Table 3.
Outcomes
| Total | NS | NSML | CS | CFCS | Noonan‐like | P value | |
|---|---|---|---|---|---|---|---|
| Death, n (%) | 21 (14.1) | 13 (11.7) | 1 (8.3) | 1 (16.7) | 1 (16.7) | 3 (27.3) | 0.083 |
| SCD, n (%) | 1 (4.8) | 1 (7.7) | — | — | — | — | |
| CCF, n (%) | 2 (9.5) | 1 (7.7) | 1 (8.3) | — | — | — | |
| Other CVS, n (%) | 1 (4.8) | 1 (7.7) | — | — | — | — | |
| Other, n (%) | 4 (19.1) | 2 (15.4) | — | 1 (100) | — | 1 (33.3) | |
| Unknown, n (%) | 12 (57.1) | 7 (53.9) | — | — | 1 (100) | 2 (66.7) | |
| Age at death (months), median (25–75th centile) | 24.1 (5.6–175.9) | 25.9 (5.6–175.9) | 1.7 | 12.9 | 191.1 | 23.1 (12–73.8) | 0.469 |
| Myectomy, n (%) | 14 (9.4) | 13 (11.7) | 1 (8.3) | — | — | — | 0.886 |
| ICD implantation, n (%) | 7 (4.7) | 7 (6.3) | — | — | — | — | 0.653 |
| CCF admission, n (%) | 10 (6.7) | 9 (8.1) | 1 (8.3) | — | — | — | 0.715 |
| Heart transplant, n (%) | 3 (2) | 3 (2.7) | — | — | — | — | 0.909 |
| NSVT, n (%) | 5 (3.4) | 3 (3) | 1 (8.3) | — | — | 1 (33.3) | 0.674 |
| SCD or equivalent event, n (%) | 12 (8.1) | 9 (8.1) | 1 (8.3) | — | — | 2 (18.2) | 0.775 |
CCF, congestive cardiac failure; CFCS, cardiofaciocutaneous syndrome; CS, Costello syndrome; CVS, cardiovascular; ICD, implantable cardiac defibrillator; n, number of patients; NS, Noonan syndrome; NSML, Noonan syndrome with multiple lentigines; NSVT, non‐sustained ventricular tachycardia; SCD, sudden cardiac death.Bold numbers represent statistically‐significant p values