9.3) | 14 (12.4) | 6 (5.8) | 0.0964 |
| LV ejection fraction <50%, n (%) | 216 | 61 (28.2) | 43 (38.1) | 18 (17.5) | 0.0008 |
| LV end-diastolic diameter (mm), median (Q1–Q3) | 237 | 45.0 (42.0–49.0) | 45.0 (42.0–50.0) | 45.0 (41.0–48.0) | 0.5533 |
| E-wave deceleration time (ms), median (Q1–Q3) | 118 | 179.0 (144.0–221.0) | 175.5 (142.0–200.0) | 183.5 (144.0–226.0) | 0.3134 |
| Stroke volume (mL), median (Q1–Q3) | 71 | 60 (49–75) | 55 (48–69) | 62 (50–76) | 0.2637 |
| Restrictive filling pattern, n (%) | 79 | 0.0240 | |||
| <1 | 20 (25.3) | 5 (20.0) | 15 (27.8) | ||
| 1–2 | 37 (46.8) | 8 (32.0) | 29 (53.7) | ||
| >2 | 22 (27.8) | 12 (48.0) | 10 (18.5) | ||
| LV mass among men (g/m2), median (Q1–Q3) | 172 | 336.4 (267.0–412.5) | 350.7 (295.0–427.0) | 298.4 (212.0–389.9) | 0.0069 |
| Mitral thickening, n (%) | 140 | 44 (31.4) | 26 (32.9) | 18 (29.5) | 0.6671 |
| Tricuspid thickening, n (%) | 136 | 18 (13.2) | 11 (14.1) | 7 (12.1) | 0.7293 |
| . | N (total) . | Total (N = 508) . | Cardiac (N = 210) . | Cardiac + neurologic (N = 298) . | P-value . |
|---|---|---|---|---|---|
| Age at onset (years), mean (SD) | 508 | 53.1 (16.9) | 60.8 (14.5) | 47.6 (16.3) | <0.0001 |
| Age at inclusion (years), mean (SD) | 508 | 61.3 (15.5) | 67.3 (13.5) | 57.0 (15.5) | <0.0001 |
| Sex, n (%) | 508 | <0.0001 | |||
| Male | 335 (65.9) | 164 (78.1) | 171 (57.4) | ||
| Female | 173 (34.1) | 46 (21.9) | 127 (42.6) | ||
| NYHA | 501 | <0.0001 | |||
| No HF/I | 265 (52.9) | 68 (32.5) | 197 (67.5) | ||
| II–IV | 236 (47.1) | 141 (67.5) | 95 (32.5) | ||
| Diastolic interventricular septum thickness (mm), median (Q1–Q3) | 270 | 17.0 (14.0–20.0) | 18.0 (16.0–21.0) | 16.0 (13.0–20.0) | 0.0006 |
| Diastolic LV posterior wall thickness (mm), median (Q1–Q3) | 248 | 15.0 (12.0–18.0) | 16.0 (13.0–18.0) | 13.0 (10.0–17.0) | 0.0001 |
| Left atrial diameter (mm), median (Q1–Q3) | 221 | 43.0 (39.0–48.0) | 46.0 (42.0–50.0) | 41.0 (36.0–46.0) | <0.0001 |
| LV ejection fraction (%), median (Q1–Q3) | 216 | 60.9 (48.6–69.8) | 55.5 (46.4–68.0) | 65.3 (57.4–70.8) | 0.0002 |
| LV ejection fraction <40%, n (%) | 216 | 20 (9.3) | 14 (12.4) | 6 (5.8) | 0.0964 |
| LV ejection fraction <50%, n (%) | 216 | 61 (28.2) | 43 (38.1) | 18 (17.5) | 0.0008 |
| LV end-diastolic diameter (mm), median (Q1–Q3) | 237 | 45.0 (42.0–49.0) | 45.0 (42.0–50.0) | 45.0 (41.0–48.0) | 0.5533 |
| E-wave deceleration time (ms), median (Q1–Q3) | 118 | 179.0 (144.0–221.0) | 175.5 (142.0–200.0) | 183.5 (144.0–226.0) | 0.3134 |
| Stroke volume (mL), median (Q1–Q3) | 71 | 60 (49–75) | 55 (48–69) | 62 (50–76) | 0.2637 |
| Restrictive filling pattern, n (%) | 79 | 0.0240 | |||
| <1 | 20 (25.3) | 5 (20.0) | 15 (27.8) | ||
| 1–2 | 37 (46.8) | 8 (32.0) | 29 (53.7) | ||
| >2 | 22 (27.8) | 12 (48.0) | 10 (18.5) | ||
| LV mass among men (g/m2), median (Q1–Q3) | 172 | 336.4 (267.0–412.5) | 350.7 (295.0–427.0) | 298.4 (212.0–389.9) | 0.0069 |
| Mitral thickening, n (%) | 140 | 44 (31.4) | 26 (32.9) | 18 (29.5) | 0.6671 |
| Tricuspid thickening, n (%) | 136 | 18 (13.2) | 11 (14.1) | 7 (12.1) | 0.7293 |
Percentages shown are the proportion of subjects with each measure out of the total number of subjects for which data on that measure was available. The Kruskal–Wallis test was performed to calculate P-values by comparing medians between groups for continuous variables. The Pearson χ2 test was performed to calculate P-values for variables with cell counts >5.
HF, heart failure; LV, left ventricular; NYHA, New York Heart Association (classification); Q1, lower quartile; Q3, upper quartile; SD, standard deviation.
Comparisons of clinical and electrocardiogram and echocardiogram characteristics in symptomatic subjects with cardiac or cardiac + neurologic phenotype
| . | N (total) . | Total (N = 508) . | Cardiac (N = 210) . | Cardiac + neurologic (N = 298) . | P-value . |
|---|---|---|---|---|---|
| Age at onset (years), mean (SD) | 508 | 53.1 (16.9) | 60.8 (14.5) | 47.6 (16.3) | <0.0001 |
| Age at inclusion (years), mean (SD) | 508 | 61.3 (15.5) | 67.3 (13.5) | 57.0 (15.5) | <0.0001 |
| Sex, n (%) | 508 | <0.0001 | |||
| Male | 335 (65.9) | 164 (78.1) | 171 (57.4) | ||
| Female | 173 (34.1) | 46 (21.9) | 127 (42.6) | ||
| NYHA | 501 | <0.0001 | |||
| No HF/I | 265 (52.9) | 68 (32.5) | 197 (67.5) | ||
| II–IV | 236 (47.1) | 141 (67.5) | 95 (32.5) | ||
| Diastolic interventricular septum thickness (mm), median (Q1–Q3) | 270 | 17.0 (14.0–20.0) | 18.0 (16.0–21.0) | 16.0 (13.0–20.0) | 0.0006 |
| Diastolic LV posterior wall thickness (mm), median (Q1–Q3) | 248 | 15.0 (12.0–18.0) | 16.0 (13.0–18.0) | 13.0 (10.0–17.0) | 0.0001 |
| Left atrial diameter (mm), median (Q1–Q3) | 221 | 43.0 (39.0–48.0) | 46.0 (42.0–50.0) | 41.0 (36.0–46.0) | <0.0001 |
| LV ejection fraction (%), median (Q1–Q3) | 216 | 60.9 (48.6–69.8) | 55.5 (46.4–68.0) | 65.3 (57.4–70.8) | 0.0002 |
| LV ejection fraction <40%, n (%) | 216 | 20 (9.3) | 14 (12.4) | 6 (5.8) | 0.0964 |
| LV ejection fraction <50%, n (%) | 216 | 61 (28.2) | 43 (38.1) | 18 (17.5) | 0.0008 |
| LV end-diastolic diameter (mm), median (Q1–Q3) | 237 | 45.0 (42.0–49.0) | 45.0 (42.0–50.0) | 45.0 (41.0–48.0) | 0.5533 |
| E-wave deceleration time (ms), median (Q1–Q3) | 118 | 179.0 (144.0–221.0) | 175.5 (142.0–200.0) | 183.5 (144.0–226.0) | 0.3134 |
| Stroke volume (mL), median (Q1–Q3) | 71 | 60 (49–75) | 55 (48–69) | 62 (50–76) | 0.2637 |
| Restrictive filling pattern, n (%) | 79 | 0.0240 | |||
| <1 | 20 (25.3) | 5 (20.0) | 15 (27.8) | ||
| 1–2 | 37 (46.8) | 8 (32.0) | 29 (53.7) | ||
| >2 | 22 (27.8) | 12 (48.0) | 10 (18.5) | ||
| LV mass among men (g/m2), median (Q1–Q3) | 172 | 336.4 (267.0–412.5) | 350.7 (295.0–427.0) | 298.4 (212.0–389.9) | 0.0069 |
| Mitral thickening, n (%) | 140 | 44 (31.4) | 26 (32.9) | 18 (29.5) | 0.6671 |
| Tricuspid thickening, n (%) | 136 | 18 (13.2) | 11 (14.1) | 7 (12.1) | 0.7293 |
| . | N (total) . | Total (N = 508) . | Cardiac (N = 210) . | Cardiac + neurologic (N = 298) . | P-value . |
|---|---|---|---|---|---|
| Age at onset (years), mean (SD) | 508 | 53.1 (16.9) | 60.8 (14.5) | 47.6 (16.3) | <0.0001 |
| Age at inclusion (years), mean (SD) | 508 | 61.3 (15.5) | 67.3 (13.5) | 57.0 (15.5) | <0.0001 |
| Sex, n (%) | 508 | <0.0001 | |||
| Male | 335 (65.9) | 164 (78.1) | 171 (57.4) | ||
| Female | 173 (34.1) | 46 (21.9) | 127 (42.6) | ||
| NYHA | 501 | <0.0001 | |||
| No HF/I | 265 (52.9) | 68 (32.5) | 197 (67.5) | ||
| II–IV | 236 (47.1) | 141 (67.5) | 95 (32.5) | ||
| Diastolic interventricular septum thickness (mm), median (Q1–Q3) | 270 | 17.0 (14.0–20.0) | 18.0 (16.0–21.0) | 16.0 (13.0–20.0) | 0.0006 |
| Diastolic LV posterior wall thickness (mm), median (Q1–Q3) | 248 | 15.0 (12.0–18.0) | 16.0 (13.0–18.0) | 13.0 (10.0–17.0) | 0.0001 |
| Left atrial diameter (mm), median (Q1–Q3) | 221 | 43.0 (39.0–48.0) | 46.0 (42.0–50.0) | 41.0 (36.0–46.0) | <0.0001 |
| LV ejection fraction (%), median (Q1–Q3) | 216 | 60.9 (48.6–69.8) | 55.5 (46.4–68.0) | 65.3 (57.4–70.8) | 0.0002 |
| LV ejection fraction <40%, n (%) | 216 | 20 (9.3) | 14 (12.4) | 6 (5.8) | 0.0964 |
| LV ejection fraction <50%, n (%) | 216 | 61 (28.2) | 43 (38.1) | 18 (17.5) | 0.0008 |
| LV end-diastolic diameter (mm), median (Q1–Q3) | 237 | 45.0 (42.0–49.0) | 45.0 (42.0–50.0) | 45.0 (41.0–48.0) | 0.5533 |
| E-wave deceleration time (ms), median (Q1–Q3) | 118 | 179.0 (144.0–221.0) | 175.5 (142.0–200.0) | 183.5 (144.0–226.0) | 0.3134 |
| Stroke volume (mL), median (Q1–Q3) | 71 | 60 (49–75) | 55 (48–69) | 62 (50–76) | 0.2637 |
| Restrictive filling pattern, n (%) | 79 | 0.0240 | |||
| <1 | 20 (25.3) | 5 (20.0) | 15 (27.8) | ||
| 1–2 | 37 (46.8) | 8 (32.0) | 29 (53.7) | ||
| >2 | 22 (27.8) | 12 (48.0) | 10 (18.5) | ||
| LV mass among men (g/m2), median (Q1–Q3) | 172 | 336.4 (267.0–412.5) | 350.7 (295.0–427.0) | 298.4 (212.0–389.9) | 0.0069 |
| Mitral thickening, n (%) | 140 | 44 (31.4) | 26 (32.9) | 18 (29.5) | 0.6671 |
| Tricuspid thickening, n (%) | 136 | 18 (13.2) | 11 (14.1) | 7 (12.1) | 0.7293 |
Percentages shown are the proportion of subjects with each measure out of the total number of subjects for which data on that measure was available. The Kruskal–Wallis test was performed to calculate P-values by comparing medians between groups for continuous variables. The Pearson χ2 test was performed to calculate P-values for variables with cell counts >5.
HF, heart failure; LV, left ventricular; NYHA, New York Heart Association (classification); Q1, lower quartile; Q3, upper quartile; SD, standard deviation.
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