CaringBridge Is Funded by People Like You

Make a donation to CaringBridge

Honor Mikella with a tax-deductible contribution to CaringBridge today.

Click here to make your donation.

Mikella’s Story

Mikella was diagnosed with Total Anomalous Pulmonary Venous Return at 3 weeks of age. By the age of 3, she's undergone 3 open heart surgeries, and just had a pacemaker implanted in December of 2009. She is our strong little fighter! You can follow her progress on this site and please sign the guestbook!

Mikella was born with TAPVR, a condition in which the pulmonary veins instead of attaching from her lungs back into her heart, attached to her liver.  She was misdiagnosed at birth, and not diagnosed until 3 weeks of age.  She had emergency surgery within 24 hours of being diagnosed, which lasted about 11 hours.  Mikella spent 6 weeks in the hospital recovering from surgery.  She suffered from severe Pulmonary Hypertension post-surgery, in addition to developing pneumonia and a Chylothorax, and required ECMO support post-op.  

Mikella was home for 6 weeks after discharge, and at her first outpatient check-up was found to have Post Repair Pulmonary Vein Stenosis.  The Pulmonary Veins that had been attached to her heart had closed off from scar tissue in the veins and she had no blood flow to her left lung.  Another complete repair was done, and due to inflammation found in her heart, Mikella was given a 50% of continuing to have her veins stenose and for it to be eventually fatal.  Again post-op, Mikella developed pneumonia and Chylothorax.  She was discharged after 3 weeks in the hospital.  A residual ASD was left to protect against future stenosis.

Mikella did well in the coming years, despite oxygen saturations that were in the low 90's or high 80's.  By age 2, she was seen on Echo to have a severe narrowing of her Superior Vena Cava.  When a pre-op cath was done, it was found that the patch from the previous repairs had given way.  In addition to the severe SVC stenosis Mikella also had a large ASD, blood being rerouted out of the heart that should be going in, and blood flowing into both Atriums at the same time, causing a sort of PAPVR.  A repair was done in April 2009, and was successful and Mikella now has normal oxygen levels.

Unfortunately, because of having so many surgeries in the area where the sinus node is, Mikella's sinus node (the heart's natural pacemaker) was damaged and her heart rate continued to slow.  Her heart and liver were enlarging and her pulmonary pressures were increasing.  Mikella had a pacemaker implanted in Dec. of 2009.  Because of her size, she had to have another full sternotomy and the pacemaker was implanted in her stomach.  Mikella is treated at the University of Minnesota and is under the care of Dr. Jamie Lohr, Cardiologist, Dr. Ann Dunnigan, Electrophysiologist, and Dr. James St. Louis, Surgeon.

Latest Journal Update

Cardiology Check-Up

Mikella took a trip up to the Cities today to see Jamie, her Cardiologist. It had been 1 whole year since we had seen Jamie last, the longest we've gone between visits so far (although we've had Pacemaker checks in between.)  Mikella was excited to go. She asked which clinic we were going to and when she found out it was the Telephone clinic (they have a play phone booth in their lobby) she was happy. And yes, she distinguishes the type of appointment she has by the toys in the lobby. :-)

I wish I could say everything was great and we don't have to go back for another year but that doesn't seem to be the case. Mikella got her vitals checked and it appears she is now more inches taller than she she weighs in pounds. Almost 4 feet tall but only 43 lbs. They did an Echo and EKG. It looks like her Pacemaker is functioning normally. There was one slight abnormality found in her Echo. Her Mean Gradient has been slowly increasing over the last couple years and currently appears to be sitting at a 7-10. What I take that to mean is that the pressure or force in which the blood is flowing through her anastomosis is increasing. (Her anastomosis being the connection the surgeon created when he reattached the pulmonary veins to the heart.)  We aren't seeing any other abnormal readings. Her pulmonary pressures are good, the size of her right atrium is normal, her liver size is normal, her oxygen sats are normal. We're not really seeing any other signs that something is wrong. It was briefly thought that perhaps she was anemic, but a quick blood test ruled out that (simple) possibility.

These changes seem to be happening pretty slowly over the last few years. The best explanation Jamie had for us was that there has been some sort of change with her Confluence as she has grown that is causing it to change or narrow or something of the sort. The confluence is that in most (all?) types of TAPVR, the pulmonary veins come together and end in a collection, called a "confluence", behind the left atrium. When TAPVR is repaired, the surgeon reattaches this whole confluence of pulmonary veins back to the left atrium.

Right now Jamie was talking to someone in imaging to see if doing a CT scan would maybe give us some better pictures of her confluence to see what is going on or what is obstructed. If they think an CT scan would be of benefit she would likely do one soon. They will also repeat another echo in 6 months at her next pacemaker check (June). If they decide a heart cath is needed, they would schedule it over the summer. A heart cath would give them a better look at what is going on inside, and possibly they could try to balloon open the veins/confluence if it was narrowing, although I don't think the successfulness of this approach is very high. If the gradient continues to increase she would likely need another surgery to try to open up the confluence. I believe Jamie said they would probably just need to carve out a little tissue and she thinks it's probably a result of changes from growing. So as much as I HATE the watch, wait and see approach I guess that's what we're doing. I don't really mind the extra appointments, etc., even the cath. I just HATE the thought of having to cut into her chest again. She already has ongoing pain and deformity from the first 4 times. I would just do anything to keep her from having to go through it again!!!! But in the end we never really have a choice. We are just blessed to have Jamie, as we know she will keep a very close, watchful eye on her.