Figure 14. Survival rates for children with hypertrophic cardiomyopathy with and without Noonan syndrome, and by risk factors at diagnosis for those with Noonan syndrome.

(A) Estimated survival since diagnosis of HCM in children with (n = 74) and without (n = 792) NS; log-rank p = 0.03. The size of the risk set is shown below the x-axis. (B) Survival by left ventricular FS Z-score in children with NS and HCM. Estimated survival since diagnosis of HCM in 48 children with NS by LV FS Z-score at the time of HCM diagnosis (<6.35 vs ≥6.35 to where 6.35 is the median). Log-rank p = 0.02. The 5-year survival is 59% for children with a Z-score <6.35 and 90% for children with a Z-score ≥6.35. The size of the risk set is shown below the x-axis. (C) Survival by age and CHF in children with NS and HCM. Estimated survival since diagnosis of HCM in 74 children with NS by age and CHF status at the time of HCM diagnosis (log-rank p < 0.001). The size of the risk set is shown below the x-axis. The subgroup of three cases with CHF who were diagnosed at age ≥6 months is not shown (one known to survive 5.5 months postdiagnosis and two were not seen after diagnosis).

CHF: Congestive heart failure; CM: Cardiomyopathy; FS: Fractional shortening of left ventricle; HCM: Hypertrophic cardiomyopathy; NS: Noonan syndrome.

Reproduced with permission from [41].