Pediatric Heart Expert Forum
Prognosis for HRHS
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Questions in this forum are answered by pediatric cardiologists, cardiothoracic surgeons and anesthesiologists from The Children's Hospital of Philadelphia. This forum is for questions and support about pediatric heart problems, symptoms and topics such as heart murmurs, palpitations, fainting, chest pain, congenital heart defects (including management and intervention), fetal cardiology, adult congenital cardiology, arrhythmias and pre-participation athletic screening.

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Prognosis for HRHS

Lexie, my daughter, was born with Hypoplastic Right Heart, didn't see that in your topics.  I was told she would have 3 surgeries without question before she was born.  She had the BT shunt at 15 days old and it closed several years ago.  Now she also has pulmonary atresia I think, her pulmonary valve was ballooned when she was small so she will need one at some point and has a small hole in her heart as well.  Her oxygen stays in the 70s most of the time, it has just started hitting low 80s every now and then but not regularly but her Echos look the same, right ventricle still small and doesn't appear to be growing yet on the echo.  We have a great cardiologist who says as long as she is doing well with those oxygen levels he thinks we can just wait it out and hopefully just put in a pulmonary valve and close the hole in about 10 years.  I can't find anywhere or anyone who has been this route.  Everyone else has had to have the other 2 surgeries.  Can you tell me if there are others out there like my Lexie and is the prognosis good or should we be overly concerned with blood clots or her heart wearing itself out?    Lisa, Lexie's Mom
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From your description it sounds like your daughter has pulmonary valve atresia, intact ventricular septum (PA/IVS) with a hypoplastic right ventricle.  This is a complex group of patients and their management must be individualized, depending upon the size of the right ventricle and tricuspid valve, and whether or not the coronary artery flow is dependent upon maintaining a high pressure in the right ventricle (RV dependent coronary circulation).  If your daughter underwent a balloon of her pulmonary valve, then she likely does not have RV dependent coronaries.  The pulmonary valve is only opened if they are trying to lower the RV pressure and encourage the right ventricle to grow (which can occur).  PA/IVS patients also have blue blood going "right to left" across the hole between the upper heart chambers called the patent foramen ovale.  That, plus limited blood flow across the pulmonary valve will cause the child to remain cyanotic. It sounds like she had an aorto-pulmonary shunt placed early in life to maintain decent oxygen levels and that has now closed.  Ideally to maintain a reasonable level of function, the oxygen saturations in room air should be no lower than 75%. Chronic blueness does make some additional work for the heart.  If she is running lower than that, then an attempt should be made to increase the amount of blood flow going to the lungs, either by redilating the pulmonary valve, or doing surgery for another shunt or patching the pulmonary outflow tract open.  The "3 surgery" plan you were told about pre-natally is staging towards a Fontan operation, which is necessary when the right ventricle does not grow adequately to do its work.  If your cardiologist is considering NOT going the route of a Fontan, this implies that the RV is getting bigger with time.  You should certainly have your cardiologist address your concerns about the plan of action in your daughter's case, or get a second opinion at a large institution that deals with alot of children with complex congenital heart disease.  
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Jeffrey R Boris, M.D.Blank
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