Pulmonary Hypertension

If there is one thing I learned during our nearly 4 month hospitalization in the PICU last summer, it is the importance of Thursday cardiac rounds. So when our cardiologist tried to schedule a catheterization on a Friday, I strongly requested the procedure to be moved earlier so the cardiologists could have the cath data to discuss during rounds (instead of waiting for the next week-learned that lesson). This proved to be very successful and helped keep things moving!

Ethan had his catheterization on Tuesday and his cardiac MRI on Wednesday. The 4 cardiologists met this morning (Thursday) to review the data from both procedures and decide the course of action. It was a very heated discussion because all 4 doctors had strong opinons. Here's what we decided on.

Diagnosis: Ethan has pulmonary hypertension. His Cath showed a high LVEDP of 11-12mmHg where the normal is around 6mmHg. However, his previous catheterization, prior to his second surgery to enlarge his aortic arch, had an LVEDP of around 25mmHg. Therefore, his current LVEDP is high but is significantly better than his first catheterization.  We also found that his lung beds are reactive to treatment. At room air Ethan's PVR recorded 8, on 100% oxygen his PVR was around 4.7, and on Nitric Oxide his PVR was 3.1. Normal PVR is 3 and below. During this test, Ethan's LVEDP remained constant around 11-12mmHg. The cath also found a small narrowing from built up scar tissue in the aortic arch that creates a high pressure of 15mmHg where the homograft patch was attached to the arch. This can be fixed using a ballooning technique during another cath. We learned that Ethan is missing his circumflex artery, one of his coronary arteries. The doctors believe this area was damaged during his complicated first open heart surgery. The cardiac MRI verified this finding. Ethan has myocardial infarction under his mitral valve in his left ventricle and it covers a fairly large area.

Dr. Kyser drew this picture of Ethan's heart after his catheterization. 

Treatment: Pulmonary hypertension can be treated with medication and we are optimistic because his lungs reacted so well to medication during the cath. However, his heart is not perfect and there is no surgical fix to Ethan's infarction. The best we can do is start/continue his heart failure medications including: milrinone, captopril, coreg, digoxin, spironolactone, and lasix. If things get worse the next step is a heart transplant.

Plan: Pulmonary hypertension is serious and so Ethan needs to be in the hospital for a while to make sure the pulmonary hypertension is safe and under control before we go home (at least 3 weeks). Ethan was extubated today and started sildenafil (pulmonary hypertension med). Next we are going to maximize his heart failure medications. Then we will put Ethan on oxygen during the day through a nasal cannula. After that we will work with GI to get his reflux under control, maybe even do a pH study of the stomach. Finally we will ween Ethan off the milrinone over a 2 week period. During this process we will follow up with echo cardiograms and BNP levels. If things start to go south we will do another recatheterization to see what his pressures are. Ethan's case will be sent up to Seattle to get a second opinion and to set the frame work for an outpatient heart transplant evaluation after discharge.

We feel good about this plan and are grateful we have some direction. We are also grateful to be in such a great hospital with wonderful doctors and nurses. It makes a huge difference. Ethan will continue his "normal" routine at the hospital. Now that he is extubated, he no longer needs to be restrained or sedated so we can sit up and play. We will also be able to hold him again so we will do our best to make the PICU our full-time home again while avoiding the 20 other rooms filled with nasty respiratory viruses outside our door. :(  

We are so grateful for the prayers on our behalf. We have felt them during this difficult time. Michael and I are at peace and we know we are where we are supposed to be. Ethan's life was spared again. I'm so glad we made it to the hospital in time to figure out what is really going on with baby Ethan and though the next few weeks will be hard, we know they will be special. Ethan is a trooper. I love him so much!

Our first home. We're back home.

Ethan is supposed to be sedated... we had such a hard time keeping him sedated and safe from pulling out his breathing tube.

We extubated straight to bi-pap today and it is going really well. His arms are free and he loves to play! I am so glad we are done with the breathing tube... for now. :)