It’s a Date

We met with Patrick’s cardiothoracic surgeon, Dr. Nigro, and Beth on Friday to discuss surgery. We were relieved to find out that Patrick’s surgery is not an emergency and can wait until after the holidays. Patrick is scheduled for a tricuspid valve repair and possible Glenn on January 6th, 2011.

The valve repair requires open-heart surgery. Once they look at and work on the tricuspid valve they will decide if Patrick also needs the Glenn Procedure to take some pressure off of the right side of his heart. The Glenn would connect the superior vena cava (the vessel that brings blood from the upper body to the heart) directly to the pulmonary artery (which normally takes oxygen-poor blood from the heart to the lungs), skipping the right side of the heart entirely.

Once Patrick has gone under anesthesia, he will get a breathing tube and an echo tube. They will make an incision in his chest and open the bone.

He will be on a heart-lung machine–his heart will be asleep while they assess and repair the valve as well as possible. Then they will decide if we need the Glenn. They will wake up Patrick’s heart and, if the Glenn is needed, they will do that procedure. They will also patch up the ASD (hole between the right and left atriums), although they may leave a little bit of a hole to relieve pressure.

Patrick will be in the PCTICU for 7-10 days. Recovery is straight-forward after he leaves the hospital. They expect him to rebound quickly. Risks are minimal–Dr. Nigro estimated a risk factor of 1-2% for major complications. Patrick will need a blood transfusion, so Braden and I had bloodwork done to see if we are a match to donate. He’ll need 3 units of FFP & platelets. We are waiting for the hospital to contact us with the results of our bloodwork, and to let us know what Patrick’s blood type is.

We feel very blessed to not be spending the holidays at the hospital. AND we were given clearance to drive to Southern California for Christmas! We are looking forward to taking our mind off things for a bit as we vacation both in California and then with my side of the family who are visiting Arizona after Christmas. Patrick has many aunts, uncles, cousins and a grandpa to meet!

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Second Opinion

It’s easy to forget that something as serious as a heart defect can be pretty subjective. Especially when observation of the problem can only be done via ultrasound. But depending on their experiences and what they see, the experts seem to come away with different assessments and expectations. Happily the surgeon, Dr. Nigro, and Beth, our fantastic Cardiac Nurse Practitioner, immediately told us this morning that they are pleased with where Pedro is at.

Even before saying hello almost, they reassured us that Pedro’s heart has only increased in size by about 10% over the last few months that they’ve been watching. While it is definitely enlarged, they estimate that it is only taking up about 52% of his chest cavity, which means his lungs have not been inhibited in their development. They anticipate that he won’t have the major breathing problems that some heart babies do at birth. AND they don’t think he will need surgery immediately. Their preference, in any case, will be to try everything else first to stabilize Pedro at birth and let him grow a little older before surgery.  Even if he is struggling, hopefully he can “eek by” until he is bigger and stronger. (Yes, the surgeon said “eek”).

In any event, we have a better idea of what to expect. Delivery should be normal, with a little extra monitoring, and just like other babies Pedro will be cleaned up and checked right there in the labor & delivery room (under a warmer). Then they’ll bundle him up for us to hold him! If he is doing okay, we should get a little bit of time with him before Braden goes with him to the NICU (which is on the same floor as L&D). He will be in the regular NICU for about 24 hours or so and then he’ll be transferred to the PCICU (Pediatric Cardiothoracic ICU). He’ll be in the PCICU about a week or week and a half. IF he does end up needing surgery right away it will be within the first week. In that case he’ll be in the PCICU for a few more weeks than he would otherwise.

Reality Check

We have been getting so excited for Pedro to arrive, and our preparations and anticipation distract us from worrying too much. Afterall, we really won’t know what will be needed until shortly after he is born. So, we are optimistic. Sometimes being optimistic makes it somewhat bitter to swallow any news other than “things look the same” or “he’s doing fine”. We find ourselves secretly hoping that one of these days the doctors will pronounce Pedro’s heart miraculously healed or something.  I know, I know, in denial much?

We made a visit to St. Joe’s this morning for a fetal echo and consult with Dr. Diab. As before, they could see that Pedro’s tricuspid valve is leaking significantly and that his heart is enlarged. Dr. Diab indicated that Pedro’s heart fills (or will soon fill) the majority of his chest cavity. Dr. Diab suspects that Pedro will need immediate surgery to put a shunt in, but it will all depend on how things function once he’s born. Apparently one of the benefits of the shunt would be that his heart would rapidly shrink down and be a closer-to-normal size. I believe the main purpose of the shunt, though, would be to make sure that enough blood can get to the lungs.

On Friday we will meet with the surgeon, Dr. Nigro, again, so we’ll have a chance to learn more about what would be entailed in the shunt procedure.

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.

“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.