Our Baby Girl

I do not know how else to start this without jumping into the heavy stuff right away. I’m 7 months pregnant with a baby girl who has a complex heart defect.  I wanted to create a record of our experience for the benefit of keeping family and friends informed as well as the hope of connecting with other parents of heart babies.  I should have started this record a couple months ago to have a more detailed recollection of our journey through this but it took some time before I could articulate the medical complexities as well as the length of time it took for me to reach a point of acceptance with this trial.

Here Comes the Sun

My pregnancy up until 2 months ago had seemed perfectly normal. Morning sickness was definitely worse than I remember having when I was pregnant with Bailey, but that was 10 years ago, it’s possible I blocked that out.  I was still able to function and go to work every day so it was nothing concerning.  My weight gain was appropriate, the baby’s heart was heard loud and clear through the doppler at every visit, my blood work was normal; everything was going as planned.  At 20 weeks, we had our much-anticipated ultrasound. This was going to be the day that we were going to learn the gender of our baby.  The ultrasound technician confirmed right away that we were having a girl :) My brain traveled to thoughts of all things pink and purple, My Little Ponies and hair bows. I wondered if she would be anything like her big sister or if she would have Mark’s curly hair.  After I exhausted my daydreams, I quickly became frustrated that the technician wasn’t done yet. I was eager to leave and celebrate. What more could she be looking at? I started to panic a little when I realized she had been quiet for some time and was I praying that at any moment she would say “All done, everything looks great!” but instead she told us she was going to get the doctor.  Anybody that has gone through a similar situation knows that those words matched with that tone have the power to stop all time as it shatters the world as we know it. The doctor quickly came in, looked at the images on the screen and then nervously explained to us that the baby’s heart was on the wrong side.  I couldn’t focus on much else after that other than the Beatles song, “Here Comes the Sun” softly playing on the radio next to the box of tissues.

Dextorcardia/Heterotaxy

If you’re anything like me, then you know nothing about the human anatomy other than our insides are filled with guts and the heart is somewhere in the center of the chest. I know now that’s not true.  A normal heart’s apex points to the left and is made up of 4 chambers with a two-sided pump. The top-half chambers are the atria and the bottom two are the ventricles. The right side of the heart (both the right atrium and right ventricle) pump the blood that returns from the body to the lungs to be oxygenated.  The left side (left atrium and left ventricle) pump the oxygenated blood back through the body.  The walls of the heart are divided by a septum which keep the oxygen rich blood and oxygen poor blood from mixing (I am a software analyst, not a heart expert so forgive my basic explanation). 

After our initial ultrasound, we were referred 3 days later to a pediatric cardiologist for an echocardiogram of our baby girl’s heart.  Our doctor was able to confirm that indeed, the heart’s apex points to the right as well as the below list of additional complications:

·         Heart on right side of chest & pointed to the right (dextrocardia)

·         Single ventricle/pumping chamber

·         One common atrioventricular valve (valve between the upper heart chambers & lower heart chambers)

·         Aorta and main pulmonary artery both come off of the single ventricle

·         Veins from the upper body & lower body drain to the right atrium

·         Main pulmonary artery & pulmonary valve is smaller in size than aorta; there is blood flow across the pulmonary valve into the pulmonary artery from the ventricle

·         Pulmonary veins (with blood coming back from the lungs) drain abnormally to a confluence behind the right atrium & then into the right atrium

To sum all of this up in a single prognosis it looks as if our baby has heterotaxy syndrome.  My own research has told me that this condition is extremely rare, occurring in only 4 of every 1 million babies that are born.  Heterotaxy simply means ‘different arrangement’, and brings with it a constellation of complications that affect other organs in the body.  Usually the intestines are malrotated, the liver resides midline and either no spleen exists (Asplinia) or multiple (non-functional) spleens are present (Polysplenia).  The severity of each case depends on the individual and each case is unique.  As of right now, we don’t know the complete anatomy of our baby girl, we can only look at the heart, which doesn’t give us very clear picture of what to expect. 

At our first visit with the cardiologist, he explained to us the structure of our baby’s heart and that we have three options:

1). Terminate the pregnancy.   

2). Carry the baby to term and select ‘Compassionate Care’ allowing the baby to pass without intervention.   

3). Carry the baby to term and pursue an aggressive series of high-risk surgeries that could never ‘fix’ the heart but could prolong life for X amount of years.

My initial feeling was to go with option number 3. It was the only option that allows our baby a chance at life.  The more that I learned about the operations I more unsettled I felt about this choice.  The statistics for a healthy outcome are grim. Many babies with hearts as complicated as our baby’s heart are not born stable enough for surgery. Some that are stable enough don’t survive the first operation.  Those that do, must also survive the second and third surgery.  The Fontan procedure, is the final heart surgery that can be done between 2-4 years of age. This is done for single ventricle patients and there is very little data on the long-term outcome of these patients as well as their life expectancy since this operation has only been practiced for around 20 years.  I spend a lot of time thinking about this and the moral implications of the 3 choices at hand.  The time for terminating the pregnancy has long since passed and it was never really an option for me anyway. I could never judge anybody for the choices they make in these situations.  I would have never known what my choice would be prior to this situation, however carrying the baby to term has been the only decision so far that I have been 100% certain about.  

Right now, we are talking to as many doctors as we can and getting as much information as we can.  Between my regular visits with the high risk OB and the cardiologist’s office, we seem to have an appointment every week.  We have met with a genetic counselor, a neonatologist and have an upcoming appointment with the cardiovascular surgeon.  We are lucky to have access to so many brilliant people that can help walk us through this very difficult process.  I have an amniocentesis scheduled for next week. I was on the fence about whether or not I should get one for some time.  The first several times it was brought up I declined it because I was afraid of the risk involved.  However, I now feel that in order to have a complete birth plan it’s best to know what is going on with our baby’s chromosomes in addition to her anatomy.  Thank you for reading and I hope to continue updating this blog with the good and bad that comes with this journey.

~Melissa