Treatment cannot begin until we have a correct diagnosis.
Diagnosis is usually made with echocardiography, an ultrasound
examination of the heart. Echocardiography is a non-invasive,
non-Xray, imaging technique that uses sound waves to picture the
heart. Occasionally MRI (magnetic resonance imaging) of the
heart is ordered for clarification of ambiguities. After
diagnosis, each patient's treatment plan is based on the
severity of symptoms, diagnosis of obstructive or
non-obstructive HCM and results of further evaluation. Since
sudden death is a potentially catastrophic complication in
patients with HCM, we perform further tests to assess risk.
Patients without symptoms who are determined to be at low risk
of developing complications or death often require no treatment.
Most patients with symptoms can be treated successfully with
medications:
Treatment of obstructive HCM: Patients with
obstruction usually are first begun on beta-blocker medication.
If this does not help, other medications, disopyramide or
verapamil often are successful at relieving obstruction and
symptoms.
Medical
treatment of hypertrophic cardiomyopathy.
Refractory symptoms: Patients with outflow
tract obstruction and symptoms that do not respond to medication
generally benefit from surgery-extended myectomy.
Surgery has been performed for more than 30 years
and is generally considered the gold-standard for relief of
obstruction in medically refractory patients. Non-surgical
interventions to relieve obstruction are available as well.
Alcohol ablation of the septum is a non-surgical
procedure to improve outflow tract obstruction. It is a
percutaneous catheter-based method to decrease septal thickness
by therapeutic myocardial infarction. Older patients or those
with significant medical conditions who are not good candidates
for surgery or ablation may benefit from a dual chamber
pacemaker implantation.
Treatment of non-obstructive HCM: Symptoms of
shortness of breath, chest pain and blackouts can also occur in
patients with no obstruction. These symptoms can generally be
treated with medication as well. For patients with
non-obstructive hypertrophic cardiomyopathy, verapamil is often
the best choice because of its efficacy in relieving myocardial
ischemia.
Medical treatment of non-obstructive hypertrophic cardiomyopathy.
The usual surgical
procedure for HCM (myectomy that relieves obstruction) is not of
help for these patients, who are then, generally treated
medically. Alcohol ablation has no role in the treatment of
non-obstructive HCM. Very, very rarely, patients with
progressive HCM require heart transplantation.
Those patients at risk for sudden death because of the
presence of risk factors may be treated with medication or an
implanted automatic defibrillator, a device that
shocks the heart back to normal rhythm if a life-threatening
irregular heart rhythm occurs.
Treatment is often a multidisciplinary approach. We coordinate
the efforts an echocardiographer, electrophysiologist, pacemaker
specialist, cardiac surgeon or interventional cardiologist for
rapid and thorough evaluation and treatment.
To
see research abstracts on HCM, click here. |
