Solving Addison’s puzzle: Uncovering a congenital heart defect

Addison-on-bike-trail1Addison is a smart, sweet and caring child who brings a smile to everyone she meets. She is known for her hugs and frequently doles them out to anyone and everyone. She loves elephants—and her heart is elephant-sized! However, until last fall, Addison’s heart was causing mysterious and persistent medical issues that prevented her from doing all the things she loves. Unbeknownst to my husband and me, Addison was born with a congenital heart defect.

As a baby, Addison thrived but always seemed to have something going on. She had issues with reflux and did not enjoy tummy time or sleeping on her back. Waking several times during the night is not uncommon for a baby, but Addison seemed to be waking in pain every hour or two. When she was 9 months old, a GI specialist diagnosed her with reflux, and as she grew into a toddler, Addison developed significant respiratory issues, including a croupy cough. Now when she woke up during the night, it was with severe coughing fits.

She was eventually diagnosed with both asthma and sleep apnea and had her adenoids removed, which seemed to alleviate the symptoms. (It took us a while to get used to her sleeping so soundly yet so quietly!)

At this point, we thought we had figured out what was going on with her. We continued to manage her asthma symptoms when they developed, but they seemed to be improving. As a family, we all started to sleep much better.
large team addisonAddison loved to play any sport with a ball, so she was thrilled when she turned 4 and could finally play for the town soccer league. But when she ran, her cough would come back, and she seemed to get pretty winded. Even though she loved the game, she just couldn’t keep up with the other kids.

Then one day, Addison fainted. We saw her pediatrician and had an electrocardiogram (EKG), but it seemed like Addison had probably just fainted from dehydration. When she fainted again at school, I started to worry. This time, her pediatrician recommended a follow-up appointment with the Heart Center at Boston Children’s Hospital.

We went to a satellite office in Peabody, as it was closer to home than Boston, and saw Julianne Evangelista, a nurse practitioner in the outpatient cardiology department.

After listening to Addison’s history, thoroughly examining her and repeating her EKG, Julianne came to the same conclusion as our pediatrician: the fainting was most likely due to dehydration. However, Julianne didn’t stop there. Experience and intuition told her to dig a little deeper. She ordered a 24-hour Holter monitor and an echocardiogram. They did the echo right then and there.

Much to the surprise of the echocardiogram technician, Julianne and me, the imaging revealed that Addison had a heart defect. After a sedated MRI and pulmonary function test, Addison’s diagnosis was confirmed: pulmonary artery sling, or PAS. PAS is a rare condition in which the left pulmonary artery emerges from the right pulmonary artery and passes between the trachea and the esophagus to get to the left lung. PAS is known to cause the symptoms Addison had been experiencing, such as fatigue and shortness of breath. Julianne had finally solved our puzzle!

Addison with EmaniIt wasn’t long after Addison was diagnosed that Dr. Sitaram Emani performed open-heart surgery on Addison to correct her heart defect at Boston Children’s Hospital. She was home just three days later and returned to kindergarten three weeks later, full of energy and enthusiasm!

Soon she was once again playing soccer and swinging from the monkey bars at school like all the other children her age. Now she can keep up with her peers and doesn’t cough or get winded while running. At a recent cardiology check-up, she surprised the ultrasound technician with one of her signature hugs. The technician said Addison had made her day!

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