Seattle Off-Milrinone Echocardiogram Results

Ethan has been off Milrinone for almost a week, his BNP was 77 (lowest it's ever been), and he looks GREAT so we started talking about discharge maybe the end of this week. To be sure his numbers looked as good as he did, we did an echocardiogram and unfortunately his numbers went up. The echocardiogram off milrinone came back with higher right ventricle function in the 50's compared to the right ventricle function on milrinone which was in the 30's. It looks like he needs milrinone.

Why does Ethan need milrinone? There are 3 possibilities:

1. Milrinone helps with contractibility. Milrinone helps the left and right side of the heart squeeze better. It specifically helps the left heart squeeze better which empties the blood from the left ventricle. 
2. Milrinone relaxes the heart. It lowers the resistance to how it squeezes and allows the left ventricle to relax more. The left ventricle is supple rather than stiff. 
3. Milrinone is a pulmonary vasodilator and cross reacts with sildenafil. Something about phosphodiesterase enzymes type III (PDEIII) and type V (PDEV) and how the combination of the two drugs help with pulmonary vasodilation. This article is basically what the doctor was telling me. It's a really good article and even addresses the different pharmacokinetics (IV milrinone vs oral sildenafil) http://bja.oxfordjournals.org/content/96/3/317.full

We don't know why Ethan needs milrinone. Doctor's thoughts:

1. Milrinone helps with contractibility. (#3) Ethan looks too good to be struggling with contractibility. His LV function didn't change much between echocardiograms like the RV function did. Ethan has really good profusions and stuff too so the doctor doesn't think this is at the top of the list. 
2. Milrinone relaxes the heart. (#1) This is at the top of the list. The relaxation of the heart and pulmonary vasodilation are pretty equal as to why Ethan needs milrinone. It's a combination of the two, according to the doctor, because milrinone made such a big difference in his RV function.
3. Milrinone is a pulmonary vasodilator. (#2) Though relaxing the heart and pulmonary vasodilation are pretty equal for Ethan needing milrinone, the doctor did specifically say it's a combination of the two because sildenafil is a better pulmonary vasodilator by itself and plays a bigger part than the milrinone in treating pulmonary hypertension. The combination is best.

Goal: Replace milrinone with an oral equivalent, BiDil.

There isn't an equivalent oral medication that treats all three areas but the doctor did consider treating the relaxation of the heart and pulmonary vasodilation since he doesn't think the contractibility is the main issue. The drug he recommends is BiDil. It's more of a heart failure drug than a pulmonary hypertension drug but in combination with sildenafil he believes it can help. Ethan's pulmonary hypertension is complicated and is somewhat related to his heart failure so we want to focus on a combination of the two. Ethan's heart is not perfect so the idea is to help the heart heal by using healthy tissue instead of scar tissue so Ethan's heart has a better chance in the future.

We're also having troubles with Ethan's heart rate and blood pressures dropping significantly at night when he is sleeping. We want to decrease the work load on the heart without lowering his blood pressure. So we're in the hospital a little longer to optimize his medications so Ethan's blood pressure and heart rate are in the right ranges for his age and size. 

I'm glad we are in the hospital where Ethan can be closely monitored 24/7 while optimizing medications. I can't imagine the stress of trying to do this at home. We are so glad to be here and are excited about Ethan's progress. Through all these changes Ethan remains happy. He embraces every day with a huge smile and makes the most out of his life. Each day is a gift and I believe Ethan knows it too. <3