Table 4.
Clinical characteristics in 79 lamin A/C genotype-positive subjects in relation to severe outcome consisting of death or heart transplantation during follow-up
| No severe outcome (n = 60) | Heart transplantation or death (n = 19) | P-value | Univariate Cox regression |
||
|---|---|---|---|---|---|
| HR (95% CI) | P-value | ||||
| Age at penetrant phenotype (phenotype pos only; n=67, years) | 40 ± 13 | 40 ± 15 | 0.93 | ||
| Age at start of follow-up (years) | 36 ± 15 | 40 ± 5 | 0.31 | ||
| Follow-up time (years) | 6.9 ± 6.3 | 10.5 ± 5.6 | 0.03 | ||
| Female/male | 28/32 (47/53) | 8/11 (42/58) | 0.73 | 0.62 (0.23–1.69) | 0.35 |
| Missense/non-missense | 4/56 (7/93) | 3/16 (16/84) | 0.35 | 1.17 (0.33–4.15) | 0.81 |
| Atrial fibrillation | 31 (52) | 17 (90) | <0.01 | 1.02 (0.21–5.08) | 0.98 |
| Atrioventricular block | 36 (60) | 15 (88) | 0.03 | 2.33 (0.51–10.60) | 0.27 |
| Ventricular arrhythmias | 31 (52) | 16 (84) | 0.01 | 1.96 (0.56–6.82) | 0.29 |
| ICD | 33 (55) | 16 (84) | 0.02 | 0.76 (0.20–2.86) | 0.69 |
| Ejection fraction (%) | 50 ± 9 | 31 ± 12 | <0.001 | 0.94 (0.90–0.97) | 0.001a |
| End-diastolic diameter (mm) | 54 ± 7 | 61 ± 7 | <0.001 | 1.07 (0.99–1.15) | 0.06 |
Data are given as mean ± SD or n (%). P-value from Student’s t-test (left column) and from Cox univariate regression analysis of predictors of severe outcome (right column).
CI, confidence interval; HR, hazard ratio; ICD, implantable cardiac defibrillator.
a
All parameters were adjusted for ejection fraction in multivariate Cox regression without reaching significance (P > 0.05). Ejection fraction remained a significant predictor in all multivariate models.