Julianne (Hypoplastic Left Heart Syndrome) - Louisiana Pediatric Cardiology Foundation

Julianne (Hypoplastic Left Heart Syndrome)

In the Spring of 2007 my husband and I anxiously arrived at the doctor’s office for my 20-week ultrasound.  It went well, except for the fact that they couldn’t get a good look at the baby’s heart.  So, we were sent over to the hospital for an ultrasound with the Perinatologist (high-risk pregnancy doctor).   We waited for a couple of hours and when we were finally called in the ultrasound seemed to last forever.  I didn’t understand a word of what the doctor and ultrasound technician were saying to each other.  After quite a long time the doctor told us what they were seeing.  They told us about something called Hypoplastic Left Heart Syndrome.  With no medical background, my husband and I had no idea what this was.  We asked the doctor what to expect and she gave us little to no hope.  We were heartbroken.  We drove home in tears and began to call our families and break the news. After a few more visits with the Perinatologist we found out we were having a girl, Julianne, and we were sent to a Pediatric Cardiologist at Children’s Hospital New Orleans for the “official” diagnosis.

We soon found out that the Perinatologist hadn’t had much experience with Hypoplastic Left Heart Syndrome and had, in fact, only seen 3 cases in 10 years of practice.  We also learned that there were much larger medical centers in the United States with high success rates in surgical treatment of HLHS.  We had the blessing of being put in touch with the family of a little girl who was living with HLHS.  The contact with that family became a huge encouragement to us.  After about 2 months of research, prayer and questioning various facilities we decided to go to Children’s Hospital Boston for Julianne’s surgeries.  We arrived in August of 2007 and on September 19th 2007 Julianne was born.

julianne-1Julianne had her first open heart surgery at 5 days old.  She did relatively well, but had some issues with bleeding around the heart and keeping her blood pressure up the first night.  After that, things seemed to be progressing well until Julianne started having runs of SVT (an extremely fast heart rate).   After dealing with this for an entire day, she was moved back to the Cardiac ICU for closer monitoring and treatment.  Very shortly after being moved to the CICU, Julianne took a turn for the worse.  Her blood pressure and heart rate were steadily dropping and she went into cardiac arrest.  The surgeons and nurses rushed to her side and after about 30 minutes of chest compressions they were able to get her onto ECMO (heart-lung machine).  She was stable on ECMO, but would need some time to recover.  Unbelievably, she was able to come off of ECMO with no complications two days later.  Her SVT causing arrhythmia was treated with the strongest anti-arrhythmic medication and was under control.   Julianne went on to have quite a bit of feeding difficulty.  After more than a month in the hospital in Boston, we chose to transfer home to New Orleans to work on Julianne’s feeding.  She continued to struggle with aggressive reflux and we ended up finally being able to take her home on November 14th with a feeding tube.

In February of 2008 we headed back to Boston for Julianne’s 2nd surgery.  She had a pre-operative cardiac catheterization two days before her scheduled Glenn surgery and things seemed to go downhill from there.  She had unexplained fevers, high heart rates, drops in oxygen saturations and was lethargic.  She was soon moved to the CICU for closer observation.  We found out that she was in heart failure, but no one knew why.  At this point the Glenn operation was too risky.  Julianne spent the next 3 weeks in the hospital trying to recover but instead was having more complications.  After an uncommon reaction to a drug the doctors ordered a brain MRI and found that she’d also suffered a stroke. Her Glenn surgery was still too risky.  In March we were told they would go ahead with her Glenn operation.  There were three possible outcomes 1.) The operation would not help her heart function and she would end up on the transplant list, 2.) She would be unable to come off of bypass or 3.) Her heart function could begin to improve.   We were so thankful that the she ended up with the third outcome…her heart finally started to improve.  She was discharged from the hospital one week later and in the following months her heart returned to “normal”. About a month after returning home, we decided to go ahead with a g-tube for feeding (a feeding tube in the belly).

julianne-3Over the next two years Julianne did well, but continued to struggle with feeding and SVT issues. After making an emergency visit to Our Lady of the Lake while visiting my family in Baton Rouge, we transferred Julianne’s care from her Cardiologist in New Orleans to Dr. Crapanzano in Baton Rouge.  A few months later, we moved to the Baton Rouge area.

We returned to Boston in May of 2010 for a cardiac catheterization and ablation (burning/or freezing the pathway causing her arrhythmia and SVT).  The catheterization lasted 8 hours but they believed they were successful in ablating the arrhythmia.   Two days later Julianne had her third staged surgery, the Fontan.  She had difficulty maintaining a stable blood pressure and required 1700 ml of blood products during the first 24 hours. The next day she began to improve!  She soon moved to the floor as we waited for the next 2 1/2 weeks for her chest tubes to finish draining.  Upon removal of the final chest tube we were soon able to head home.  A few days after arriving home Julianne was admitted to Our Lady of the Lake in SVT.  Her heart rate was over 240 beats per minute. We were disappointed that the ablation didn’t give her a complete cure for her SVT but were relieved that she could be treated with a much less dangerous medication because the ablation had made her arrhythmia a lot less difficult to treat.   After another hospital stay a few days later for an unknown virus, things really began to look up.  Julianne regained her energy. Doctors appointments were fewer and farther between and life returned to “normal”.

julianne-4Lately, Julianne has begun eating more and seems to be turning a corner in her struggle with feeding.  Although we’re nowhere near being able to get rid of the feeding tube, we are making progress. Julianne is currently about 50% tube fed, is on 8 medications a day and sees various specialists and therapists. However, you would never know any of this is true unless she removed her shirt.

Each day is a true miracle and we are so thankful that she is here with us! It has been such a blessing that God has allowed us to have the wonderful physicians and hospitals both here and in Boston that have cared for our little girl. We never would have dreamed that traveling 1500 miles for our daughter’s care would be possible, but it was! Our prayer is that all parents facing what we’ve faced would realize that there is hope!