Table 5.

Factors associated with early onset hypertrophic cardiomyopathy and major cardiac event (n = 524)

	Univariable HR (95% CI)	P-value	Multivariable HR (95% CI)	P-value
Early phenotype-positivity
Male gender (ref = female)	2.048 (1.099–3.816)	0.024	2.028 (1.081–3.807)	0.028
Family history of SCD	2.618 (1.474–4.650)	0.001	1.935 (1.071–3.497)	0.029
Family history of SCD before age 25 years	1.615 (0.756–3.452)	0.22
First-degree phenotype or genotype-positive relative	1.695 (0.527–5.456)	0.38
Family history of ICD	2.842 (1.612–5.010)	<0.001	2.212 (1.218–4.019)	0.009
Family history of transplant	1.240 (0.385–43.990)	0.72
Symptomatic at screening	4.130 (1.002–17.026)	0.050	—	—
P/LP MYH7 variant	2.995 (1.526–5.877)	0.001	3.247 (1.555–6.782)	0.002
P/LP MYBPC3 variant	2.451 (1.331–4.513)	0.004	2.319 (1.173–4.587)	0.016
P/LP variant in other gene	0.385 (0.053–2.787)	0.34	—	—
P/LP variants in more than one gene	3.234 (0.446–23.445)	0.25	—	—
MaCE a
Male gender (ref = female)	1.176 (0.448–3.090)	0.74	—	—
Family history of SCD	3.516 (1.299–9.513)	0.013	1.177 (0.325–4.260)	0.80
Family history of SCD before age 25 years	5.829 (2.217–15.326)	<0.001	2.621 (0.687–9.990)	0.158
Family history of ICD	3.497 (1.347–9.076)	0.010	2.869 (0.903, 9.119)	0.074
Family history of transplant	1.195 (0.158–9.018)	0.86
Symptomatic at screening	15.400 (4.408–53.805)	<0.001	57.611 (9.549–347.595)	<0.001
P/LP MYH7 variant	6.068 (2.234–16.481)	<0.001	18.574 (4.183–82.475)	<0.001
P/LP MYBPC3 variant	2.884 (1.06–7.802)	0.037	9.340 (2.123–41.190)	0.003
P/LP variant in other gene	1.126 (0.149–8.491)	0.91	—	—

Bold values significant on multivariable analysis.

CI, confidence interval; HR, hazard ratio; ICD, implantable cardioverter-defibrillator; LP, likely pathogenic; MaCE, major cardiac event; P, Pathogenic; SCD, sudden cardiac death.

^aFirst degree affected relative and presence of multiple P/LP variants were not included in the models for MaCE because HR was unestimable as the events did not occur in one of the two groups.