Table 1.
Characteristic | Overall | FDCM | IDCM | P Value* |
---|---|---|---|---|
N† | 870 | 223 | 647 | |
Age at diagnosis | ||||
Mean (SD), years | 4.9 (5.9) | 6.2 (6.4) | 4.5 (5.8) | 0.001 |
Median (IQR), years | 1.2 (0.3–10.3) | 3.3 (0.3–12.1) | 1.0 (0.3–9.2) | 0.04 |
Age < 1 year at diagnosis, % | 47.4 | 42.2 | 49.2 | 0.07 |
Male, % | 50.6 | 54.7 | 49.1 | 0.16 |
Race/ethnicity, % | 0.06 | |||
White | 54.2 | 60.1 | 52.2 | |
Black | 21.2 | 16.1 | 23.0 | |
Hispanic | 17.3 | 15.1 | 18.0 | |
Other | 7.3 | 8.7 | 6.8 | |
Congestive heart failure at diagnosis, % | 74.6 | 64.1 | 78.2 | < 0.001 |
Family history of sudden death, % (total n) | 10.5 (727) | 39.0 (159) | 2.5 (568) | < 0.001 |
Left ventricular echocardiographic z-scores, mean (SD) | ||||
End-diastolic dimension (total n = 699, 175, 524) | 4.71 (2.60) | 4.12 (2.61) | 4.91 (2.57) | < 0.001 |
End-systolic dimension (total n = 614, 160, 454) | 6.35 (3.00) | 5.79 (2.89) | 6.55 (3.01) | 0.006 |
Fractional shortening (total n = 713, 183, 530) | −8.74 (3.95) | −7.85 (3.98) | −9.06 (3.89) | < 0.001 |
End-diastolic posterior wall thickness (total n = 553, 145, 408) | −0.60 (2.58) | −0.63 (2.66) | −0.59 (2.56) | 0.86 |
End-diastolic septal thickness (total n = 515, 126, 389) | −0.98 (1.92) | −0.67 (1.99) | −1.09 (1.88) | 0.03 |
Mass (total n = 545, 143, 402) | 2.49 (2.78) | 2.32 (3.08) | 2.55 (2.67) | 0.43 |
Medications at diagnosis (n) | ||||
Any cardiac medication | 94.7% (786) | 91.4% (198) | 95.8% (588) | 0.03 |
Anticongestive therapy¶ | 86% (843) | 82.2% (213) | 87.3% (630) | 0.07 |
Antiarrhythmic therapy | 20.6% (462) | 13.7% (139) | 23.5% (323) | 0.02 |
ACE inhibitor | 62.7% (461) | 60.9% (138) | 63.5% (323) | 0.60 |
Beta blocker | 13.2% (461) | 15.1% (139) | 12.4% (322) | 0.46 |
Carnitine | 1.5% (461) | 0.7% (139) | 1.9% (322) | 0.68 |
FDCM, familial dilated cardiomyopathy; IDCM, idiopathic dilated cardiomyopathy; IQR, interquartile range.
P values compare the differences between FDCM and IDCM.
Sample size is smaller than the total stated for selected variables (family history; LV mass).
Anticongestive therapy is defined as digoxin and/or diuretics