Table 6.

Predictors of SCD or equivalent event

	Hazard ratio	Standard error	95% CI	P value
Demographics and baseline clinical characteristics
Gender	1.47	0.89	0.45–4.84	0.522
Age at diagnosis	1	0.01	0.99–1.02	0.556
Age at baseline assessment	0.99	0.01	0.98–1.01	0.506
Proband	0.57	0.60	0.07–4.5	0.598
FHx HCM	0.63	0.66	0.08–4.93	0.659
PMHx CHD	1.65	1.11	0.44–6.15	0.457
PMHx CCF	0.34	0.23	0.09–1.26	0.096
PMHx arrhythmia	6.42E + 14	2.30E + 22	—	1.000
Symptoms	1.53	0.96	0.45–5.25	0.497
Medications	0.48	0.30	0.14–1.64	0.243
CCF admission	1.75	1.83	0.22–13.68	0.596
NSVT	6.1	4.84	1.28–28.91	0.023
Syndrome				0.514
NSML	1.11	1.18	0.14–8.88	0.921
CS	5.08E − 16	3.88E − 08	—	1.000
CFCS	5.09E − 16	3.45E − 08	—	1.000
Noonan‐like	3.07	2.45	0.64–14.6	0.159
Gene	1.24	1.04	0.24–6.41	0.82
Gene negative	1.81	1.22	0.49–6.75	0.376
Echocardiographic phenotype
LVEDD	0.87	0.80	0.74–1.04	0.126
LVEDD z score	0.64	0.17	0.38–1.08	0.106
LA diameter	0.96	0.08	0.81–1.14	0.657
LA diameter z score	0.99	0.09	0.82–1.19	0.893
MLVWT	1.00	0.07	0.88–1.15	0.944
MLVWT z score	1.01	0.03	0.95–1.08	0.783
LVOT gradient	1.02	0.01	1–1.04	0.031
RVOT gradient	1.02	0.02	0.99–1.06	0.186
Ejection fraction	1.03	0.09	0.86–1.24	0.726
Average E/E′	5.09E − 16	3.45E − 08	—	1.000
RVH	0.43	0.37	0.08–2.36	0.332
Mid‐cavity obstruction	0.70	0.54	0.16–3.16	0.647
SAM	0.67	0.51	0.15–2.97	0.597

CCF, congestive cardiac failure; CFCS, cardiofaciocutaneous syndrome; CHD, congenital heart defect; CI, confidence interval; CS, Costello syndrome; FHx, family history; HCM, hypertrophic cardiomyopathy; LA, left atrial; LVEDD, left ventricular end‐diastolic diameter; LVOT, left ventricular outflow tract; MLVWT, maximal left ventricular wall thickness; NSML, Noonan syndrome with multiple lentigines; NSVT, non‐sustained ventricular tachycardia; PMHx, past medical history; RVH, right ventricular hypertrophy; RVOT, right ventricular outflow tract; SAM, systolic anterior motion of the mitral valve; SCD, sudden cardiac death.Bold numbers represent statistically‐significant p values