Htpertrophic Obstructive Cardiomyopathy

  My 8 year old son has severe HOCM. He underwent a septal myectomy 2 1/2 years ago with good results, his LV gradient dropped from 73mmHg to 13mmHg. He has only been seen twice in the last two years and his last echo was in May of 1997 with the following results: LV gradient-13mmHg, EF-76%, RV systolic pressure-31mmHG, septal thickness-21mm, Lv mass index 187 g/m2,  mild mitral and trivial tricuspid and pulmanary regurgitation, pulmanary artery end diastolic pressure-15mmHg, left coronary artery to left ventricule fistula. X-Ray showed an unusually shaped and enlarged heart. He has been having the following problems: episodes of extreme shortness of breath, fever and very lethargic, tiring easily with play, very hot and sweaty even in cool atmosphere and drop in appetite. He saw the pediatric cardiologist this past week who listened to his heart and said everything sounds fine and told us to return in a year. No echo, no ekg, no x-ray! Do you feel these symptoms warrant further investigation/second opinion with the mentioned tests?  Thank you.

[CCF CARDIO MD APS]

Dear Dolly,
Anytime there are symptoms such as you describe in your son, an evaluation is indicated.  If you do not feel that a physician is addressing your concerns, that is to say if you ever feel like your questions are not being answered, you should seek a second opinion,  
I can comment as follows based on all the information you gave me regarding your son's evaluation in May of 1997.  One important point is whether or not the left coronary to left ventricle fistula was repaired or not.  You son's symptoms sound serious and warrant an investigation such as a stress test which in the form of a stress echo would give the physician lots of information.
For example, the echo portion would reassess the gradient under a stress situation (although a gradient at rest is normal this does not mean the gradient does not rise to significant level with stress/exercise), and the stress ecg portion of the test would evaluate the fistula for steal phenomenon (the ecg portion looks for ischemia-the heart starving for oxygen because the blood flow in the left coronary is compromised from the fistula drawing blood into the left ventricle.
These are just a few possible explanations for your son's symptoms.  But even had the fistula been fixed there still is the question of an abnormal gradient with exercise, just in case you are wondering this would not necessarily mean more surgery, right now you need to concentrate on finding a physician who would investigate these symptoms.  
If you would like to have your son seen at the Cleveland Clinic Foundation just call 1-800-CCF-CARE for an appointment with one of our pediatric cardiologists.  Information providedin the heart forum is intended for general informational purposes only, any diagnosis or treatment can only be made by your physician(s).