Table 5.
Potential risk factors affecting MVA-free and HTX-free survival.
| Cumulate Incidence |
p-Value Log-Rank |
Univariable | Mutivariable | |||
|---|---|---|---|---|---|---|
| HR (95% CI) | p-Value Wald | HR (95% CI) | p-Value Wald | |||
| MVA, from date of first visit | at 8 years of follow-up (n = 44) | |||||
| Sex: Male vs. Female | 24 vs. 20 | 0.929 | 1.07 (0.24–4.79) | 0.929 | - | |
| Mutation type: Missense vs. Other | 25 vs. 22 | 0.727 | 0.69 (0.08–5.73) | 0.729 | - | |
| AV block: yes vs. no | 42 vs. 0 | 0.007 | NA | - | ||
| nsVT: yes vs. no | 37 vs. 9 | 0.031 | 7.38 (0.88–61.7) | 0.064 | - | |
| LVEF: <45% vs. >45% | 48 vs. 16 | 0.100 | 3.32 (073–15.04) | 0.120 | ||
| LVEF: <55% vs. >55% | 42 vs. 11 | 0.026 | 5.54 (1.02–27.78) | 0.047 | - | |
| NT-proBNP: >150 vs. <150 pg/mL | 67 vs. 6 | 0.002 | 13.40 (1.6–112.7) | 0.017 |
10.4
(1.21–89.79) |
0.010 |
| hsTn T: >20 vs. <20 ng/L | 66 vs. 6 | 0.003 | 13.16 (1.49–115.8) | 0.020 | - | |
| MVA, from date of birth | at 60 years (n = 53) | |||||
| Status: Proband vs. Relative | 65 vs. 62 | 0.746 | 1.18 (0.43; 3.29) | 0.746 | - | |
| Sex: Male vs. Female | 100 vs. 41 | 0.087 | 2.61 (0.84; 8.05) | 0.096 | - | |
| Mutation type: Missense vs. Other | 58 vs. 64 | 0.095 | 2.52 (0.82–7.72] | 0.106 | - | |
| End-stage HF, from date of birth | at 60 years (n = 53) | |||||
| Status: Proband vs. Relative | 83 vs. 41 | 0.034 | 3.71 (1.02; 13.47) | 0.046 | - | |
| Sex: Male vs. Female | 84 vs. 50 | 0.004 | 5.55 (1.52; 20.27) | 0.009 | 6.18 (1.66–23.0) | 0.007 |
| Mutation type: Missense vs. other | 100 vs. 63 | 0.041 | 3.18 (0.99; 10.25) | 0.052 |
3.83
(1.14–12.85) |
0.029 |
Legend: AV block: atrioventricular block; CI: confidence interval; HF: heart failure; hsTnT: high-sensitive Troponin T; HTX: heart transplantation; LVEF: left ventricular ejection fraction; MVA: malignant ventricular arrhythmia; NA: not applicable; nsVT: non-sustained ventricular tachycardia; NT-proBNP: N-terminal prohormone brain natriuretic peptide.