Many of you have asked what this next surgery will entail. As promised, I have included some information over viewing HLHS and the surgeries involved. I have also included some pictures to illustrate. This is a compilation of information we've received from our cardiologist and surgeon as well as articles we've researched. I have also included a brief update at the end as well.



Hypoplastic Left Heart Syndrome (HLHS) is a congenital heart defect in which the left side of the heart is severely underdeveloped.

If part of the endocardial tube gets pinched shut in a region that becomes the future ventricle, hypoplastic heart syndrome will occur. If the pinched part of the endocardial tube is the bulbus-cordis region of the developing heart, hypoplastic RIGHT syndrome will occur. If it is in the ventricle region it will be the LEFT side that is hypoplastic.
There is evidence associating it with Gap junction protein, alpha 1(This is a genetic link).


Presentation
In babies with HLHS, the aorta and left ventricle are very small, and the aortic and mitral valves are either too small to allow sufficient blood flow or are atretic (closed) altogether. As blood returns from the lungs to the left atrium, it must pass through an atrial septal defect to the right side of the heart.


In a healthy human, the left side of the heart receives oxygen-rich blood from the lungs and pumps it out to the rest of the body; with these structures underdeveloped, they cannot circulate blood to other organs, and the right ventricle must pump blood to both the lungs, as it would normally, and to the rest of the body, a situation which cannot be sustained for long.


In cases of HLHS, the right side of the heart often must pump blood to the body through a patent ductus arteriosus. As the ductus arteriosus usually closes within eleven days after birth, blood flow is severely restricted and eventually cutoff, leading to dangerously low circulation and eventually to shock.


Treatment
Without life-prolonging interventions, HLHS is fatal, but with intervention, an infant may survive. A pediatric cardiothoracic surgeon may perform a series of operations or a full heart transplant. In the meantime, the ductus may be kept open to allow blood-flow using medication containing prostaglandin. Because these operations are complex and need to be individualized for each patient, a cardiologist must assess all medical and surgical options on a case-by-case basis.


Currently, infants who undergo either the staged reconstructive surgery (Norwood procedure almost immediately after birth, Glenn procedure at 3 to 6 months of age, and the Fontan procedure at 3 to 5 years of age) or who undergo cardiac transplantation have a 5-year survival of approximately 50-60% (we have seen some research showing survival rates as high as 75%; much of this depends on where the surgery takes place). Further, studies show that about 50% of those children who survive surgery have developmental delay or need special education. About 25% of surgical survivors have severe disabilities.


The traditional three-stage procedure is a palliative procedure (not a cure), as the child's circulation is made to work with only two of the heart's four chambers.




The first step is the Norwood procedure. In this procedure, the right ventricle is used to pump blood into the systemic circulation. Since the right ventricle is no longer directly pumping blood to the lungs, a shunt is required in order to pass deoxygenated blood through the lungs. Either the subclavian artery can be connected to the pulmonary circulation (Blalock-Taussig shunt), or a shunt is made directly from the right ventricle to the pulmonary circulation (Sano shunt). [Olivia had The Sano Shunt.] The narrow aorta is enlarged using a patch to improve blood-flow to the body.


During this time the baby may be medically fragile and have feeding problems because the heart is working very hard. There is a considerable degree of venous mixing in the right ventricle, leading to lower oxygenation saturations. In addition, the Blalock-Taussig shunt and the Sano shunt both expose the lungs to systemic arterial pressures, leading in the long term to pulmonary hypertension and eventually to heart failure.




The second stage, the bi-directional Glenn procedure or Hemi-Fontan (see also Kawashima procedure) relieves some of the above problems. In this operation, the superior vena cava is ligated from the heart and connected to the pulmonary circulation. At this time, the Blalock-Taussig or Sano shunt is taken down. At this point, the lungs are no longer exposed to systemic arterial pressures, but much lower venous pressures. Although venous blood from the upper half of the body is no longer mixing with oxygenated blood in the right ventricle, there is still venous mixing from the lower half of the body, leading to some degree of oxygen desaturation.


During this time the child may have improved quality of life as the heart does not have to work as hard.



The final procedure, the Fontan (Fontan procedure) completes the repair of the hypoplastic left heart. Although there are several variations, the functional effect is to redirect venous blood from the lower body (through the inferior vena cava) away from the right atrium to the pulmonary artery. Now, there should not be any mixing of oxygenated and deoxygenated blood in the right ventricle. The right ventricle performs the traditional job of the left, supplying the body with oxygenated blood, while the passive systemic venous pressure performs the traditional job of the right, passing deoxygenated blood to the lungs.


The Norwood Procedure is generally performed within a week of birth, the second stage at 3–6 months of age, and the Fontan at 18 months to four years of age. There are two types of Fontan: the Lateral Tunnel Fontan, and the Extracardiac Fontan. When the Fontan Procedure was first being done for children with HLHS, the only Fontan was the Lateral Tunnel Fontan. This requires actual cutting in the heart itself to create a "tunnel" by which the blood can travel passively to the lungs. Within the last decade, doctors have created an Extracardiac Fontan. This operation creates a tunnel outside the heart itself which reduces the chances of Fontan patients developing scar tissue on the heart which might later cause arrhythmias. At this time it is believed Olivia will have the Extracardiac Fontan.


Prognosis
While infants successfully treated for HLHS have a good chance of survival, they may experience chronic health problems for the rest of their lives. The 3-stage surgeries were developed in the early 1980s with no survivors prior to that time. Therefore, the earliest survivors are in their mid 20s and the long term prognosis is unknown. However, the advances in surgical and medical techniques have helped increase the survival rate dramatically since the surgeries were first developed.


As is true for patients with other types of heart defects involving malformed valves HLHS patients run a high risk of endocarditis, and must be monitored by a cardiologist for the rest of their lives to check on their heart function.


The three stage Norwood procedure only increases the life of the heart as HLHS cannot be cured. The child may need a heart transplant at some point in the child's lifetime.
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Well, we have officially entered the 2 ½ week mark until surgery. This also means we are now quarantined. Over the past week we have been very careful where we go and who comes around, but we have still allowed for a bit of fun while we still can. Olivia has enjoyed a lot of time outside with the nice weather we've been having.




Vroom...vroom!



Thomas has also enjoyed some time outside. This is a picture of his very first trip outdoors!




The other day I came outside to tell Steven and Olivia it was time for supper. This is what I found! No wonder she loves playing outside with Daddy!




We've also had plenty of time inside doing crafts, playing with water in the sink, and hanging with family.

Hangin' with Gramps


Playing with our doll house.




We also had a surprise birthday party for Grandma Lynch. Miss Olivia is quite the social butterfly, so she loves helping me plan parties, get everything ready, and entertain our guests.




Thomas has been doing great. He started rolling over a few weeks ago and can roll all over the floor now! He smiles all the time and is just a big sweetie-pie! I call him, Mr. Happy-Go-Lucky. It's hard to believe he will be 6 months old in a few weeks. Since we aren't going to be home, I went ahead and snapped a few pics while we were playing the other day.











Thankfully, Thomas is so laid back, it's going to make getting passed around the next month a little easier on everyone. However, I feel terrible about it. I had thought about having him stay at the hospital with us, but we decided it wouldn't be fair to him or Olivia. I'm still going to have him stay a few nights, but the other nights one of his Grandmas will have him. It's a lot different having to worry about another little one at home as well.




I love this picture...just the way they are so lovingly starring up at one another....It melts my heart.

Many of you have asked how we are holding up. I can't speak for everyone else, but to be completely honest...I'm really not doing so well. I'm usually the one that eventually turns my worries over to God and lets my body go into auto pilot after that. It's gotten me throught the last two surgeries. But, to be painfully honest, I am having a hard time turning over my worries. Every single day my biggest fears swell up in my throat until it is almost too much to bare. I look at my sweet baby girl, trying to memorize every tiny detail. The sound of her laugh, her beautiful blue eyes, the smell of her hair...I am just so afraid...afraid that in two weeks my biggest fears could become a reality. Please pray for us. Please pray the doctors and nurses have all the knowledge they need in succeeding with Olivia's surgery and keeping her as safe and healthy as possible. Please pray for Steve and I as well as the rest of our family. This was tough to do the first two times, but I think this is going to be the toughest by far.