Notes from appointment with Dr.Heller

This meeting went EXTREMELY well. It could not have gone better unless they had told us that they goofed and her heart was perfect.

Best guess is that the right side of her heart is Hypoplastic. This is good because the left ventricle is the pumping chamber.

Her hole is closing – it’s smaller than in the first ultrasound.

Why / How this happened:

The valves developed abnormally. Instead of two small valves (one on each side of the heart) Olivia has one large valve – diagnosis is Unbalanced AV Canal.

They may not have to do anything to the valve considering the hypoplastic right side of her heart.

The Aorta is most likely the large artery we’re seeing in the U/S, which is beneficial (hard to create the aorta artery). The pulmonary artery has “Atresia” – it’s small and possibly will be completely closed or sealed over before birth.

Her heart is not true dextrocardia. It’s angled a bit to the right but not extreme.

Due to the angle of her heart we need to be conscience of spleen misdevelopment. It may not have developed properly and if that’s the case there is a vaccine that can be given along with preventative antibiotics taken daily.

When she is born, all the blood in her body will be “mixed” – as in the oxygen enriched blood will be mixing with the oxygen poor blood. This is ok short term, but the goal of all these surgeries is to separate them.

Delivery of Olivia:

For Olivia’s best interest, I will be most likely induced two weeks prior to my due date at Hartford Hospital – we’ll be looking at approximately July 15th (TBD). Induction is so that we guarantee Dr. Mello (the pediatric surgeon) will be in town and available. Hartford Hospital is connected to CCMC (Connecticut Children’s Medical Center) so for Olivia’s benefit, that’s where this should be.

There is no reason I cannot have another vaginal delivery. The determining factors for a c-section would be the same as any other delivery.

If Olivia is born feisty and breathing well and crying loud, they most likely will give us a few minutes to hold her before whisking her away. Dr. Heller warned us that the pediatric team will be “nervous” to take her as they want to get her settled in the NICU (neonatal intensive care unit) as soon as possible.

When she is brought to the NICU, they will insert an IV into her belly button “an umbilical venous line”. This will allow them to administer the PGE1 medicine to keeping her PDA valve open. PDA valve is a valve that allows the baby’s blood to flow differently when in utero vs. after birth when normally it is closed – Olivia’s will be kept open using medications until the first surgery takes place. This medicine is a continuous infusion, meaning she has to be on it at ALL times until the surgery.

There are side effects to the PGE1 medicine: it makes the babies jittery; they sometimes have a higher temperature and it can cause apnea. Based on how much apnea she exhibits, she may or may not require a ventilator. Ideally, she wouldn’t have one so we can concentrate on feeding before surgery.

After she’s all settled in the NICU, the pediatric cardiologist on call will come in and do an ultrasound on Olivia. This will help them to confidently define what we’re definitely dealing with. They’ll also need to determine where her pulmonary veins are so when they operate they’ll be able to avoid them. The pulmonary veins are those that return the blood from the lungs back to the heart.

After anatomy is defined, we’ll sit down again and set up a plan of attack.

They like to wait 24-48 hours at LEAST after birth but it could be 1-2 weeks after to have surgery. They will want me to start nursing her prior to surgery so she won’t lose the sucking reflex and have to relearn it again.

Her oxygen levels may be “low” after birth.

Surgeries:

Due to the diagnosis of Unbalanced AV Canal and Pulmonary Atresia, the first stage of surgery is much less complicated and less risky than a standard HLHS or HRHS surgery. First surgery will be placement of a “Central Shunt”

connecting the aorta to the pulmonary veins – allowing blood to reach the lungs. They will use gortex material (waterproofing material used to make rain jackets) to create this shunt. Surgery is approximately 4-5 hours and the PDA valve will be removed at this time.

CCMC totes a very high 90’s% success rate with the shunt. Olivia will have to be on a low dose of baby aspirin to prevent clotting within the shunt.

After surgery we can expect 80-85% oxygen levels.

The second surgery is determined based on Olivia’s oxygen levels. When they start to drop into the low 70’s it’s an indicator that the shunt is too small to provide enough blood to her growing heart and we need to look at the next step.

The eventual goal is to sort the mixed blood into red blood and blue blood.

The Glenn procedure is generally done at 4-6 months of age. But ultimately Olivia will decide when it’s necessary. The Glenn is to work on sorting the mixed blood. The surgeon will take the upper half of the body’s blue blood and send it directly to her lungs. At this point the shunt is removed. We should expect some swelling in the upper half of her body after the surgery.

The Fontan procedure is generally done at 3 years of age. But it’s more based on the size of the baby. The goal of the Fontan is to take the lower half of the body’s returning blue blood and send it to the lungs. They add an external (to the heart) conduit tube at this point.

If Olivia is anything like Maddie Rose, she will be not having this procedure until she is closer to 4 ;) as Maddie Rose is STILL not 35 lbs.

Heart / Lung Bypass is required for both the Glenn and the Fontan procedures.

Long term after the Fontan:

  • Pregnancy will be an issue for Olivia. She may not be able to carry her own children.
  • Activity levels: she will be similar or better to an asthmatic child.
  • Prior to the Fontan, when she gets upset or works hard, she will look “bluer” than the average child. After, she will be nice and “pink”.
  • Endurance will be an issue: she won’t be able to run a marathon or play varsity sports. But the key is she will be the one who limits herself. We shouldn’t push her once she is saying, “I’m tired”. She will be able to participate in swimming and sports, but the teachers have to honor her “I’m tired” as being the word – and not push her beyond that.

Insurance:

  • This morning I called over to the insurance company. Gave them Olivia’s diagnosis and what surgeries she will need. Because technically Olivia isn’t on the policy, they can’t “guarantee” coverage, but this is what they said “If you were to have a baby born with a congenital heart defect, any of the surgeries you mentioned would be covered as they are medically necessary and required for her life.”

What’s next:

  • We are going to schedule a consultation with Dr. Mello – surgeon at CCMC.
  • We are going to tour the CCMC intensive care units along with the operating rooms
  • I have contacted Dr. Cluett’s office regarding coordinating with the Fetal Maternity group at Hartford Hospital for continuing Level II ultrasounds, etc. Rachel (nurse) is out of the office today but will be back on Monday.
  • I am going to take VERY good care of myself to hopefully prevent any complications that could cause Olivia to come too early.