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Heartline Magazine April - June 2004
What is Hypertropic Cardiomyopathy?
Cardiomyopathy is a condition in which the muscle of the heart is abnormal
in the absence of an apparent cause. There are three types of cardiomyopathy: "hypertrophic",
"dilated" and "restrictive". The main feature of hypertrophic cardiomyopathy is
an excessive thickening of the heart muscle (hypertrophy literally means to
thicken). Heart muscle may also thicken in normal individuals as a result of
high blood pressure or prolonged athletic training. There is a fine line between
and athletic heart and a heart with HCM.
What's in a name?
Remarkably, this disease (hypertrophic cardiomyopathy) has been given 75
separate names by individual investigators over the last 40 years. Literally, no
other disease can make that claim. The principal reason for the proliferation of
names has undoubtedly been the heterogeneity and diversity in which the disease
is expressed. These names include IHSS (or idiopathic hypertrophic subaortic
stenosis), which was the firstpopular term used in the United States; "stenosis"
means obstruction. The same can be said for HOCM (hypertrophic obstructive
cardiomyopathy), which is still used in the United Kingdom, largely out of habit
and convenience.
Nevertheless, virtually all HCM experts and other cardiovascular specialists
now regard as the best single name for this broad disease spectrum -
hypertrophic cardiomyopathy or HCM. This term emphasizes the hypertrophy, which
is the diagnostic marker in most patients and the fact that this disease is a
form of cardiomyopathy - or heart muscle disorder; without mentioning
obstruction. Therefore, it is preferable to describe the disease as either "HCM
with obstruction" or "HCM without obstruction."
The Heart in Hypertrophic Cardiomyopathy
The major abnormality of the heart in Hypertrophic Cardiomyopathy is an
excessive thickening of the muscle. The distribution of muscle thickening or
hypertrophy is variable. The left ventricle is almost always affected and in
some patients the muscle of the right ventricle also thickens.
Complications
In a minority of cases a number of specific complications can occur:
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Arrhythmias: Irregularities of the
heartbeat are a common complication.
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Endocarditis: This is an infection of
the heart, which occurs rarely in Hypertrophic Cardiomyopathy. Heart Block:
The normal electrical signal may travel down to the ventricles slowly or may
even be completely blocked.
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Sudden Death: Overall, in patients with
Hypertrophic Cardiomyopathy there is an increased risk of premature death,
which can occur with little or no warning. Sudden death can strike at any age.
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"Burnt Out" or "End Stage" HCM: The heart
reaches a point of generalized weakening and thinning of the muscle walls with
dilated chambers, particularly the left ventricle. The weakening and the
dilation of the heart muscle eventually lead to heart failure.
Is a cure available?
At present there is no cure for Hypertrophic Cardiomyopathy, HCM. There is a
slight possibility that some drugs may decrease the degree of muscle thickening.
However, no treatment has been shown to return the heart to normal. Research
continues in this area. Developments are most likely to come from the early
detection of persons carrying the gene for Hypertrophic Cardiomyopathy and
treating them to prevent the development of hypertrophy.
The achievement of this goal will require much further research and is many
years away from reality.
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