Pediatric Heart Expert Forum
congenital heart defects
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congenital heart defects

My baby has been diagnosed with pulmonary atresia with hypoplastic right ventricle. what does this mean and what effects will it have on my baby? will this effect the type of birth, and what is the mortality rate in cases such as this?
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Dear Nikki,

Pulmonary atresia with hypoplastic right ventricle, also known as pulmonary atresia with intact ventricular septum, is a congenital cardiac defect in which the pulmonary valve (the second valve on the right side of the heart) did not appropriately form.  The outlet to the right ventricle, the pumping chamber on the right, is typically completely obstructed.  Without evaluating your baby, I cannot say exactly what will happen.  By the way you are writing, I am assuming that your baby has not been born yet.  Overall, these babies do just fine in utero, as long as there is no obstruction anywhere else in the heart and the blood is flowing appropriately to the rest of the heart and the body.  As well, it should not affect the type of birth BUT it does affect where your baby should be delivered, as you will see below.

Once the baby is born, there are changes that occur in the blood vessels and lungs that need to be dealt with.  Your baby will likely need to be treated with an IV infusion of prostaglandin, a medication that helps to keep the ductus arteriosus open.  The ductus is a blood vessel that all babies have in utero that typically spontaneously closes soon after birth—this is a normal event.  However, your baby will actually need the ductus to remain open (patent) in order for blood to get to the lungs from the left side of the heart.  We typically recommend that these babies be born at, or near, a children’s hospital with a complete pediatric cardiac center, including surgery, interventional catheterization, and cardiac intensive care services, so that they can immediately get the evaluation and care that they need to prevent any complications.

Without any intervention, this cardiac defect is eventually lethal.  However, with initial treatment to ensure that the ductus arteriosus remains patent, this allows for time for the baby to be considered for intervention.  There are various interventions that can be performed.  One actually perforates the atretic pulmonary valve plate to establish blood flow forward from the right ventricle to the lungs.  This can potentially establish a two-ventricle heart.  Another would replace the ductus arteriosus with a shunt that semi-permanently directs blood into the lungs until the baby grows bigger.  If this is the case, which means that the heart is not felt to be adequate to support a two-ventricle repair, the baby will need to undergo a series of three operations to make the heart into a single-ventricle heart.

Sometimes, the right-sided coronary arteries are dependent upon the very high pressures generated by the right ventricle.  When this pressure is relieved by opening up the pulmonary outflow tract, it can cause decreased blood flow to the coronary arteries and, subsequently, to the heart.  This can be life-threatening, and can make the heart not amenable to any kind of reasonable repair.  Thus, these patients may need a cardiac transplant to survive.  Without seeing your baby’s heart, it is difficult for me to tell what will need to be done specifically.  I would recommend further discussing this with your cardiologist.  If you feel that you are not getting the answers to your questions that you need, you should consider a second opinion.
2 Comments
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I am not a doctor, and my daughter does not have HRHS. But I wanted to comment because I have met the woman who founded the Hypoplastic Right Hearts organization. It is an organization of parents and families who have children with Hypoplastic Right Hearts and as of last count they had 700 member families. They have an online message board as well phone and in-person supports. While they are not doctors, they all have children with HRHS and could provide you with parent perspective about what the future will hold. They don't allow you to post links on here, but the web address is hypoplasticrighthearts and the woman's name is Amanda. I know if you contact her/them they will get back to you quickly. I can tell you that her son is 7 and currently doing well.

I wish you and your baby all the best as you learn what the future will hold.

Rene

  
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