Justin's Heart Update 2003

This is the "all you ever wanted to know about Justin's heart" letter. We have done tons of research and discussed things with Justin's pediatric cardiologist (PC) again this week after a somewhat disappointing (but still good) checkup on April 2. Some of the things we have thought or "understood" before were not correct or have changed, so bear with us as we try to understand all of this ourselves...

Background

When he was born, Justin had very critical pulmonary valve stenosis (PS)...they called it "near atresia" (atresia means complete blockage)...and a hypoplastic right ventricle. Basically that means that the pulmonary valve (going from the right side of his heart and leading to the lungs) was misformed and almost closed...the right ventricle (bottom right chamber) was misshapen and much too small. He also had some problems with the right atrium (upper right chamber) being enlarged from the extra pressure buildup and the tricuspid valve (between the two right chambers) being somewhat narrowed and leaking. Finally, he had two holes...a PDA between the aorta and the pulmonary artery (artery carrying blood from the right side of the heart to the lungs) and a PFO between the two atria (top chambers). In general, his heart was in overall worse condition than we realized.

The procedure they did when he was a baby was with a catheter. They used two sizes of balloons to open up the pulmonary valve...and they used an occlusion device to close the PFO. When the PFO closed, his oxygen levels dropped from around 90% to 60% (on oxygen), so they had to use another balloon to make a hole (called an ASD) between the two atria (top chambers) to release pressure on the right side of the heart.

Today Justin still has the ASD, a misformed pulmonary valve, some narrowing of the tricuspid valve, as well as leaking in both valves. The leaking causes the chambers to enlarge from the added pressure, so he has moderate enlargement of the right atrium, mild enlargement of the right ventricle and some enlargement of the arteries leading to the heart and from the heart to the lungs (superior vena cava and pulmonary artery). The right ventricle is still slightly hypertrophied, meaning the wall is too thick from working harder to get blood through the narrow pulmonary valve. His right heart just isn't normal and doesn't function efficiently.

What No Surgery in First Three Years Means

We have learned that *every* child with Justin's degree of PS automatically has an operation called a B-T shunt...this reroutes some of the blood from the aorta back through the pulmonary artery to the lungs, which allows it to pick up more oxygen. This is the surgery we thought Justin would have before he left ICU as a baby (but he didn't). This procedure is intended to help the child have more oxygen and be able to survive and function better until he is old enough to have an open-heart (OH) correction or replacement of the misformed valve. (This was a revolutionary procedure in the 1940s when there was nothing that could be done and diagnosis of critical PS was only possible by autopsy...) So...by standard practice any other kid born with Justin's condition in the year 2000 would have had two (or more) surgeries by now...the B-T shunt and OH valve fix/replacement.

Thankfully we had some cutting-edge doctors at Texas Children's Hospital (TCH) who decided Justin was a good candidate to try using *only* the balloon catheter procedure (which is done routinely on less-severe cases of PS) to see if that would do what the B-T shunt would have...that is, get him to an older age with a good quality of life before he has to have surgery...and it has worked really well!!! Justin has grown wonderfully and been an active and happy boy! We have experienced a lot of confusion with what we have been told simply because the doctors just don't know what to expect...as it turns out, they've never had a kid with this degree of PS who has never had surgery...it is all new territory...wow!!

Future Prognosis

The bad news is that it seems Justin will still need the corrective surgery at some point. They say some kids do OK for years with this moderate amount of leaking, but that we can expect valve replacement sooner or later...and possibly another kind of shunt (Glenn procedure) that would take blood coming back to the heart and route it directly to the pulmonary artery and the lungs...rather than sending it through the right side of the heart. We assume the purpose is to just take some pressure off of the heart.

With valve replacement now in mind, we have learned all about that...they use several types of artificial valves, but our PC said they are not happy with any of them and would hope to have a better valve before Justin needs his. They tend to "calcify" easily and sometimes (too frequently) have to be replaced even as soon as 6 to 8 months after being put in. Of course, some do better and last for years. There's the concern of growth and having to replace the valve repeatedly due to size, and there's also the issue of how long the valve itself lasts. Mechanical (metal or plastic) valves last longer but the child has to be on blood thinners and have blood drawn once a month for checks. As you know, with most boys/kids there is enough risk of injuries that being on a blood thinner is not that great an idea...head injuries particularly can be dangerous. Other valves are derived from cows (bovine) or pigs (porcine), but they only last 5 to 10 years. So, once we do replacement, it starts a chain reaction and a cycle of OH surgeries to replace the valve.

New Procedure to Consider

The best news we have found (mentioned by our PC a while back and by another heart mom this week--thanks Sherri A.:-))) ) is that in the last two years there is a doctor in London who has been doing valve replacements via catheter (rather than OH surgery) for the first time. They have done about 20 with great success, including several kids flown from the US. We are hopeful that Justin may be a candidate for this so that he can continue to avoid OH surgery. Because our PC has been so proactive in giving Justin chances, we plan to involve him in our push to have this procedure when the time comes. He said if we can wait long enough with Justin, they may be doing this here in the US by the time he needs a replacement. We think since our PC is director of the catheterization lab at TCH that he would be interested in even being involved in bringing this new technology to the US, or we would be willing to take Justin to London to have the procedure done there. Since we are not facing surgery immediately and still have time to wait, we are going to hold off discussions for now and see where things are when Justin needs corrections done.

We have Justin's next checkup next April, and we should know alot more at that time, so this is an important time to pray for improvement and for the doctors and technology available. We know Justin will do fine no matter what he has to go through, but we would go to great lengths to spare him any amount of pain.

Thanks for wading through all this and for all your prayers and concerns over the last three years.

Love to all,
Toby, Sharon & Justin Dunn

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