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8.3. Management of Patients With HCM and Advanced HF
COR LOE
Recommendations
1 C-LD
1. In patients with HCM who develop systolic dysfunction
with an LVEF <50%, GDMT for HF with reduced EF is
recommended.
1 C-LD
2. In patients with HCM and systolic dysfunction, diagnostic
testing to assess for concomitant causes of systolic
dysfunction (eg, CAD) is recommended.
1 B-NR
3. In patients with nonobstructive HCM and advanced HF
(NYHA functional class III to class IV despite GDMT),
CPET should be performed to quantify the degree of
functional limitation and aid in selection of patients for heart
transplantation or mechanical circulatory support.
1 B-NR
4. In patients with nonobstructive HCM and advanced HF
(NYHA class III to class IV despite GDMT) or with life-
threatening ventricular arrhythmias refractory to maximal
GDMT, assessment for heart transplantation in accordance
with current listing criteria is recommended.
1 B-R
5. In patients with HCM who develop persistent systolic
dysfunction (LVEF <50%), cardiac myosin inhibitors should
be discontinued.
2a C-EO
6. For patients with HCM who develop systolic dysfunction
(LVEF <50%), it is reasonable to discontinue previously
indicated negative inotropic agents (specifically, verapamil,
diltiazem, or disopyramide).
2a B-NR
7. In patients with nonobstructive HCM and advanced HF
(NYHA functional class III to class IV despite GDMT)
who are candidates for heart transplantation, continuous-
flow LVAD therapy is reasonable as a bridge to heart
transplantation.
2a C-LD
8. In patients with HCM and persistent LVEF <50%, ICD
placement can be beneficial.
2a C-LD
9. In patients with HCM and LVEF <50%, NYHA functional
class II to class IV symptoms despite GDMT, and LBBB,
CRT can be beneficial to improve symptoms.