Borderline Heart Function

[terriboyles]

My 10 year old granson was diagnosed with IHSS/hypertrophic cardiomyopathy when he was 6 weeks old.  At about two and a half months the obstruction was removed and a small patch was placed. He has had very few problems.

This year when he had his cardiologist visit the Dr. said he had an extra beat.  They did holter monitoring and there were not many incidents of the "extra beat."  This was followed by a cardiac cath and then a cardiac MRI.

His cardiologist says the MRI shows, his words, "borderline heart function" and wants to present his case to a panel to see if they recommend an implanted defribrilator.  Is "borderline heart function" a medical term and what does it mean?  He says there could be several reasons for this, including his original surgery.

Also, does this disease progress as children enter puberty?  He has been realatively symptom free until now.

Thank you!

[Jeffrey R Boris, M.D.]

Dear Terri,

Borderline heart function means that the squeeze of the heart is just at the lower limits of normal.  Hypertrophic cardiomyopathy (HCM) is a genetic disease that is occurs when the heart muscle cells grow abnormally.  The walls of the heart become significantly thickened, and can even obstruct blood flow out of the heart.  Sometimes, that obstruction can be enough to decrease adequate blood flow ejection out of the heart which, over time, can decrease blood flow to the coronary arteries.  This, in combination with the amount of work the heart has to do, can cause scar tissue to form in the heart muscle.  The scar tissue can lead to decreased ability of the heart to squeeze as well as lead to a risk of arrhythmias.  As well, the genetic abnormality associated with HCM can also cause abnormal function of the heart muscle cells.  In some cases of HCM, we do see what we refer to as a “burned-out” phase, in which the heart, which used to squeeze exceedingly well, now squeezes poorly.  The left ventricle, which is the pumping chamber on the left, can dilate and can also increase the risk for arrhythmias.

I cannot say for sure what the reason for your grandson’s change in his function is without reviewing his information and evaluating him directly.  It is reassuring that he did not have many extra beats.  However, an implantable cardioverter-defibrillator is a special pacemaker-like device that is sometimes used when patients are at risk for life-threatening arrhythmias.  It is able to detect these arrhythmias and, if they are persistent, deliver a shock to the heart to electrically reset the heart and stop the arrhythmia.  This can help in the short term, but if the function of the heart continues to decrease, he may require a heart transplant.  As you suggested, this can progress during puberty, where this is rapid growth and hormonal changes that can affect the body and the heart’s need to keep up.  His parents may end up seeing increased symptoms as he grows.  It may be helpful to have genetic testing performed, as this may be able to help with not only prognosis but also diagnosis of other family members.  At minimum, all first degree relatives (parents, siblings) should be screened for HCM.

[grendslori]

My daughter was diagnosed with HCM when she was 6 years old. We were told by her doctors at Texas Children's Hospital in Houston that this disease can get worse during puberty due to the rapid growth period. They told us if she survived her childhood that she would be in failure by the time she reached her teen years and they were absolutely right. She hit 12 and developed heart failure. She had a transplant at 22. You have to understand that she had a VERY serious case of this disease, one of the worst in the country according to the NIH; all of her ventricular walls and septal walls were 4cm. thick and she had a slew of electrical problems as well. (WPW, SSS, A-Fib, NSVT and a Maheim Fiber among other problems) so her case got to be very intense and complicated. This may not be the same for your grandson. Many children live fairly normal lives even with this disease, I think I heard once that only 5% of HCM patients require transplants. The odds are in your grandson's favor. The family should have all been evaluated for this disease as it is most often genetic in nature although sometimes it can be sporadic, as it was in my daughter's case. I hope they are also keeping tabs on his electrical system as well so he doesn't have electrical problems that have not been discovered. he should be evaluated by a good team of doctors who tend to see this problem in children, who understand it and in turn, who know how to treat it. take care