Pediatric Heart Expert Forum
9 month w/ williams syndrome and PS
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9 month w/ williams syndrome and PS

My 9 month old baby suffers from pulmonary stenosis and on her las visit she had a pressure of 71. Her cardiologist said that he is kind of afraid of having the ballon procedure done on her and that he hopes she will get better by herself. When she started seeing him 9 months ago she had a pressure of 30. Do you think she will get better by herself? Should I seek for another opinion? Does her PS still on moderate or is severe now?
Please help me. Thank you.
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773637_tn?1327450515
Dear Ediefaith,

I do not have all of the information that I need to be able to tell you exactly what to do with your daughter at this point.  From what I can tell, she has pulmonary stenosis that has certainly gotten worse.  I am assuming that her pressure that you tell me is an estimated gradient of 71 mmHg, which was obtained by echocardiography.  However, this gets influenced by several factors.  One of those factors is something that you don’t mention in the information you gave me but you do in the title of your posting, which is that your daughter has Williams syndrome.  This is a genetic abnormality of the elastin gene, that codes for a protein that makes up the connective tissues.  One of the places where we see the abnormal elastin is in the arteries leading out from the heart.  The pulmonary artery and the aorta can have obstruction, or stenosis, in them.  However, the stenosis is not at the valve, but above the valve; we call this supravalvar stenosis.  My guess is that your daughter has supravalvar pulmonary stenosis, if she has Williams syndrome.  In this case, performing a cardiac catheterization with balloon angioplasty, in which a catheter with a balloon on the end of it is placed in the artery and inflated to eliminate the stenosis, usually does not work.  The artery simply recoils after the balloon is deflated and the stenosis remains.  Therefore, surgery is what is typically indicated if the obstruction becomes moderate or severe.  If this has progressed as you say, it will not get better by itself and will need intervention.  If your cardiologist is not answering your questions adequately to make you feel comfortable, you should consider obtaining a second opinion and assessment of your daughter’s cardiac condition.

For our other readers, Williams syndrome has other features besides the abnormality of the connective tissue.  This includes developmental delay, a classic facial appearance, some musculoskeletal problems, sensitive hearing, and an increased likelihood of hernias, dental problems, and kidney problems.  If you haven't already, you should look online for the Williams Syndrome Association for more information.
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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