Nothing is ever super straightforward in the heart world and we saw that again today with Kendall’s heart cath results. But first and foremost, Kendall’s doing well. She tolerated the procedure well even though she had some ballooning done in both central branch pulmonary arteries (PAs) and in her shunt, which had shrunk from 5mm to 4mm. Luckily, they were able to open the shunt back up to 5mm without any issues. However, her central branch pulmonary pressures were slightly worse than we saw in her last cath back in June despite being on the continuous Remodulin infusion for the last 5 months. Not at all what we wanted to hear as this doesn’t give us any surgical options to help Kendall and further solidified the team’s 4–5-year outlook as a “best case” scenario. That conversation will never get any easier no matter how many times we have it.

After we got to join Kendall in her room in cardiac stepdown (yay for not being admitted to the CICU! - though after seeing Kendall’s name on the procedure list, the CICU was all prepped and ready for her as they know she likes to keep everyone on their toes…), I got a call from Kendall’s pulmonary hypertension specialist at Children’s Dallas. She was pleased with Kendall’s distal pulmonary pressures out into her lungs, which is where Remodulin is most effective. In June, those pressures were 19-23 and they are now 10-12 on both sides. The talk about the distal pressures was relatively new to us as we have always been focused on the central branch pressures.

Unfortunately, the improvement in the distal arteries still doesn’t really change the game plan since the central branch pressures are still way too high. The central branch PAs are just too stenosed from an anatomical perspective for the pressures to decrease. The only thing that could help is another open-heart surgery to try to augment the central branch PAs, but we’ve already been down that road and it didn’t help.

Next Steps: Kendall’s cardiologist will discuss her case with the larger cardiology group at Cook Children’s to get a group consensus on Wednesday, but I think we’re looking at just continuing to do a cath every 9 months to optimize things as best they can and try to get the most longevity out of her shunt as possible. We’ll hopefully have a better update (or at least a more clear update) after the conference on Wednesday.