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