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What is hypertrophic cardiomyopathy?
The term cardiomyopathy is purely descriptive, meaning disease of the heart muscle. There are four main
types of cardiomyopathy: hypertrophic (HCM), dilated (DCM), restrictive (RCM) and arrhythmogenic right
ventricular (ARVC).
The main feature of HCM is an excessive thickening of the heart muscle (hypertrophy literally means to
thicken) in the absence of an apparent cause. Heart muscle may thicken in normal individuals as a result of
high blood pressure or prolonged athletic training. In HCM, however, the muscle thickening occurs without
obvious cause.
In addition, microscopic examination of the heart muscle shows that it is abnormal. The normal alignment of
muscle cells is absent and this abnormality is called myocardial disarray.
The term cardiomyopathy is purely descriptive, meaning disease of the heart muscle. There are four main
types of cardiomyopathy: hypertrophic (HCM), dilated (DCM), restrictive (RCM) and arrhythmogenic right
ventricular (ARVC).
The main feature of HCM is an excessive thickening of the heart muscle (hypertrophy literally means to
thicken) in the absence of an apparent cause. Heart muscle may thicken in normal individuals as a result of
high blood pressure or prolonged athletic training. In HCM, however, the muscle thickening occurs without
obvious cause.
In addition, microscopic examination of the heart muscle shows that it is abnormal. The normal alignment of
muscle cells is absent and this abnormality is called myocardial disarray.
Heart transplants
A heart transplant is necessary for a few individuals who have severe impairment of the pumping action of
the heart.
A heart transplant is necessary for a few individuals who have severe impairment of the pumping action of
the heart.
Hypertrophic Cardiomyopathy defined.
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Dominic is currently taking a beta-blocker to slightly slow his heart rate. This will theoretically reduce the
stress on the heart muscle which should help decrease or limit the uneven growth. Before we visited the
Hospital in Pittsburgh, we understood that in some cases this could help the body to "adjust" itself by
allowing the rest of the heart around the affected portion to essentially "catch up".
The other option that was discussed with us was a myectomy (surgery to effectively shave away the excess
muscle). Until now, we had considered that the worst case scenario, transplant seemed so foreign to us...
The choice, however, was not ours to exercise. The severity of Dominic's condition puts him into the 97th
percentile. We were told to expect a transplant within the next few years.
stress on the heart muscle which should help decrease or limit the uneven growth. Before we visited the
Hospital in Pittsburgh, we understood that in some cases this could help the body to "adjust" itself by
allowing the rest of the heart around the affected portion to essentially "catch up".
The other option that was discussed with us was a myectomy (surgery to effectively shave away the excess
muscle). Until now, we had considered that the worst case scenario, transplant seemed so foreign to us...
The choice, however, was not ours to exercise. The severity of Dominic's condition puts him into the 97th
percentile. We were told to expect a transplant within the next few years.
By following the arrows you can trace the flow of
blood through the heart chambers:
1. in to the right atrium
2. through tricuspid valve into the right ventricle
3. through the pulmonary valve toward the lungs to
be oxygenated.
4. oxygenated blood returns to the heart through
the left atrium.
5. through the mitral valve into the left ventricle
6. from there it exits the heart through the aorta with
enough force to shoot dozens of feet into the air.
blood through the heart chambers:
1. in to the right atrium
2. through tricuspid valve into the right ventricle
3. through the pulmonary valve toward the lungs to
be oxygenated.
4. oxygenated blood returns to the heart through
the left atrium.
5. through the mitral valve into the left ventricle
6. from there it exits the heart through the aorta with
enough force to shoot dozens of feet into the air.
- the gray area indicates the area of the heart muscle that is
typically affected in HCM.
This diagram illustrates the affect that HCM has on the
heart; specifically Asymmetric septal hypertrophy with
obstruction. The circle indicates the area where the
enlarged septum can limit the ability of the mitral valve to
function properly.
heart; specifically Asymmetric septal hypertrophy with
obstruction. The circle indicates the area where the
enlarged septum can limit the ability of the mitral valve to
function properly.