28
Treatment
8. Management of HCM
8.1. Management of Symptomatic Patients With Obstructive HCM
8.1.1. Pharmacological Management of Symptomatic
Patients With Obstructive HCM
COR LOE
Recommendations
1 B-NR
1. In patients with obstructive HCM and symptoms* attributable
to LVOTO, nonvasodilating beta blockers, titrated to
effectiveness or maximally tolerated doses, are recommended.
1
B-NR
†
2. In patients with obstructive HCM and symptoms*
attributable to LVOTO, for whom beta blockers
are ineffective or not tolerated, substitution with
nondihydropyridine calcium channel blockers (eg,
verapamil,
†
diltiazem
‡
) is recommended.
C-LD
‡
1 B-R
3. For patients with obstructive HCM who have persistent
symptoms* attributable to LVOTO despite beta blockers
or nondihydropyridine calcium channel blockers, adding a
myosin inhibitor (adult patients only), or disopyramide (in
combination with an atrioventricular nodal blocking agent),
or SRT performed at experienced centers,
§
is recommended.
1 C-LD
4. For patients with obstructive HCM and acute hypotension
who do not respond to fluid administration, intravenous
phenylephrine (or other vasoconstrictors without inotropic
activity), alone or in combination with beta-blocking drugs,
is recommended.
2b C-EO
5. For patients with obstructive HCM and persistent dyspnea
with clinical evidence of volume overload and high left-sided
filling pressures despite other HCM GDMT, cautious use of
low-dose oral diuretics may be considered.
2b C-EO
6. For patients with obstructive HCM, discontinuation of
vasodilators (eg, angiotensin-converting enzyme inhibitors,
angiotensin receptor blockers, dihydropyridine calcium
channel blockers) or digoxin may be reasonable because these
agents can worsen symptoms caused by dynamic outflow tract
obstruction.
3: Harm C-LD
7. For patients with obstructive HCM and severe dyspnea at
rest, hypotension, very high resting gradients (eg, >100 mm
Hg ), as well as all children <6 weeks of age, verapamil is
potentially harmful.
* Symptoms include effort-related dyspnea or chest pain and occasionally other exertional
symptoms (eg, syncope, near syncope) that are attributed to LVOTO and interfere with
everyday activity or quality of life.
†
Symbol corresponds to the Level of Evidence for verapamil.
‡
Symbol corresponds to the Level of Evidence for diltiazem.
§
Comprehensive or primary HCM centers with demonstrated excellence in clinical outcomes
for these procedures (Table 3, Table 4).
