Pediatric Heart Expert Forum
Hypoplastic Right Heart Syndrome
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Hypoplastic Right Heart Syndrome

Im 21 weeks pregnant and have just found out my baby has Hypoplastic Right Heart Syndrome! I know its more rare than the left but im trying to find someone from uk whos child has the same thing. I can only seem to find info or story's about the left. The only story's i found about the right side are all in America. So please if there is anyone who can help id be very gratefull xx
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Dear Lou Jacob,

I cannot connect you with someone in the UK w/ hypoplastic right heart syndrome (HRHS), and as an American cardiologist, I am not going to be able to give you much more information than you have likely already found.  Hypoplastic right heart syndrome is another term that is more typically associated with pulmonary atresia and intact ventricular septum.  In this case, the pulmonary valve is a muscular or membranous plate.  As well, there can be varying degrees of hypoplasia (undergrowth) of the pulmonary artery.  Finally, the right ventricle, the ventricle that pumps out to the pulmonary valve and pulmonary artery, is usually severely thickened and often small.  As well, there can be abnormal connections to the coronary arteries from this ventricle.

I cannot give you a specific prognosis for your baby, but can discuss some general topics.  Depending on the size and adequacy of the right ventricle as well as the pulmonary valve, it may be possible to get across the atretic pulmonary valve plate with either a special radiofrequency burning a hole or with a stiff wire puncturing a hole.  The valve can then be dilated with the attempt to establish forward flow out to the lungs and give a two ventricle result.  Sometimes, though, this is not possible (for many different reasons).  If this is the case, then performing a series of operations, including an initial Blalock-Taussig shunt (a tube connecting the aorta to the pulmonary artery) to establish pulmonary blood flow followed eventually by a Glenn and then a Fontan operation, which would take down the shunt and attach the head and neck veins and the lower body veins, respectively, directly to the pulmonary arteries would be indicated.  Your cardiologist will work with you to determine which will be the best approach.  There is some evidence to suggest that patients with a left ventricle as the systemic ventricle, as in HRHS, do better with their Fontan than do patients with hypoplastic left heart syndrome over the long term.  However, we will need to see if this continues to be borne out with improvements in the surgery overall.
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