I wrote this at 36 weeks pregnant in 2019, I am now updating it, 2.5 years in as to what our experience was/is. Just a reminder if you are a heart mom, no two babies and no two hearts are the exact same. Your heart warrior will pave their own unique journey, even if their heart condition may seem very similar to another baby <3
I had this vision of what things would look like, how many days we would be in the hospital, trying to plan each surgery out, each hospital stay... and when things when better than the plan the high was HIGH but when things didn't go as planned, it felt like the ultimate failure. I have continued to work on taking things day by day, and on the hard days when things are changing so much, it's moment by moment.
I have put all updated text in this color and also note that I go in to MUCH more detail in the blog posts that were posted during that time :)
Hypoplastic Left Heart Syndrome (HLHS): 4 words we had never heard before, has entered us in to a whole new, scary world of unknowns. After about 15 hours of consulting with doctors the past couple weeks, Brandon and I could go on and on about the anatomy of the heart and what HLHS is- and to think, this is only the very beginning.
It took me awhile to write this post, because I don't like to drown in the reality and text book statistics of what we are about to face, even more than we have to already. But it was a post that I had to make....and fast. I am so thankful for the support we have received thus far. But to be frank, I am emotionally exhausted, mentally drained, full of raging hormones, and just super pregnant/uncomfortable- so truth is when people ask what HLHS is, if it's genetic or "what caused it" or compare it to that one time their friends friend had a heart murmur and they were fine.... I almost lose it. So this is my post to give you the very brief run down of what we know thus far about our Baby Noah's heart and the surgical intervention route that we have chosen to go. I will keep it super simple and add some links at the bottom to read more information!
This is a "normal" heart....4 chambers... 2 right... 2 left.. red and blue blood (oxygenated and deoxygenated) separated.
And this is a HLHS heart. Right Chambers are there but the left chambers are either non-existent, or underdeveloped and not functioning... oxygenated and deoxygenated blood is all mixed together and not being separated.
The Centers for Disease Control and Prevention (CDC) has estimated that each year about 960 babies in the United States are born with hypoplastic left heart syndrome (about 1 in every 4,344 babies).
So what does this mean?
The left side of the heart is unable to support the circulation needed by the body's organs, even though the right side of the heart is typically normally developed.
While the anatomy of HLHS is "not compatible with life" and typically fatal within the first few hours or days of life, surgical intervention is required shortly after birth. Every case is so different, as some children have other complex cardiac conditions in addition to HLHS, so it is difficult to know exactly what we are up against until he is here and we can see everything. Currently we know that Noah has MA/AA, Mitral Atresia/ Aortic Atresia with a moderate leaky tricuspid valve, and a decreased "squeeze" of heart as of 36 weeks.
Since we were prenatally diagnosed at our 20 week scan, we were given three options;
1. Termination- terminate the pregnancy completely. We said no to this one immediately.
2. Comfort Care- Essentially this is hospice. Either the baby's heart is too sick for surgery, or intervention is not the route of choice for the family. Although this route isn't completely out of the question for us, since we do not know everything, we prayed about this one a lot and ultimately chose the next option.
3. Surgical Intervention- if the baby qualifies; this is a series of three open heart surgeries for reconstruction- while it is not a life long cure, and some children still go in to heart failure, eventually needing a heart transplant. The surgeries are called: The Norwood, The Glenn, and the Fontan.
When Noah arrived on July 30th, 2019 he surprised everyone! He did have a procedure in the delivery room where they place an IV directly through the umbilical cord to administer medication to keep his PDA open. He was handling room air (did not require oxygen) for 6 days. After 6 days, he began to need more assistance. His color started to change and become more grey/purple and his respiratory rate was very high. He then received his Norwood at 8 days old.
Three Stage Surgical Intervention
The Norwood:
This is the first surgery and typically takes place a couple days- a week after birth. Surgeons essentially turns the right ventricle into the main ventricle pumping blood to both the lungs and the body.. which is a lot of work for just that right side to do, so it's very temporary. A connection called a shunt is placed between the pulmonary arteries and the aorta to supply blood to the lungs. Although this is a short explanation of the surgery, it is a very difficult and risky surgery, that can take anywhere between 6 - 10 hours and since it's a directly invasive surgery on the heart itself, these babies must be on full heart/lung bypass and have their bodies cooled to a near death state to protect the brain since they do not send blood to the brain during surgery. It is common that after surgery the chest remains open for a day or two, due to swelling. Then the babies enter "Interstage", this is the time frame between the Norwood and the Glenn Surgery. It's an extremely fragile and unstable time for these babies. Texas Children's has prepared us to be inpatient during this time, but there is a chance we could be released to go "home" (our temp place in Houston), and there the nurses would check in frequently and close monitoring is necessary.
Noah had the Norwood surgery at 8 days old. He was in the OR for about 12 hours. He completed the Norwood as well as a tricuspid valve repair to fix his leaky valve, this required them to completely stop Noah's heart. They did a few other adjustments, like widening his Pulmonary artery, etc.
We were reunited with our sweet boy and it was a very heart breaking sight that no mother and father could ever be prepared for. His chest completely open and his color was very purple, he remained intubated and on dialysis. I remember just staring at his heart beating through the clear dressing. Such a painful sight yet the most direct visual of an answered prayer; he was alive, his heart was beating.
Despite everything that was hooked up to our little 7 lb baby boy, the next few days went overall smooth. each day more and more lines were removed. I got to hold him 5 days post op for the first time. He did develop Chylous Effusion (I talk about in another post) and we got him taking bottles by mouth just a few weeks after surgery! We were discharged to go to our temporary apartment near the hospital just 40 days after surgery. This was a huge victory as we were prepared to be inpatient until post Glenn.
Interstage:
This is the timeframe between the Norwood and the second surgery, The Glenn. It's a fragile period and Texas Children's has prepared us that we will most likely be inpatient during this time and even if we do get released, we will have to stay within reach of TCH here in Houston. Daily, close monitoring is essential during this time as many things are "unstable".
A feeding tube is typically used to help the babies get all their nutrients as it is not uncommon that during the Norwood, the vocal cords are paralyzed.
Germs are a big no no. Any little sickness or cold can be life threatening to an infant in interstage- so visitors are limited during this time and mommy and daddy will be soaking up all the snuggles when we can.
The right ventricle has been doing all the work up until this point, pumping blood to both the body and the lungs, so it's time for the next surgery to relieve some of that stress.
Noah's Glenn was originally scheduled for December 27th, so the plan was to stay home, monitor extremely close, and have weekly check ups ( or more ).
Interstage was tough. It was going smooth at home and Noah seemed to be just a normal baby. He was eating my mouth which was very stressful as he could only take 1-2 ounces at a time for most feedings and we had a minimum amount of formula he must take each day.
We were required to take his weight daily, and chart all feeds, sats and heart rates which we would take in each week to go over it with the cardiologist. We went in to clinic weekly for echos and EKG to ensure we were not missing anything. Noah had a Sano Shunt placed during his Norwood. His weight gain and getting him to eat was a struggle at times. He was remaining stable until about 4 weeks in to interstage and during an echo we received the news that his function was decreasing (mild to moderately depressed) and his tricuspid valve leak was beginning to worsen (mild to moderate). Two days later he aspirated while taking a medication and couldn't recover, he turned purple and continued struggling to try to take a breath. Alone at the time, I scooped him up, rushed across the street to the hospital ER and they completed an echo where his heart was significantly worse (severe depressed function and severe TVR leak). We were then admitted and began evaluation for heart transplant as the team was unsure if he would be able to come off bypass if continuing with the plan to have the Glenn.
The Glenn:
Now it's time for the second open heart surgery. This usually takes place between 4-6 months of life; baby's body will tell us when he is ready for this one. This surgery takes some of the work the right ventricle has been doing, the shunt that was placed prior, is removed, and a direct connection is made between the pulmonary artery and the vessel returning oxygen- poor blood from the upper part of the body. This enables blood returning from the body to flow directly to the lungs.
This can be another lengthy surgery, with a long list of risks, but typically recovery can be a little easier and once fully recovered, baby and family are released to go HOME. Like HOME HOME, away from the hospital!! Monitoring and daily medications are usually still needed during this time, until the next surgery. But, we would be able to move back to Austin and be in our home with our Baby Noah. From speaking with other families, this is like the Honeymoon stage, it's the light that you begin to see at the end of the tunnel.
Of course, this seems like such a dream, that's far away right now- but we are hopeful.
This is where our journey threw a huge curve ball at us and things got extremely tough. "Most" HLHS stories that I heard, the Glenn was exactly what their baby needed. There bodies recovered quickly, the hospital stay was short and then they were home enjoying the "honeymoon stage".
Because Noah's function was so depressed, we weren't sure how his heart would handle another massive open heart surgery. At this point I couldnt even feed him with out him becoming tachy and going in to distress.
Going in to the Glenn, it was 50/50, which was terrifying. We started the transplant evaluation process which required lots of blood from Noah, and myself. Meeting with the transplant team, and a long list of other requirements.
Noah went in for his Glenn November 14th. It was a long 10 hours day as we waited for updates. We were surrounded by family and friends. Brandon, my mom and I were able to see Noah in recovery, closed chest and he looked so much better than I was expecting (comparing to the Norwood). But just a couple hours alter and Noah took a terrible turn, he started desatting and losing blood very fast. His sats were getting in to the 30s and the team was unable to keep up with the amount of blood he was losing. The team had to do compressions to get him stable, He was rushed back to the OR in the middle of the night where they had to reopen his chest and address a hemothorax. It was a terrfying night and made for a very long recovery. The next few weeks were some of the hardest days and weeks. Noah had a coding episode and had to be emergently intubated, he had a paralyzed diaphragm, chylous effusion, and we had multiple failed attempts of getting him off milrinone. By the Grace of God he was successfully weaned of milrinone on Christmas Day. His heart function was still not great, and he did end up getting a Gtube after developing an oral aversion. Three and a half months after surgery, we were able to take our baby boy to our home in Austin for the very first time! Although it was a lot, with over 35 doses of meds a day/night and attached to a feeding pump all day/night- we were so happy to just be home with our sweet boy. Then two weeks later COVID HIT!
The Fontan:
This is Noah's next surgery on the horizon. At this point we are not sure that he will qualify for the Fontan due to his function and leak.
His heart function remains very depressed with upper moderate/severe Tricuspid valve regurgitation. We are currently just waiting and still having every 3 month cardio visits until we schedule a heart cath. If the team does not think this is best for Noah, then we will take the steps to get him on the Heart Transplant List.
This is the third stage that usually takes place around 3-4 years of age. It connects the blood vessel that drains deoxygenated blood from the lower part of the body into the heart, to the pulmonary artery by creating a channel through or right outside the heart to direct blood to the pulmonary artery. Up until now, the blood low in oxygen from the lower part of the body has mixed with blood high in oxygen. After this stage, blood high in oxygen and blood low in oxygen are separated meaning more oxygen can get to the body!
Once these surgeries are completed, these babies are not "cured", and they can have lifelong complications.
Of course there are so many unknowns on how Noah will handle his heart anatomy when he is not in utero and how he will recover from the surgeries. We are blessed that we have caught this prenatally, as it's allowed us time to prepare, move to Texas Children's, and give Noah the best shot at life that we can. Only 20 more days until we meet baby boy, unless he makes his debut a little earlier.
As of now, I am starting to dilate. We also found out last week that Noah's heart "squeeze" has decreased in function and his Tricuspid Valve is moderately leaky (from mild). This was not news we wanted to hear, but we are continuing to pray everyday and I am enjoying the kicks and punches he is giving me letting me know he's okay!
More Resources:
How generous of you to take the time and care to help others understand.
Hoping hard for the best of outcomes Lynn
Hi sweetie thanks for this incredibly layman description!! Always sending you healing loving prayers with the Most positive outcome imaginable!! Noah is one very lucky little guy to have you both as his parents!! Xoxo Stacy