Gettysburg Medical News
The Clinical View
by P.E. Hoffsten, MD
21 March 2007 

CAN YOUR HEART BE TOO STRONG?

            On May 6, 1954, Sir Roger Banster, then a 25-year-old physician, became the first human being known to run a mile in less than four minutes.  His resting heart beat was only 32 beats per minute.  His heart was incredibly strong.  It could fill with a large amount of blood so each heart beat resulted in a huge amount of blood being pumped out to the body.  His heart was well conditioned, somewhat bigger than a normal heart.  We call his condition “athletic hypertrophy”.

Hyper- means larger than.  -Trophy means growth.  Hypertrophy of the heart means that the muscle has grown larger then usual.  This is an expectation in trained athletes.  When they stop training, the heart decreases in size more towards normal. 

            But there is a disease state that is very difficult to diagnosis in which the heart becomes hypertrophic without the individual doing any athletic training.  The gentleman below exemplifies this problem and how difficult it can be to deal with. 

            The gentleman was 72-years old and somewhat overweight.  He came to the clinic complaining that he was becoming short of breath when he took his walk.  He said that he use to be able to walk six blocks with no trouble at all.  He related that now by the time he got one block from home, he was so short of breath that he had to stop to rest.  He also developed chest pain.  The cause of concern was that he might be developing coronary artery disease that would cause a heart attack.  He was referred to the cardiologist.  A coronary angiogram was done to evaluate blocks that might be present in the arteries that supply blood to his heart.  There were two blockages noted and these were opened with stents.  It was thought that now all would be well. 

            But he came back to the clinic a short time later stating that he was still very short of breath.  He related that now his chest pain did not occur but that his ability to walk was not re-established.  He indicated that if he strained at all, such as trying to hoe in his garden, he would become very weak and short of breath.  He indicated that his garden had been overgrown with weeds because he just couldn’t take care of it. 

            It was at this point that I thought perhaps his lung function was impaired.  So his lungs were examined and found to be normal.  And then a test called an echocardiogram was done.  Echocardiograms evaluate how well the valves of the heart are working and can determine the thickness of the wall of the heart.  Ideally, the heart wall should not be thickened unless the person is a trained athlete.  This 72-year-old gentleman was not a trained athlete.  Yet his echocardiogram showed that there was a very abnormal thickening of the wall of the heart, near the top of the heart. This is where blood exits to go to the body.  Thick muscle at the top of the heart resulted in a strange situation whereby the top of his heart contracted too tightly.  The top of the heart contracting too tightly kept the muscle in the lower part of the heart from pumping blood out past the obstructing top of the heart.  This resulted in a very high pressure in the inside chamber of the heart and that pushed blood backwards into the lungs causing his shortness of breath.  In other words, the top of his heart was too strong and he had done absolutely nothing in an athletic endeavor to make this happen.  His disease is called hypertrophic cardiomyopathy.  Hypertrophic means the heart has grown larger.  Cardio- has to do with the heart.  Myo- means to do with muscle and –pathy means there is disease.  Thus hypertrophic cardiomyopathy means the muscle of the heart has grown too much and represents a disease state.  This is different than an “athletic heart”.

            Fortunately, this condition occurs very infrequently at the rate of only 2 for every 1,000 people.  When it does occur, it is very difficult to diagnosis because we are always expecting that an elderly gentleman with shortness of breath has a weak heart.  This man’s heart was too strong. 

            The cause of this condition is unknown.  It appears to have hereditary factors in about half of the cases.  Those with hereditary factors tend to get the condition earlier in life and often asymptomatically.  In these young people, the first symptom is sometimes the last symptom because they can develop an abnormal rhythm and die suddenly.  When the condition develops in older individuals such as this gentleman, hereditary factors do not appear to play a part and we don’t know why this condition occurs. 

            Now comes the part that is really bizarre.  If you can imagine, treating this condition requires medicines or surgery that weaken the heart.  A medication called verapamil is most commonly used for this condition.  It has the side effect of causing constipation and slowing the rate at which the heart beats.  When this drug was tried in this gentleman, his heart rate slowed to the 40’s and now he really felt horrible.  So that drug had to be stopped.  Another medication that might be used is called a beta blocker.  Fortunately, this medication won’t cause constipation but it can slow the heart rate.  A third medication called disopyramide has also been used with some success.  It has the advantage of not slowing the heart rate but it does weaken the heart muscles slightly.  The cardiologists are still struggling with how to treat this gentleman.

            This condition is relatively unusual and it flies in the face of what healthcare providers try to do for elderly individuals who are short of breath.  We usually think that hearts are weaker and that we have to give them less work to do and/or strengthen the heart.  Also, swelling of the ankles is a common accompaniment of elderly short-of-breath individuals so we use diuretics to decrease the back pressure in the lungs.  This is the exact opposite of what should be done with a person who has hypertrophic cardiomyopathy.  Trying to deal with this condition is a humbling experience for healthcare providers. 

            The healthcare providers at your local clinics do the best they can to help deal with heart problems.  Ninety nine percent of the time, we are dealing with weak hearts that need less work to do and fluid removal.  But in this gentleman that just didn’t work.  Unfortunately, health care providers are not always right in what they believe to be the case.   A basic rule in life or medicine is, “When what you are doing doesn’t work, do something else.  But don’t keep doing the same thing because you have already shown that that doesn’t work.”  Obeying this rule requires time to stop and think and re-evaluate situations.  The healthcare providers at your local clinics are faced with this problem very frequently and it is always a struggle that we work to overcome.