Table 2.
Clinical presentation of dilated cardiomyopathy in relation to familial vs. sporadic presentation, mutation status, and causative gene in the Finnish dilated cardiomyopathy cohort (n = 145)
| Familial (n = 63) | Sporadic (n = 82) | P | Mutation-positive (n = 50) | No mutation (n = 95) | P | TTN (n = 25) | LMNA (n = 12) | DSP (n = 7) | Other (n = 6) | |
|---|---|---|---|---|---|---|---|---|---|---|
| Age at diagnosis (years) | 44.3 ± 12.2 | 46.8 ± 11.7 | 0.21 | 43.7 ± 11.8 | 46.7 ± 11.9 | 0.12 | 43.4 ± 11.4 | 46.7 ± 13.3*d | 49.0 ± 4.9*d | 31.7 ± 9.0*b,c |
| BMI (kg/m2) | 26.0 ± 4.3 | 25.6 ± 3.9 | 0.51 | 24.9 ± 3.9 | 26.2 ± 4.2 | 0.04 | 25.3 ± 3.8 | 23.1 ± 1.5 | 25.6 ± 6.1 | 25.6 ± 4.2 |
| LVEF (%) | 25.2 ± 10.2 | 23.0 ± 7.5 | 0.14 | 24.9 ± 10.4 | 23.5 ± 7.9 | 0.31 | 23.1 ± 9.3 | 27.3 ± 10.1 | 29.7 ± 10.6 | 23.3 ± 14.6 |
| LVEDD index (mm/m2) | 35.1 ± 5.2 | 36.9 ± 5.1 | 0.05 | 36.2 ± 3.7 | 35.3 ± 5.7 | 0.48 | 36.1 ± 3.7*b | 33.4 ± 5.5*a | 37.3 ± 7.4 | 36.0 ± 5.2 |
| Alive (%) | 79.4% | 85.4% | 0.23 | 79.6% | 84.4% | 0.52 | 88.0%*b | 58.3%*a | 85.7% | 83.3% |
| None | 50.0% | 56.1% | 0.38 | 57.1% | 52.1% | 0.79 | 68.0%*b | 8.3%*a,b,c | 85.7%*b | 66.7%*b |
| Pacemaker | 11.1% | 3.7% | 10.2% | 5.2% | 0.0%*b | 41.7%*a,c | 0.0%*b | 0.0%*b | ||
| ICD | 19.0% | 17.1% | 16.3% | 18.8% | 20.0% | 8.3% | 14.3% | 16.7% | ||
| CRT | 7.9% | 6.1% | 6.1% | 7.3% | 4.0% | 8.3% | 0.0% | 16.7% | ||
| CRT-D | 11.1% | 17.1% | 10.2% | 16.7% | 8.0% | 33.3% | 0.0% | 0.0% | ||
| Any ICD (ICD + CRT-D) | 30.1% | 34.2% | 26.5% | 35.5% | 28.0% | 41.6% | 14.3% | 16.7% | ||
| Angiography | 76.2% | 80.5% | 0.55 | 75.5% | 80.2% | 0.67 | 84.0% | 75.0% | 57.1% | 66.7% |
| Resuscitated (%) | 22.2% | 17.1% | 0.54 | 20.4% | 18.8% | 0.44 | 12.0%*b | 41.7%*a | 28.6% | 16.7% |
| Transplantation (%) | 27.0% | 31.7% | 0.59 | 32.7% | 28.1% | 0.70 | 28.0% | 41.7% | 28.6% | 33.3% |
| Age at transplantation | 49.7 ± 10.3 | 46.9 ± 10.4 | 0.39 | 45.9 ± 8.8 | 49.2 ± 11.1 | 0.32 | 46.7 ± 6.8 | 44.8 ± 11.2 | 55.0 ± 5.7 | 37.0 ± 4.2 |
| Atrial fibrillation (%) | 47.6% | 37.8% | 0.24 | 51.0% | 37.5% | 0.16 | 40.0%*b | 83.3%*a,c | 28.6%*b | 50.0% |
BMI, body mass index; LVEF, left ventricular ejection fraction; LVEDD index, left ventricular end-diastolic diameter indexed to body surface area; ICD, implantable cardioverter defibrillator; CRT, cardiac resynchronization therapy; CRT-D, pacemaker with CRT + ICD. All data are presented percentages or mean ± SD. Resuscitated includes both resuscitated and those with appropriate shocks by ICD. Other mutation group includes two RBM20 mutations, one in each MYH7, TNNT2, TCAP, and DMD.
*Mean significant difference compared with other mutation group (aTTN, bLMNA, cDSP, and dother).