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Back to Cardiovascular Diseases
Hypertrophic
Cardiomyopathy
Left Ventricular Outflow Tract Obstruction (LVOTO)
Hypertrophic cardiomyopathy (HCM) is a condition in which there is
asymmetric hypertrophy of the myocardium. The
thickened heart muscle can obstruct blood flow and prevent the
heart from functioning properly.
Causes
HCM is due to a genetic disorder caused by
various mutations in genes encoding sarcomeric proteins. It is an
inherited disease in 50% of the cases (autosomal dominant); however, 50%
of the cases are sporadic.
Symptoms and signs
Hypertrophic cardiomyopathy may present at any age. Patient may present
with angina,
dyspnea, syncope or sudden death (due to ventricular
fibrillation). Symptoms can become exacerbated by exertion. On auscultation there will be a harsh ejection systolic
murmur, S4.
Diagnosis
1. EKG will show exaggerated septal
Q waves suggestive of MI, as well as, evidence of left ventricular hypertrophy,
ischemia in
the inferior and lateral leads, arrhythmias (atrial fibrillation,
ectopics, ventricular tachycardia or fatal ventricular fibrillation).
2. Echocardiography will reveal asymmetrical septal hypertrophy, small left
ventricular cavity with dynamic obstruction due to valve motion abnormalities
(anterior mitral valve).
3. Cardiac
catheterization may help assess the severity of the gradient.
Cardiac
catheterization carries the risk of inducing ventricular tachycardia.
Treatment
1. Beta-blockers
Beta-blockers is the drug
of choice for relief of symptoms.
2. Calcium channel blockers
Verapamil can also help decrease the symptoms.
3. Treatment of arrhythmias
4. Septal myomectomy
Surgical or chemical septal myomectomy may be used
for those with severe symptoms.
5. Percutaneous transcoronary septal reduction
Percutaneous transcoronary septal reduction with alcohol may be possible
in some patients.
6. Dual chamber pacing
7. Implantable cardiac defibrillators
Preventing sudden cardiac arrest may be possible with the use of
implantable cardiac defibrillators
and amiodarone.
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