Team Approach to Congenital Heart Defects Helps Davey Jean Thrive
At 30 weeks, Brittney Stanley’s joy over her pregnancy turned to anxiety and worry. Tests showed that her unborn daughter had serious congenital heart defects. Later, more tests revealed a condition known as tetralogy of Fallot with absent pulmonary valve syndrome. Corrective surgery for valve and tetralogy of fallot repair within days of her birth was a near certainty.
It was up to the baby’s parents to decide where they would go for such critical care. They trusted The Willson Heart Center at Valley Children’s, which serves more than 9,000 families and children each year with advanced technology and state-of-the-art therapeutics for both congenital and acquired pediatric cardiovascular diseases.
“Valley Children’s is in our backyard, and we grew up with the Hospital,” Stanley, a former gymnast who was treated there in her youth, said. “We love that Hospital.”
Their confidence was rewarded because today daughter Davey Jean is an energetic 2-year-old with a love of Barney and vegetables. “She’s an amazing little kid,” her mother said.
Stanley credits the medical team at Valley Children’s, which was involved even before her daughter was born. Valley Children’s team approach to patient care means that many different experts – in areas ranging from genetics to cardiology – collaborated on Davey Jean’s complex case.
Rare Combination of Defects, Starting with Tetralogy of Fallot
Davey Jean is missing a small section of chromosome 22 – a condition known as 22q11.2 deletion syndrome which produces a range of heart-related and other problems. Her first diagnosis was tetralogy of Fallot, a condition caused by four congenital defects that affect the function of the heart.
The four defects – a hole between chambers, an enlarged right ventricle, a narrowing of the pulmonary artery and an overriding or misplaced aorta – restrict the heart’s ability to move blood to the body and lungs.
In addition, Davey Jean’s deformed pulmonary valve, which compromised the flow of blood to her lungs, led doctors to diagnose absent pulmonary valve syndrome. The twin diagnoses of tetralogy of Fallot and absent pulmonary valve syndrome occur in only about one of every 25,000 newborns.
As the news unfolded, Stanley held onto the words of Valley Children’s experienced pediatric cardiothoracic surgeon, Dr. Malcolm MacDonald: “He gave us a slogan that we lived by: Plan for the worst, but hope for the best.”
Davey Jean was born two days before Christmas 2013 and transported within hours to Valley Children’s for further assessment and treatment. The newborn responded so well that doctors put off corrective surgery. The delay gave the closely monitored Davey Jean – and her heart – time to grow over the next 11 weeks.
On March 10, 2014, Davey Jean had surgery at Valley Children’s to address the heart defects. Dr. MacDonald and fellow cardiothoracic surgeon, Dr. Frank Hanley, had to make decisions as they examined her heart. The entire procedure “went beautifully,” Stanley said.
Because Davey Jean’s heart function was strong enough, doctors repaired the tetralogy without installing an artificial valve. This allowed Davey Jean to indefinitely delay another open-heart surgery.
Eventually, Davey Jean will need a replacement valve as she grows and demands on her heart increase. “It’s just now a waiting game on when she will need that valve,” Stanley said.
She hopes the procedure can be done at Valley Children’s through a catheter rather than open-heart surgery, which requires a longer recovery time. Valley Children’s performed the region’s first nonsurgical heart valve replacement several years ago.
The Best Comprehensive Care
Following surgery, Davey Jean spent 28 days at Valley Children’s Hospital and has been a frequent visitor ever since. Because of her health issues, she has received care from departments ranging from immunology and orthopaedics to cardiology and genetics. She is experiencing some developmental delays such as expressive speech and low muscle tone of her mouth and ankles. These are likely tied to the same chromosomal deletion that caused her heart problems.
Stanley, who is in nursing school, said Valley Children’s excels at sharing her daughter’s information between departments. “That’s one of the things I love the most,” she said. “The communication and accessibility of her records is amazing. I don’t have to remember everything; they can just pull up her chart and see it.”
Stanley speaks often about Davey Jean’s medical care and also has started a support group, Heart Families of Central California. Last year, Stanley did her pediatric nursing rotation at Valley Children’s, where she someday hopes to work.
“It was amazing to be a part of that side of my daughter’s care, and with the doctors I idolized,” she said. “They saved my daughter’s life.”