10
Treatment
Table 6. Screening With Electrocardiography and 2D
Echocardiography in Asymptomatic Family Members*
Age of First-Degree
Relative Initiation of Screening
Repeat
ECG, Echo
Pediatric
Children and adolescents
from genotype-positive
families, and families
with early onset disease
At the time HCM is diagnosed in
another family member
Every 1–2 y
All other children and
adolescents
At any time aer HCM is diagnosed in a
family member but no later than puberty
Every 2–3 y
Adults At the time HCM is diagnosed in
another family member
Every 3–5 y
* Includes all asymptomatic, phenotype-negative, first-degree relatives deemed to be at-risk
for developing HCM based on family history or genotype status and may sometimes include
more distant relatives based on clinical judgment. Screening interval may be modified (eg, at
onset of new symptoms or in families with a malignant clinical course or late-onset HCM).
ECG indicates electrocardiogram; Echo, echocardiogram; and HCM, hypertrophic
cardiomyopathy.
6.2. Echocardiography
COR LOE
Recommendations
1 B-NR
1. In patients with suspected HCM, a transthoracic
echocardiogram (TTE) is recommended in the initial
evaluation.
1
B-NR
(children)
2. In patients with HCM who have no change in clinical status
or events, repeat TTE is recommended every 1 to 2 years
to assess the degree of myocardial hypertrophy, dynamic
left ventricular outflow tract obstruction (LVOTO), mitral
regurgitation (MR), and myocardial function (Figure 1).
C-LD
(adults)
1 B-NR
3. For patients with HCM who experience a change in clinical
status or a new clinical event, repeat TTE is recommended.
1 B-NR
4. For patients with HCM and resting peak LVOT gradient <50
mm Hg, a TTE with provocative maneuvers is recommended.
1 B-NR
5. For symptomatic patients with HCM who do not have a
resting or provocable outflow tract peak gradient ≥50 mm Hg
on TTE, exercise TTE is recommended for the detection and
quantification of dynamic LVOTO.
