The only word I can use to describe this week is rollercoaster. It has been the fastest, most wild and most disappointing rollercoaster we’ve been on thus far.
Since 17 weeks, we’ve had the news that our baby boy’s critical aortic stenosis had rendered his left ventricle dilated, scarred and severely dysfunctional. His aorta is tiny, and he is not a candidate for any surgical intervention both now in the womb and also at birth. His leaky aortic and mitral valve were absolute contraindications for the palliative surgeries, and a dilated left ventricle complicates the Norwood procedure and decreases its chances of success. The Norwood procedure is the first of three “band aids” they perform on babies born with Hypoplastic Left Heart Syndrome, and happens to be the most complicated one with the highest mortality rate. Until very recently, baby G did not have a diagnosis of hypoplastic left heart syndrome (HLHS), as his left side of his heart was not hypo plastic, but instead dilated.
At our last echocardiogram, we discovered the mitral valve and the aortic valve were no longer leaking and that the aorta was even smaller in comparison to the pulmonary artery. The cardiologist mentioned that our case was discussed during their grand rounds, and mentioned in passing about the Norwood again, but that she wasn’t sure baby G was a candidate and if he was, he would certainly be a high risk one because of the other cardiac comorbidities that he has.
When I showed up to my regular MFM appointment on Monday, she explained again that our case was discussed the previous week among all the disciplines involved in our care. She told us that several of the cardiologists on the panel felt strongly that our baby’s condition had evolved into a HLHS, which in some cases is “fixable” with a success rate of up to 85%. WHAT. She explained that she was feeling like we were lead down a very specific path where we had chosen a palliative route, due to the initial information about G’s heart, but that his condition had evolved and it was possible that this was something that surgeons could, in fact, fix. We could potentially bring our baby home with us. She strongly urged us to seek a second opinion with a congenital pediatric cardiac surgeon at St. Joseph’s Hospital in Tampa. I left that appointment in total shock and emotionally drained thinking about the possibility of taking a baby we have completely prepared to lose, home with us.
On Tuesday, I called the surgeons office to see when they’d be able to see us since I’m 32 weeks pregnant. If we were going to completely veer off the course we’ve prepared for, time was of the essence and this ball needed to get rolling. They already had all of our images and information and asked if we could come in on Wednesday at 10am. I said yes, and the appointment was set.
Wednesday came and off we went to the surgeons office. We met with him and his PA and discussed the course of the pregnancy and relayed the information we knew. He had reviewed our images from our last echo, as well as all the office notes. He spoke a little bit about HLHS, and the surgery, but didn’t want to go into too much detail because he wanted us to go and have another echo and consult with one of the perinatal cardiologists at St. Joe’s. He asked if we could go over right then. Of course, we said yes.
The MFM nurse navigator met us after the appointment to discuss more about the processes at St Joes, and shared with us about her personal and professional experiences with congenital heart malformations, and how these things are handled at their facility. We toured the PCICU. She set the echo appointment up and we headed over there. When it was complete, we met one of the perinatal cardiologists and discussed in depth his findings on the echo. I guess since we had just had one two weeks prior I wasn’t expecting any changes, but considered we’ve never had an echo without a new finding I shouldn’t have been surprised.
First of all, the left ventricle was no longer big and dilated, but had shrunk significantly. This, he explained, is the natural course of malformations that lead to HLHS. Initially, the LV dilated and calcifies, and eventually it does shrivel up and become hypoplastic. The surgeon had explained that a hugely dilated left ventricle would make the Norwood a bad idea, and that his understanding from speaking with Dr. Gupta and reviewing our images was that baby G’s LV was still large. So, I guess this was “good” news? This particular doctor clearly likes to deliver good news first, because he went through HLHS in detail, explained the three surgeries performed on these babies and their success rates, expected hospital stays, etc. St Joe’s has been able to cut the length of stay significantly (which, for those of you who are not medical people, is really really good).
And then, the sucker punch. Our baby had an official diagnosis of Hypoplastic Left Heart Syndrome, which according to our MFM does not generally lead to the decision for palliative care, at least not right away. But our baby had also developed a severely restricted (but more than likely) intact atrial septum. It’s incredibly complicated, but to put it in basic terms, all fetuses have two holes in their heart, a PDA and a PFO. Because oxygen is delivered to the baby through the umbilical cord (and not the lungs), these holes help to ensure the baby bypasses the lungs, and in a baby with HLHS, the PFO specifically (found between the top two chambers of the heart) is essential. It is essential that it is open, so that blood can bypass the nonfunctional left side of the heart and go to the right side to be pumped out. The closing of this hole was a new finding and one that does not have favorable outcomes. As soon as the baby is cut off from the placenta (its oxygen source) at birth, the baby loses the ability to pump oxygen rich blood to the body. This would not be the case if his FO remained open, as there are medications to keep the holes open until HLHS babies undergo the Norwood procedure. Additionally, with the hole now closed, increased pressure on the pulmonary vasculature is created because there is no transfer of blood from one side to the other to skip the nonfunctioning lungs. These kiddos often end up with severe pulmonary hypertension and thus severe lung issues. There would be less than a 50% chance he’d make it to 1 year old, and that’s if he survived the immediate-upon-birth cardiac catheterization to puncture a hole between the top part of his heart and introduce a stent in there to create the flow of blood to the right atrium. AND if he survived the Norwood.
They explained that my delivery would look something like this: a c-section at 38 weeks in an adult cardiac operating suite at 8am. 30+ people would be in the room and prepared to immediately bring baby into either the pediatric cardiac operating suite or the pediatric cardiac Cath lab, both of which are adjoining to the operating suite I’d be delivering in. They’d immediately attempt the atrial septoplasty and he would be brought to the PCICU with an attempt to stabilize him. A few days later, if stable, he would potentially undergo the Norwood procedure which has a mortality rate of 5% in “simple” HLHS cases, but of course is much higher in our case. Long term outcomes, he reiterated, were not great. Kiddos often developed severe lung issues even if all interventions on the heart were successful. Many kids also end up needing a heart transplant, which in itself is very temporary as they do not last forever. He explained that a baby who receives a heart transplant could potentially require three more before they’re 25 years old. These babies are incredibly fragile, even without transplant, and a common cold virus often earns them a stay in the ICU. With severe lung issues likely, an ICU stay where baby requires intubation, may prove to be too much for him. And most of all, the likelihood of him having a good quality and “normal” life, though not zero, would be very low if he were to survive all the surgeries and overcome all the obstacles. AND I would have to make it to 38 weeks which seems unlikely at this point considering my amniotic fluid is already off the charts high.
So in a matter of three days, we had a rock solid palliative care plan, which turned into a glimmer of hope that our baby may survive this, to meeting a bunch of new professionals, just to find out that yet another complication had arisen with baby G’s heart. To say it’s been overwhelming is an understatement. Neither Barry nor I got too excited about the prospect of our plan changing, and maybe it was self preservation because we both had a feeling it wouldn’t turn out the way that we both desperately wanted it to. At the end of every day, we both wish so badly that we weren’t faced with this situation. We wish everyday that things were different and that our baby would be coming home with us. We don’t want to feel any guilt when all is said and done about the direction we go in and while there is a great argument on both sides of the fence, we have agreed that there is still only one decision that is best for our family. As soon as baby is born and the cord is cut, there will not be any oxygen rich blood circulating to his body. We will lose him within hours. There is no uncertainty in that. It’s the first piece of concrete information we’ve received on this journey, in fact. If he is born alive, and he has a mere hours to live, and those hours are hooked up to machines in a different room than me, I don’t think I could ever forgive myself for allowing his only time on Earth with strangers and not with us. And the guilt we’d feel if we put him through countless surgeries, put him on countless medications, only to be here on Earth with no quality of life or ability to enjoy it, would far surpass the potential guilt of letting him slip away peacefully in our arms.