3
4. Assessing a patient's risk for sudden cardiac death is an important
component of management. Integrating the presence or absence of
established risk markers with tools to estimate individual risk score
will facilitate the patient's ability to participate in decision-making
regarding implantable cardioverter-defibrillator placement. These
discussions should incorporate a patient's personal level of risk
tolerance and their specific treatment goals.
5. The risk factors for sudden cardiac death in children with HCM
carry different weights and components than those used in adult
patients. Pediatric risk stratification also varies with age and must
account for different body sizes. Coupled with the complexity of
placing implantable cardioverter-defibrillators in young patients
with anticipated growth and a higher risk of device complications,
the threshold for implantable cardioverter-defibrillator implantation
in children often differs from adults. These differences are best
addressed at comprehensive HCM centers with expertise in caring
for children with HCM. New risk calculators, specific to children and
adolescents, have been validated and can help young patients and
their families contextualize their estimated risk of sudden cardiac
death.
6. Cardiac myosin inhibitors are now available to treat patients with
symptomatic obstructive HCM. This new class of medication inhibits
actin-myosin interaction, thus decreasing cardiac contractility and
reducing left ventricular outflow tract obstruction. Mavacamten
is currently the only U.S. Food and Drug Administration-approved
agent. These agents can be beneficial for patients with obstructive
HCM who do not derive adequate symptomatic relief from first-line
drug therapy.
7. Invasive septal reduction therapies (surgical septal myectomy and
alcohol septal ablation), when performed by experienced HCM teams
at dedicated centers, can provide safe and effective symptomatic
relief for patients with drug-refractory or severe outflow tract
obstruction. Given the data on the significantly improved outcomes
at comprehensive HCM centers, these decisions represent an
optimal opportunity for referral.
Top 10 Take-Home Messages (cont'd)
