Ultrasound & Echo – 30 Weeks Pregnant

Pedro was nice to the ultrasound tech today and he even let her  take some images of his face! I think he liked her because she said nice things about his cuteness. Braden thinks that the tech was just sneaky/stealthy (read: she didn’t push very hard) and therefore Pedro did not detect anything to attack. Whatever the reason, we are happy to see his wee little mug:

And here is his weapon of mass destruction (my ribs cringe at the sight…)

The tech calculated his weight at 3lbs. 9 oz. Apparently that is more than a large jicama, but less than a pineapple (if you like to  think in terms of produce, and who doesn’t?) A pineapple is much cuter than a jicama, but I doubt either of them would be as good-looking as Pedro is going to be in his Angels gear and various duds from the grandmas and others. (thanks ♥)

Now about his heart.  Today they could see that the right side is significantly enlarged. The regurgitation of blood from the right ventricle back into the right atrium is causing the atrium to fill up and expand. While it is no surprise that this is happening–everyone had hoped it would be later in the pregnancy (or not at all, please!)

I *think* this next image shows the problem. I could be way off because we weren’t looking at anything in particular when the doctor told me about the enlarge-ness. But I believe these measurements of his heart show a large circle to capture the circumference of the whole heart, and a smaller circle around the right side of his heart:

We’re still crossing our fingers for a full term baby. Starting after my St. Joe’s ultrasound next week, I’ll go into the OB’s office twice a week so that they can keep a close watch.

In the meantime, it’s a really good sign that Pedro is so active and it should be easy to tell if his heart starts slowing him down. I’m not so sure that this little dude would let anything deter him from his regiment of kick-boxing and aqua aerobics, but I’ll notice if he does actually get quiet.

This last image is just one that I thought was interesting–it shows the blood flow to and from Pedro via the umbilical cord:

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All Roads Lead to Nigro

Yesterday I had a consultation with Dr. Jedeikin from the Arizona Pediatric Cardiology group (affiliated with the Phoenix Children’s Hospital) for a second opinion/option kind of thing. We were tempted to cancel the appointment because we feel so good about the St. Joe’s group, but we decided that we should see what Dr. Jedeikin  had to say. You know, due diligence and everything.

Dr. Jedeikin seems really great. (And I’m not just saying that because I like his South African accent…) He is very, very passionate about hearts and cardiology. He did a fetal echo and went over Ebstein’s Anomaly with me again. I always learn new things about the heart, and I came away in awe of all of the many facets of a human heart–how do all of those parts work perfectly in most people?!

Dr. Jedeikin’s assessment and approach to Pedro’s case is pretty much the same as those we’ve heard from the other doctors involved. He hopes that immediate surgery will not be necessary, and has pretty much the same plan for monitoring our little guy’s heart–both pre and post delivery. He had good things to say about the Phoenix Children’s Hospital (PCH), but apparently he could work with us even if we choose St. Joe’s for delivery. He encouraged me to deliver at St. Joe’s since that is where my new OB delivers (rather than switch OB’s again in order to deliver at the hospital near PCH). From this I concluded that both hospitals are a good choice, and we just need to pick the one that we feel most comfortable with. They both have Level 3 NICU’s.

Probably the most interesting/encouraging part of this consultation was that Dr. Jedeikin would involve Dr. Nigro for the surgery aspect. Dr. Nigro seems to be the go-to guy for surgery in these cases, regardless of which hospital we go to.

So, since:

  1. the hospitals are comparable
  2. we get Dr. Nigro either way
  3. the Pediatric Cardiologists are excellent either way

…we are free to choose the hospital and Pediatric Cardiologist(s)  that we feel the best about. We still appreciate that at St. Joe’s I would deliver and recover at the same hospital where Pedro would be in the NICU. We also feel like the program with the St. Joe’s cardiology group is a better fit for us–much more supportive and user-friendly. Also, once you meet Beth, the nurse practitioner, it’s a no-brainer. She is off-the-hook amazing.

Although it did not change our mind, I’m really glad I went to see Dr. Jedeikin. Now we can feel even more confident about our decisions, and I appreciated learning more about Pedro’s heart. Here are a few things I now understand after meeting with him:

  • The tricuspid valve is made up of three leaflets, one of them is the ‘Septal leaflet.’ Pedro’s septal leaflet is dysplastic–which means it is gnarly, thickened and crumply. The other two leaflets are doing all of the work to open and close the valve as best they can–which is why one of the leaflets is longer and floppier, it’s trying to cover the Septal leaflet’s territory too.
  • Dr. Jedeikin did not think that Pedro’s Septal Leaflet would be fixable because it is so dysplastic.
  • If surgery does become necessary, it is possible that they would move the leaflets closer together so that the functional leaflets can adequately close the valve with each heartbeat.
  • He didn’t go into all of the details, but if it becomes necessary to surgically re-route Pedro’s heart so that it operates with a single ventricle scenario (not preferable), there are a few different procedures:

Blalock-Tuassig Shunt

Starnes Procedure

Glenn Procedure

Fontan Procedure

{Pedro could need all, none, or some combination of the procedures. We have heard a little about these procedures before, but had not learned all of the names yet. I’ll activate hyperlinks if I find good descriptions for them.}

Oh and, Pedro’s new trick of the day was rolling. The sonnographer laughed with me this time when Pedro would roll away while they were trying to record his heart. Then he would roll back and punch the ultrasound wand. He definitely has a little personality already ♥

Meet the Surgeon

Here I am getting more rotund, these were taken this morning right before we visited St. Joe’s again:

Our activity of the day was to sit down with Dr. Nigro (\nye-grow\)  in order to get to know him and play 20 Questions. I think we got really close to 20 Questions–but they didn’t give us a prize or anything…Bummer. Dr. Nigro has extensive experience handling similar cases of Ebstein’s Anomaly with successful outcomes. The only fatalaty he has seen was a result of an unrelated illness, and not from the surgery itself.

Dr. Nigro was very encouraging. He talked about the range of severity of Ebstein’s in babies like ours, a spectrum that goes from the severe end to the mild end. At the mild end the babies seem like any other baby when they are born (on the outside). Dr. Nigro feels that Pedro will probably be closer to the mild end and he is hopeful that surgery won’t be necessary right away. He compared Pedro to a little girl who was born with a similar case of Ebstein’s and she just barely had her first surgery (and they hope her only surgery) at about 17 months. Compared to surgery as a newborn, surgery after the first year sounds much better.

Dr. Nigro also feels that the Fontan procedure , where the plumbing of the heart is re-routed to skip the faulty valve, will not be needed for Pedro. He seems hopeful that Pedro’s leaky valve will be repairable. Whether or not it takes multiple surgeries will depend on how early surgery is required and on how well Pedro does with it.

All around, we enjoyed meeting Dr. Nigro–especially because in addition to being capable and professional, he has a good sense of humor. We had read his bio beforehand and knew that he had done his residencies at USC. So, of course, Braden’s first question was sports related–and Dr. Nigro just ran with it. Is humor a basis for selecting a hospital and a surgeon? Nope.com. But it’s nice to feel a connection with them anyway.

Help me OB-one

This morning I met my new OB, Dr. Blumrick and he is great—very personable and optimistic. Also, very chatty, which is nice in some respects. He set a goal for delivering Pedro after 39 weeks (so sometime after Sept. 9th would be ideal). Dr. Blumerick was pleased that Pedro’s Right Atrium is still the normal size, and besides the Right Ventricle, the other parts of the heart are all in the 50th percentile for size. So his heart overall is just the right size—not too big and not too small.

He also told me that when my former OB did bloodwork on June 10th to check for Gestational Diabetes (which I don’t have-yay!), my platelet count was at 140—so we are getting even closer to a normal range and rising. A small win, but a win nonetheless.

And what would a doctor’s visit be without an ultrasound?—so we did that again as well. Does this image look a little even more blurry than usual?

Yep. That’s because he refused to hold still. Pedro kept kicking and wiggling when he wasn’t supposed to–and wouldn’t budge when they needed him to move into a position that would make things more visible. Oi! We are in for it with this kid!

Dr. Solomon, a Maternal Fetal Medicine Specialist, did part of the ultrasound. She explained more about hydrops: when the tricuspid valve is regurgitating blood, the leaked blood can sometimes pool up and cause fluid to be where it shouldn’t. If untreated long-term, this could cause heart failure. If untreated short-term, it could cause damage to the surrounding organs, most notably the lungs. We vote “no” on hydrops, and so far we have every reason to hope that we’ll get through the pregnancy without them developing.

“St. Joe’s” – 27 Weeks Pregnant

Early this morning we had a fetal echo and a consultation at the Scott and Laura Eller Congenital Heart Center at St. Joseph’s Hospital and Medical Center (St. Joe’s).  The ultrasound showed that Pedro’s tricuspid valve is leaking a significant amount of blood from the Right Ventricle into the Right Atrium. We sat down with a Fetal/neonatal Cardiac Nurse Practitioner and a Pediatric Cardiologist. We felt really comfortable with them and came away much more reassured. They confirmed the diagnosis of Ebstein’s Anomaly and explained the condition using diagrams and a plastic model heart. Here are some things we learned from them:

  • Congenital heart defects are the most common birth defect
  • Ebstein’s Anomaly (EA) accounts for < 1% of congenital heart defects—it’s very rare and seemingly random
  • They are not sure what causes EA, but they know that it starts forming between 4-6 weeks of gestation and that it seems to be linked to the sequencing of DNA. So they think it isn’t genetic and it isn’t environmental. It’s a fluke.
  • After birth, circulation changes and there’s a chance that the leak will not be as severe—so it’s possible that his valve and heart could function well enough to buy some time for him to mature and grow a bit before surgery.
  • Pedro’s Pulmonary Artery (which takes blood to the lungs) is developing as it should. This is significant because in cases of EA sometimes the artery is affected. Pedro’s healthy artery increases the chances of his little heart functioning adequately after birth.
  • Families who have children with congenital heart defects are usually amiable to meeting and mentoring people like us—the nurse is getting us connected with parents who have been through this.

Their approach to Pedro’s case:

  • The goal is to go full term and deliver at St. Joe’s with a team of cardiac specialists—everyone who would be involved, from the nurses to the surgeons, has a background in working with babies and children with congenital heart defects.
  • The only reason to deliver early is if hydrops form. Hydrops are moisture retention around the heart.
  • We will get to hold Pedro for a few minutes when he is born, as long as he is breathing okay. Then Pedro will go to the NICU to be monitored, Braden will go with him.
  • Pedro will most likely need heart surgery at some point in his life—after he is born we will know if he needs surgery immediately or if it can wait until he’s a little older.
  • If Pedro needs surgery immediately, they would probably keep him at St. Joe’s for a month. We would stay there with him in a family suite.
  • There are two different surgical approaches on the table:
    • If Pedro’s tricuspid valve is repairable, then it should only take one surgery to fix it up and reduce the leaking. He will never have a normal heart and there is a chance that he would eventually need a valve replacement when he’s older—but there is also a chance that it would never bother him again.
    • If Pedro’s valve is beyond repair, they can re-route the blood so that it skips the right side of his heart completely. This would take three surgeries—probably one in the first week of birth, a second surgery at 3-4 months old, and a third surgery at 2-4 years of age.
    • A valve replacement is not a good option in a newborn because the valve would not grow with him.
    • In the NICU they will nourish Pedro through an IV into his umbilical cord stub. They will also use the umbilical cord to draw blood samples so that they don’t have to stick him every time.
    • As long as the surgery/surgeries go well, Pedro should have a normal life and a normal life expectancy.
    • Dr Diab estimated Pedro’s chances of survival to be over 90%
    • Barring the development of hydrops—they do not anticipate that I will need to be induced or have a C-Section. If either of those procedures do become needed, it would be for the usual reasons and not because of Pedro’s heart.

Fetal Echo – 26 Weeks Pregnant

It has become an interesting mix of dread and excitement when I go to these appointments now. I still love, love, love to see my baby. The highlight of this particular visit was that Pedro had become quite the kicker (or elbow-er, headbutt-er, whatever he can throw at you-er). It was probably more entertaining for me than for the poor ultrasound technician when Pedro would kick the ultrasound wand. The kid’s got good aim!

Despite Pedro’s burst of activity, the technician and then Dr. Nyberg were able to see that the condition had progressed and our Pedrito was diagnosed (officially) with Ebstein’s Anomaly. By this point, his tricuspid valve was more obviously misshapen. As a result, it had become floppy and leaky. Apparently, blood is only supposed to be on a one-way track through the heart—in the right side of Pedro’s heart the blood is traveling in both directions.

The Fetal & Women’s Center has an amazing Doctor/Patient Care Coordinator, Susan, who worked with me via email to set up consultations with the cardio groups at both St. Joseph’s and the Phoenix Children’s Hospital. It will be important for us to deliver at a hospital with a NICU—even if Pedro doesn’t need surgery right away, he will definitely need to be monitored closely. Susan also sent me some information and arranged for me to transfer to the Center’s OB for my regular pregnancy visits. (My original OB does not have privileges at either of the hospitals with a NICU.)

Fetal Echo (Ultrasound) – 21 ½ Weeks Pregnant

Instead of having an ultrasound at my obstetrician’s office, the ultrasound people (aka the Fetal & Women’s Center of Arizona) had me come to their own office. We thought this would be another boring visit, so we didn’t bother scheduling it at a time that both of us could be there. Afterall, ultrasound technicians are not really allowed to tell you much, so I went solo to this appointment to let them examine Pedro’s “a little small” heart. The technician checked all the other anatomy again for good measure and she tried in vain to get a 4D image of his face for us, but Pedro was determined to thwart her plans—a scheme he must have inherited from his father. The photographer in me groaned for my future, but I couldn’t help but laugh at our funny little dude:

Blah, blah, blah she checked everything and then spent some time looking at, measuring, recording, and otherwise studying Pedro’s heart. We were right—she couldn’t tell me much. Imagine my surprise, though, when she stepped out to get “the doctor” so that he could look if he wanted. And he wanted.

Dr. David Nyberg was absolutely wonderful. He came in and did his own ultrasound to get a few more images. Pedro tried his best to move around at all the right (translation: wrong) times, but in spite of that Dr. Nyberg was able to see what he needed to. He explained as he went along that the valve on the right side of the baby’s heart seemed to be shaped a bit abnormally. Afterward, he had me come into his office to look at diagrams (from a ginormous book that he himself had co-authored).

Dr. Nyberg explained that the misshapen tricuspid valve in Pedro’s heart appeared to be similar to a congenital heart defect called Ebstein’s Anomaly.  He was quick to say that if it was indeed Ebstein’s, it was a super mild (okay, he didn’t say “super”) case of it—so much so that he was not even comfortable diagnosing it as Ebstein’s at that point. He recommended that we look at it again in few weeks to see how it would develop. He told me not to research Ebstein’s yet, because it would just scare me and would not be applicable to Pedro since his funky valve was only mildly funky.

I tried not to be scared—but they aren’t kidding about those pregnancy hormones!

We started praying more regularly for his heart to be strong, and it took me awhile not to chuckle when I would say, “…bless his heart…” in my prayers. I guess that comes from spending a lot of time in the South—which conditions a person to expect some kind of disparaging remark immediately after such a phrase. (i.e. Bless her heart…Jessica is a lot  of things, but coordinated is NOT one of them.)

Ultrasound -18 Weeks Pregnant

We posted pictures from the ultrasound on our regular/photography blog, but what we didn’t post at the time was that the ultrasound technician popped back in while they were taking my blood pressure to say something like this: “The doctor wants you to come in for another ultrasound in a few weeks—the baby’s heart looks a little small.”

Then she disappeared with no further explanation and we were left wondering how we could help Pedro to build a bigger heart for himself…