Justin's Diagnosis

We were confused for a long time on what to look up stuff under. They gave us "pulmonary stenosis" as the term that we latched onto, but when I look that up, all I find is "usually very mild and no intervention is needed" kinds of descriptions. Well, that's not the kind of start that we had with Justin! We now know that they classify Justin's defect as "critical pulmonary stenosis (near atresia)" on most of the clinical reports, and on some they just refer to it as "pulmonary atresia."

So, look up stuff on "pulmonary atresia." There are two kinds of that, one that is "pulmonary atresia with VSD" and the other more rare one is "pulmonary atresia with intact ventricular septum (IVS)." Justin's is the pulmonary atresia w/IVS. That is the more severe one because with the VSD, there is an alternate way for the blood to get through, and with the IVS, the right ventricle doesn't have an outlet so it doesn't fully develop, leaving the baby with a very poor pumping chamber. They told us numerous times that just 10 years before Justin was born in 2000 that there really wasn't a sure way to treat PA w/IVS, and the research I find supports that. The statistics given in research in the 1980s are very grim with only a small portion of the babies surviving the first year even with treatment. By the mid '90s, the numbers were greatly improved.

PA w/IVS falls within an umbrella category called "hypoplastic right heart" (which includes PA w/IVS and other different defects that also lead to the right ventricle being too small). Known as HRHS (hypoplastic right heart syndrome), this is considered one of the two most lethal heart defects. Only HLHS (hypoplastic left heart syndrome) is more serious because the left ventricle is so much more critical in getting blood back to the body. So, when I mention to people that he has a heart problem and they ask "Oh, did he have a murmur?" I have to really bite my tongue and keep myself from HITTING them (just kidding!!). Point being that this is a very severe heart defect even among other heart defects.

So, there are your two phrases to do searches on: "pulmonary atresia" and "hypoplastic right heart" (or HRHS).

Specifics on Justin's Situation

Since there is a wide spectrum within each defect, each kid is really dependent on their own particular combination of defects and how they do. Justin was better off than many other kids with HRHS or PA w/IVS because his ventricle was only moderately hypoplastic. The doctors working with him disagreed on his treatment, and we were very confused for a long time. Apparently one of the drs. wanted to do the "standard" treatment, which would have left him *much* more limited in activity level and possibly in longevity. The standard treatment is what they call a "Fontan track" which is a series of three open-heart surgeries to "stage" them to a one-ventricle system. The first surgery is done as a newborn, called the BT shunt. The second surgery is done around 6 months old, and is called the Glenn. And the third surgery is done anywhere from 2 to 4 years old, and is called the Fontan. That series of surgeries would essentially re-route the blood coming back from the body directly into the pulmonary artery to go to the lungs, rather than sending it through the defective right side of the heart. It uses gravity and blood pressure variants to get the blood to the lungs without the pumping action of the right ventricle. If they had done the BT shunt and started him down that track, Justin would never have had a chance at a four-chamber heart.

"Dr. Doom" as we called her, kept telling us that Justin would have the BT shunt by the end of the week. The director of the catheterization lab (Justin's cardiologist today) and one of the drs. there on a fellowship wanted to give his ventricle a chance to grow. So when he was two days old, they did a procedure with a balloon catheter where they opened up the bad pulmonary valve and closed the PDA. When they did that, his oxygen level plummeted to 60% (while on oxygen), so they knew his right heart couldn't handle the whole load, so they made a "popoff" in the form of a hole between the two upper chambers (called an ASD for "atrial septal defect"), so that the extra volume of blood could escape over to the left side of the heart rather than just enlarge the right side of the heart. That was good for when he cried or when he was eating (which they consider a tremendous amount of work for an infant), because when his heart was working hard, he wouldn't have a big problem from it, just more blood going over to the left side. However, that also allowed "non-oxygenated blood" to mix in with the blood in the left side of the heart that had just come back from the lungs. That reduced his blood oxygen level significantly at first. So, for a week, they waited for him to be able to handle the workload of the heart and get his oxygen saturation up above 70% (normal people have 98 to 100% oxygen saturation). He looked pretty blue from the low oxygen sats.

Meanwhile "Dr. Doom" kept telling us that he would be in surgery for the BT shunt any day now. Our cardiologist has told us since then that he and the fellow had to "lay their bodies across Justin's bed to keep them from rolling him out to surgery." Thankfully they won, and his oxygen sats gradually came up, and they let us come home without the BT shunt at 10 days old with oxygen sats in the upper 70s. We fully expected some kind of surgery that first year, but he continued to surprise them all. He's one of a handful of kids with his condition who have never had open-heart surgery. Pretty AMAZING!! Many prayers were answered there. By the end of the first year, his oxygen sats were in the low 90s, where they have remained since then.

Remaining Issues

When the balloon opened his valve up, it left it damaged, and he has had a moderate amount of leaking since then. They said that was actually good at first because it let extra volume into the right ventricle, which would force it to grow bigger -- which is exactly what we needed above anything else -- and that worked! His ventricle is now in the normal size range, although the muscle is still too thick due to working hard to force blood through a defective valve. And now his right heart is enlarged to some degree from the extra volume from the leaking, and also the ASD that they made is allowing blood to shunt over from the left side of the heart. That is what we think is contributing to the susceptibility to upper respiratory stuff and colds. The extra volume in the right heart creates higher pressure in the vessels in the lungs, which affects the ability to fight off respiratory stuff. However, the cardiologist said yesterday that he thinks Justin's hole is too small to be causing those problems, so they are leaving his next echo and checkup until April.

The other issue related to colds/etc. is that anyone with a heart defect or a faulty valve is at increased risk for developing "bacterial endocarditis." This is an infection that settles on the already defective valve and grows. Any infection in the body can travel through the bloodstream and attach to the defect. That's why heart patients have to take antibiotics prior to any dental work, including cleaning, and before any surgeries, etc. This is a very serious infection of the heart that if untreated can kill them, and if detected takes a minimum of 4 weeks of very high-dose antibiotics and possible hospitalization for that length of time to stop the endocarditis. It can leave the heart in an overall weakened condition. So, we have strong incentive to avoid any infections that we can with Justin.

What the Future Holds

Prognosis is.... "we don't really know." Many of the ones who are reaching adulthood who have had the Fontan track are needing heart transplant by early adulthood, but since we are not on the Fontan track, we are just playing the wait and see how he does game. I think the doctors really don't know with him. They expect that at some time he will need an artificial valve, which would require open heart surgery. If they do that while he is still growing, he will likely need it replaced about every 4 years so it can "grow" with him. So, that's not something we want to start any sooner than we have to. They also think he may still need some of the volume load taken off of the right side of the heart. Over time, any muscle that is working too hard will begin to fail, so to avoid that they would do the Glenn surgery (the 2nd in that series on the Fontan track), where the blood coming back from the upper half of the body would be directed straight to the lungs, and the blood from the lower half of the body would continue to go through the right side of the heart and the artificial valve that they would place. This is called a "one-and-a-half ventricle fix" as opposed to the two-ventricle system that he has now and the "one-ventricle fix" of the Fontan track.

They've always told us that around age 4 to 6, he would need to have his ASD closed, which can be done with a catheter. At last year's visit though, his cardiologist told us that many doctors use that as the next step since sometimes that will help balance out all the other stuff going on; however, he didn't think in Justin's case that closing it would help, and he would rather skip putting him through a catheter procedure, and just do the other surgeries and close the hole while they are in there. Last year's visit definitely sounded more like "when" than "if" on open-heart surgery. But nothing is definite, and it just depends on what they see with the echocardiogram at his next visit. He may still look great, or if not, they may have some medications to help put off surgery for a while longer.

Back

[ Previous | List Sites | Stat's | Next ]
This Congenital Heart Disease RingSurf
site belongs to
The Justin Dunn Congenital Heart Foundation.
Click Here To Join The CHD Webring

Next