Table 2.
Transplant-free survival for each type of cardiomyopathy (CM).
| Patient characteristics | Transplant-free survival rate after diagnosis | References | |||||
|---|---|---|---|---|---|---|---|
| Type of CM | Subgroups | Age range at diagnosis (median age) | Number of cases | 1 y | 5 y |
10 y (>10 y) |
|
| DCM | All | 0–10 y | 175 | 74% | 65% | 62% (20 y: 56%) |
NACCS (22) |
| All | 0–18 y (1.54 y) | 1,426 | 69% | 54% | 46% | PCMR (20) | |
| Idiopathic | 941 | 61% | 47% | 42% | |||
| Myocarditis | 222 | 79% | 73% | 60% | |||
| Familial | 66 | 81% | 59% | 59% | |||
| IEM | 54 | 84% | 78% | 78% | |||
| MFS | 15 | 91% | 76% | 76% | |||
| NMD | 125 | 83% | 52% | 26% | |||
| HCM | All | 0–10 y (0.48 y) | 80 | 86% | – | 80% (20 y: 78%) |
NACCS (8) |
| All | 0–18 y | 855 | – | – | – | PCMR (23)a | |
| Idiopathic | (7.07 y) | 634 | 94.4 % | 89.8% | 85.3% | ||
| IEM | (0.42 y) | 74 | 53.6% | 41.7% | – | ||
| MFS | (0.41 y) | 77 | 82.4% | 74.4% | 74.4% | ||
| NMD | (10.10 y) | 64 | 98.2% | 98.2% | 91.7% | ||
| All | 0–16 y (5.2 y) | 687 | 95.6% | 90.6% | 86.3% | UK study, Norrish et al. (6) | |
| Non-syndromic | 433 | 97.6% | 92.7% | 87.5% | |||
| RASopathy | 126 | 92.5% | 90.5% | 85.9% | |||
| IEM | 64 | 82.2% | 66.4% | 66.4% | |||
| FRDA | 59 | 100% | 97.1% | 97.1% | |||
| RCM | All | 0–18 y | 152 | – | – | – | PCMR (10) |
| Pure RCM | (6.1 y) | 101 | 48% | 22% | – | ||
| RCM/HCM | (6.3 y) | 51 | 65% | 43% | – | ||
| LVNC | All | 0–10 y (0.3 y) | 29 | 69% | 52% | 48% (15 y: 45%) |
NACCS (13) |
| All | 0–14 y (9.3 y) | 242 | – | – | – | USA study, Brescia et al. (57) | |
| Dilated | 46 | – | 63% | – | |||
| Hypertrophic | 66 | – | 86% | – | |||
| Mixed | 68 | – | 64% | – | |||
| Pure | 62 | – | 98% | – | |||
IEM, inborn errors of metabolism; MFS, malformation syndromes; NMD,neuromuscular disorders; FRDA, Friedreich's ataxia.
a
Numbers represent overall survival rates by aetiologies, transplantation status not further specified.