I’d almost forgotten what a “normal” appointment felt like. Today was one of the first appointments we’ve had since February where I smiled from start to finish.

The u/s technician was wonderful. Went over everything with us. Didn’t focus on the negatives at all. Kept commenting on how cute Olivia was.

What we saw:

• Olivia’s bladder was full which means the kidneys are functional and “good”.
• The umbilical cord looks fabulous. We were able to see all three vessels that make it up. In some of the pictures we have it looks like there are bubbles in the fluid – which is actually a cross section of the umbilical cord.
• Olivia is head down!
• The placenta is of good quality (no signs of aging which can lead to early labor). The amount of amniotic fluid was also good.
• Stomach was where it should be but angled a bit.
• No signs of the spleen, but it could be the type of machine they are using.
• Diaphragm looked fabulous.

Overall:

• Olivia weighs approximately 2lbs, 4 oz and is in the 50th percentile. Makes sense based on her older sister being a peanut as well.
• Pictures, pictures, pictures! We were given a ton of pictures to take home with us. Arnie will scan them for me tonight. Olivia looks just like Maddie in my opinion. She’s starting to look “chunky” and more baby and less “alien”
• My diabetes test came back perfectly normal! I was well within the normal range (right in the middle) so that is such a blessing.

Regarding scheduling the tour of the NICU. I will be hearing from Kathy Socha in the next month or so once the physician schedule for the NICU has been determined for July.

Dr. Cortland just called me to give me some updates on everything.

She just spoke with Dr. Renee Babrowski who is a perinatologist at Hartford Hospital. Based on our concerns about the heterotaxy, they are going to talk to Radiology at Hartford Hospital and possibly set up a Fetal MRI for Olivia to get a good look at her spleen.

Dr. Cortland is also going to setup a growth scan (u/s) to be scheduled within the next week.

I should be hearing from Kathy Socha from the NICU at Hartford Hospital in the next day or two to setup a consultation with a neonatologist and a tour of the NICU.

The appointment went very well. Olivia’s heartbeat was 150 bpm. My blood pressure was “very good”. Weight was the same as when I saw Dr. Cluett the week prior. Dr. Cortland did an internal and there are no problems with my cervix.

Discussions:

I requested we have another Level II ultrasound to verify the heterotaxy syndrome and determine what organs have been affected. We would like to know how her spleen is to determine if we’re facing antibiotics for life. Not anything we’re losing sleep over, but just to be as prepared as we possibly can be, given the situation.

Dr. Cortland stated that based on Olivia’s diagnosis she would recommend an induction be scheduled somewhere around 38 to 39 weeks. They like these deliveries to be “controlled”.

Dr. Cortland is in a group practice with 5 other doctors. We talked about how I felt about rotating through to meet everyone. But at this point, being 25 weeks and knowing I’m going to be induced by Dr. Cortland, I don’t see the need to meet everyone.

I am going to be having the glucose (diabetes) test in the next few weeks. I have the paperwork and just need to contact the lab for their hours.

I received a copy of this letter today from Dr. Cluett’s office. I found it very positive. There are a few things I need to do more research on (mainly protein losing enteropathy and palliation) but the rest is very easy for us to understand at this point. Note: we went to the appointment with my mother, not Arnie’s, but I’m typing it as she sent it without corrections.

March 25, 2004

Dear Doctor Cluett:

Your patient, Tricia B, was seen in my office today for a family meeting regarding her fetus with congenital heart disease. She was accompanied today by her husband and mother-in-law. As you know, Tricia’s pregnancy was complicated by parvovirus exposure. An 18 week ultrasound demonstrated possible hypoplastic left heart syndrome. Amniocentesis demonstrates normal fetal chromosomes. The family had absolutely no interest in considering termination. A follow-up ultrasound was done on March 16th at 21 weeks gestation. I reviewed that study at our recent fetal echo conference. I felt that the anatomy was more consistent with an unbalanced type of AV canal defect rather than a true hypoplastic left heart. There was mostly mesocardia (perhaps slight dextrocardia). There was a very small right-sided morphologic left ventricle and a large left sided right ventricle consistent with ventricular inversion. There was only one large great vessel appearing to arise anteriorly off the right ventricle. This had the appearance of a typical aorta (no pulmonary branching noted). There appeared to be a much smaller pulmonary artery which was likely atretic located more medically indicating that the great vessels are transposed but it still appeared to arise from the large right ventricle. There appears to be probably retrograde filling of that small pulmonary artery from the ductus. There was an inlet type of ventricular septal defect. There was no evidence of AV valve regurgitation. In addition to the cardiac abnormalities, the gallbladder was noted on the left side as well as the stomach bubble. This suggests probable heterotaxy syndrome.

Discussion: Tricia’s fetus has a complex cardiac abnormality which at this time appears to be an unbalanced type of AV canal defect with pulmonary atresia. This is probably part of a heterotaxy syndrome. I explained to the family that this means there could be polysplenia or asplenia, gut malrotation, and anomalies of the systemic and pulmonary veins. These will all be evaluated after delivery. I spent the majority of the time discussing our three-staged approach to single ventricle repair. This infant will likely be ductal dependent at birth and will go immediately to the NICU for prostoglandin infusion. The baby will likely need some sort of central shunt (aorto-pulmonary shunt) created to insure adequate pulmonary blood flow. We would then plan on proceeding with a Glenn procedure to be done somewhere around 4 to 6 months of age and ultimately undergo a Fontan operation at around 2 to 3 years of age. The baby would have chronic cyanosis until after the Fontan was completed and then she would still end up with palliation, not a normal heart. However, the long-term functional status of most of our Fontan patients is relatively good. They often do have some exercise limitations and there are, of course, long-term risks such as protein losing enteropathy, but they can anticipate a relatively normal lifestyle. I indicated that the survival rate with each of the three stages of surgery was probably in the range of 90+%. Early on many of these babies have feeding difficulties and require some chronic cardiac medication.

I talked to the B’s about what to expect in terms of length of hospital stay and the anticipated neonatal course. I encouraged them to set up a meeting with Dr. Mello prior to delivery as well as to arrange a tour of the hospital. We talked briefly about the risk of recurrence for future pregnancies. I indicated that it would be necessary for the baby to be delivered here in Hartford, probably by scheduled induction and she will, therefore, need to establish care with the maternal fetal medicine group here. However she can continue to receive the majority of her routine prenatal care with you locally.

Although this is certainly a complicated cardiac problem, I was able to be reasonably optimistic about the baby’s chances for long-term survival. The family seemed to have an excellent understanding of the situation and they seemed relieved by much of the discussion today. They have already been in touch with the Little Hearts support group. They have requested that I continue to be the baby’s cardiologist after delivery. I will be happy to do so. It has been a pleasure meeting this lovely family and I encouraged them to call with any questions or concerns between now and delivery.

Sincerely,

Felice Heller, MD

CC: Sari Friedman, MD (pediatrician)

Renee Babrowski, MD

Appointment went very well. Blood pressure 114/60. Olivia’s heartbeat was 145 bpm. I am measuring 26 cm.

We discussed induction at Hartford Hospital. Dr. Cluett has delivery rights there, but 99.99% of her patients deliver at Bristol Hospital. As she’s not in a group practice, it would be complicated for her to be in both places at once. Based on this, she feels it would be best for the Fetal Maternity Group up at Hartford Hospital to handle my delivery. But Dr. Cluett is willing to handle all weight / blood pressure checkups in between.

Rachel will schedule an appointment for me and let me know when.

We discussed the Sibling class for Maddie Rose. She feels it would be better at Bristol Hospital as it’s a smaller group. Maddie Rose can bring along her favorite doll (aka Piglet, I’m sure) and they’ll show her how to diaper and hold the baby. I’m going to call to find out when the classes are held.

Diabetes test will be scheduled for 28 weeks.

I expressed some concern about Olivia’s lack of movements. Sure enough, as soon as I mentioned that, she’s been a wiggle worm all day. It could be that I don’t notice her movements as much on the weekends when we’re very busy.

Olivia’s still breech. Hopefully she turns and stays put. I’d prefer a “natural” delivery versus a c-section. But I’ll do what is best for my princess.