Follow Up with Pediatric Cardiologist (5/29/09)

We wanted to update you on our appointment today with Dr. Shanti Sivanandam (our Pediatric Cardiologist).

We found today's appointment to be much more informative, mostly because we are past the initial shock of the news and because we went with a list of questions and clear heads (and a great note-taker: Angie).

We learned that our baby's official diagnosis is a "Single Ventricle Defect". More specifically, her condition is known as "Ventricular Inversion with a Hypoplastic (underdeveloped) Right Ventricle" along with "Left Transposition of the Great Arteries" and "Complete Heart Block."

Here are the definitions of her heart conditions:
-Ventricular Inversion: Refers to a specific congenital heart defect in which the ventricles are exchanged in position so that the left atrium enters the right ventricle and the right atrium enters the left ventricle (She said that they are "twisted")

-Left Transposition of the Great Arteries:  These vessels (Great Arteries) arise from the wrong ventricle. They are "transposed" from their normal position so that the aorta arises from the right ventricle and the pulmonary artery from the left ventricle.

-Complete Heart Block: This means that the heart's electrical signal doesn't pass from the upper to the lower chambers. When this occurs, an independent pacemaker in the lower chambers takes over. The ventricles can contract and pump blood, but at a slower rate than that of the atrial pacemaker.

Here is the prognosis if each condition stood alone:
-Complete heart block: 50% of babies make it to birth
-Ventricle Inversion: 100% of babies make it to birth

If she had only both of the above (Heart Block + V Inversion) the prognosis would be 20% chance of making it to birth  **But - since our baby has a Hypoplastic Right Ventricle (underdeveloped ventricle) as well as Heart Block and V Inversion - her chances of making it to birth is 10-15%
We know that this statistic sounds very grim - but when we initially heard this news on Wednesday, we thought that the prognosis was less than 5%. The fact that she has a 10-15% chance to make it to birth has ignited us with even more hope in our fight for our baby girl.

As the weeks go by we will know more and more. I will go in to see Dr. Shanti 1x/week to have a fetal echo done and see our regular OB (Dr. Block) 1x/week to check the heartbeat. Dr. Shanti clarified that her heart condition willl not improve. What we are hoping for is that her heart remains stable (which is a heart beat of 47 beats/minute and that it continues to function well.)  She said she does not want it to go below 40 beats/minute. She said the way it was functioning on Wednesday was a good sign. They will be keeping a close eye on the myocardium to make sure it functions well. Indicators that it is not functioning well is fluid building up in different parts of her body.

Dr. Shanti has had one patient who's baby had similar defects. She said her baby made it to 29 weeks, but the baby's heart was not functioning as well as our baby's heart.

After meeting with Dr. Shanti today we feel like we are in the best hands possible. Since Wednesday she had talked to all of her colleagues in her group at the U of M, as well as her mentor who has had 40 years of experience at Columbia University in this specific area, and she mentioned she had been on the phone with her contacts all over the country who seem to be the most knowledgeable people in this field (we were so impressed and comforted by this!!)  We have a 2nd opinion meeting scheduled for Monday. We do not feel like we need it but are going to go anyway...even if it means gaining more information or understanding about her heart condition.

We asked if there are any procedures that we could do now (in utero) and she said there are things we could do (steroids, etc) for heart block to increase the heart rate but w/ her other conditions this would be lethal.

We only talked briefly about what steps would be taken if she continues to stay stable.  The plan would be to do a C-Section at 34 weeks (which is about the first week in September) at Riverside and all of the surgical procedures for the baby would be at the University. (We initially were thinking we would prefer to be at Children's Hosp, but because of the expertise of the U of M in cardio - we feel very comfortable there).  Then, once she is born and they are confident that every other body system is functioning well (lungs, suck/swallow, kindeys, neuro, pee, etc) the first step would be to implant a pacemaker (to correct the Heart Block). After that there would be only a few, simple procedures for the Single Vent, Ventricular Inversion & LTGA.  (She said Dr. James St. Louis at the U would be the surgeon for the pacemaker). 

Because Alli's side of the family has a heart condition called "Long QT Syndrome" which has unfortunately affected 2 of her dad's sisters, Dr. Shanti said she may send us to the U in Madison for further testing. Madison and Italy are the only two facilities which have the technology to thoroughly examine and diagnose arrythmias in utero.  Long QT syndrome has never been detected before birth but this could be a possibility now with their technology. Dr. Shanti has already contacted them and is waiting to hear if they can/will do it.  This would maybe help explain the Heart Block issue and be good information for Dr. Shanti's team's plan as well as information for our subsequent pregnancies.

We also asked her what Alli can be doing to help her stay stable and she said to be as healthy as possible and to "HOPE." She said there is a chance she will make it and we need everyone in our lives to pray and surround this baby with all of the positive energy we have.

We feel SO lucky and so blessed to have had so much support from our wonderful families and friends since our initial test result at 11 weeks. We know you have all been praying and sending all of your positive thoughts since then and ask that you please, please, please continue to pray for our little girl.  Thank you SO much for all of your love and support :) 

Love,
Dan, Alli, Jack & Rufus

ps- We know this is definitely WAY more info than I'm sure you would like but we want to document what happens at each appt :)