10 yr old with Aorta Stenosis

  My 10 year old niece, Samantha, was diagnosed with Aorta Stenosis.  Her
  valve is 50% closed but her doctors do not want to operate until it is
  75% closed.  
  For the past 2 years she's been having mild seizures every once in a while.
  Lately, her seizures have become much stronger and occur much more
  frequently (about every 2 days). We are terrified.  
  The doctor can not confirm that the seizures are caused by her heart
  problem.
  Also, she has developed spots, like bruises, all over the left side
  of her body (from neck to toes).  The spots are darker brown than her skin
  and do not hurt.  The doctors have no idea what has caused this.
  Can anyone shed some light??  We are scared and do not know whether to
  trust these doctors who can not diagnose the problems.  Please help.
  My work number is: 1-800-225-5693 ask the operator for extension 6783.

[CCF Cardio MD - MTR]

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Dear Veronica, thank you for your question.  From the information you've provided, I do not believe that the seizures are related to the aortic stenosis.  They may be separate, but concurrent problems.  As for the seizures, your niece should see a pediatric neurologist who could determine the cause and proper treatment for this problem.  Certainly, the seizures should be fully investigated before considering aortic valve replacement.  From what you describe, I can't determine what's causing the new lesions (spots) on her skin.  Perhaps she should see a dermatologist who could perform a biopsy of one of the lesions to diagnose the cause.  Again, I know of no association between aortic stenosis and the skin lesions you describe.  Finally, the decision to replace an aortic valve in a child is individual-specific and is determined by the degree of valvular obstruction, the age of the child, and the symptoms.  The common techniques to replace an aortic valve include a mechanical valve prosthesis, an aortic valve homograft, or the Ross Procedure.    Mechanical valves are very durable, but would not "grow" with her as she ages and require life-long anticoagulation (blood thinners).   Aortic valve homografts are obtained from cadavers and do not require anticoagulation, but would be expected to degenerate over 20-25 years and would then need to be replaced.   The Ross Procedure is performed by highly experienced surgeons in selected centers.  During this procedure, the pulmonic valve is moved to the aortic position and the pulmonic valve is replaced by a homograft. The advantage to this surgery is that the "new" aortic valve would be expected to grow with the child since it's composed of native tissue.  The pulmonic valve homograft would not need to grow since the pressures on this side of the heart are low to begin with so the pulmonic valve would not need to enlarge as the child grows.  Generally, valve replacement is delayed as long as possible to allow the child to grow, but specifics about that decision need to be elucidated by her cardiologist.  We have a number of excellent pediatric cardiologists here who would be happy to see your niece, if your family would like to bring her to Cleveland for an evaluation.  We also have excellent pediatric cardiac surgeons here as well.  To make an appointment with a pediatric cardiologist, call 1-800-CCF-CARE and ask for Desk M-42 .  I hope you find this information useful.  Information provided in the heart forum is for general purposes only.  Specific diagnoses and therapies can only be prescribed by your physician.