23
7.1.2. SCD Risk Assessment in Children and Adolescents
With HCM
COR LOE
Recommendations
1 B-NR
1. For children and adolescents with HCM, a comprehensive,
systematic noninvasive SCD risk assessment at initial
evaluation and every 1 to 2 years thereafter is recommended
and should include evaluation of these risk factors (Figure 1,
Figure 3, Table 7):
a. Personal history of cardiac arrest or sustained ventricular
arrhythmias;
b. Personal history of syncope suspected by clinical history to
be arrhythmic;
c. Family history in close relative of premature HCM-related
sudden death, cardiac arrest, or sustained ventricular
arrhythmias;
d. Maximal LV wall thickness, EF, LV apical aneurysm;
e. NSVT episodes on continuous ambulatory
electrocardiographic monitoring.
1 C-LD
2. For children and adolescents with HCM who have a
borderline risk for SCD, or in whom a decision to proceed
with ICD placement remains uncertain after clinical
assessment that includes personal and family history,
echocardiography, and ambulatory electrocardiographic
monitoring, CMR imaging is beneficial to assess for extent of
myocardial fibrosis with LGE (Table 7).
2a B-NR
3. For patients <16 years of age with HCM, it is reasonable
to calculate an estimated 5-year sudden death risk that
includes echocardiographic parameters (interventricular
septal thickness in diastole, LV posterior wall thickness in
end-diastole, left atrial diameter, maximal LVOT gradient)
and genotype, which may be useful during shared decision-
making for ICD placement (Table 7).
