During some of her regular visits to The Children’s Hospital of Philadelphia, Abigail plays with dolls, blocks, balls and puzzles. Abby, who is 1½, doesn’t know that this fun is serious: doctors and other specialists are keeping close watch for developmental delays. As a group, children with complex CHD, like Abigail, have a higher likelihood of experiencing problems related to neurodevelopmental issues compared to children without CHD.
Abby has been a patient of CHOP’s Cardiac Center since before she was born. Her mother, Tara, was 16 weeks pregnant when a routine fetal ultrasound showed an irregularity in her baby’s heart. She and her husband Adam were referred to the Fetal Heart Program at the Cardiac Center. The program provides highly specialized expertise in diagnosing and managing heart conditions before birth.
Jack Rychik MD, director of the Fetal Heart Program, confirmed that Abby had a complex congenital heart defect in which only one of the heart’s two lower pumping chambers (ventricles) is developed enough to function adequately. The condition, known as double inlet left ventricle, is so complex that the overwhelming majority of babies with this diagnosis do not reach their first birthday without some form of treatment.
The treatment for double inlet left ventricle needed for Abby to survive, required a series of cardiac surgeries to reconstruct her tiny heart and re-route circulation. Surgery does not give children with double inlet left ventricle a normal circulation, but it allows their heart to better pump blood to their lungs and the rest of their body.
For the rest of the pregnancy, Tara and Adam returned to CHOP monthly for visits with the Fetal Heart Program allowing the medical team to carefully plan for Abby’s arrival and care. There they discovered yet another reason to be grateful they’d come to this world-renowned children’s hospital: the Garbose Family Special Delivery Unit (SDU).
The SDU is the world’s first birthing facility dedicated exclusively for mothers carrying babies with birth defects, such as double inlet left ventricle. The Special Delivery Unit allows these infants to be born within a pediatric hospital where the highest level of care is available from the moment of birth. Moms stay in comfortable private rooms, just steps away from the operating rooms and intensive care units in which their babies are treated and recover.
On October 6, 2008, with Adam at her side, Tara gave birth to Abigail at the Special Delivery Unit at CHOP. The Cardiac Center team was waiting to stabilize the baby and immediately transport her to the Cardiac Intensive Care Unit, where Tara could visit any time. “It made a huge difference to be with Abby as much as I could,” she recalls.
At 3 weeks of age, Abby’s condition deteriorated. She was feeding poorly and had low oxygen levels in her blood. She was seen by cardiologist Gil Wernovsky, MD, and the decision was made to admit her to the Cardiac Intensive Care Unit (CICU). A follow-up echocardiogram showed a tumor, called an atrial myxoma, growing rapidly in her heart’s right atrium. The tumor, combined with the double inlet left ventricle, were responsible for a very rapid heart rate. The next day, Thomas Spray, MD, Chief of Cardiothoracic Surgery at CHOP, performed open heart surgery to remove the tumor and placed a shunt to improve Abby's oxygen levels. She recovered quickly with a rapid improvement in her feeding, growth and oxygen levels. As planned for treatment of the double inlet left ventricle defect, Abby had a second operation at 5 months of age, and a third when she was 3 years old.
Abby's relationship with CHOP was just beginning. She is among the first participants in the Hospital's NeuroCardiac Care Program (NCCP). The program is led by Gil Wernovsky, MD, Medical Director, and Jean M. Carroll, RN, MSN, Program Manager. The NCCP was created in 2009 in response to research at CHOP and other institutions that showed that children with complex CHD, such as double inlet left ventricle, are at higher risk of neurodevelopmental and behavioral difficulties compared to children without CHD. This program brings together an interdisciplinary group of practitioners to provide screening, evaluation and ongoing coordination of care for children with CHD at risk for these issues.
During her twice-yearly visits to the NeuroCardiac Care Program, Abby sees a developmental pediatrician, a physical therapist, an occupational therapist, a speech and language pathologist, a neurologist and a cardiologist. The team evaluates motor skills, muscle tone and developmental benchmarks such as speech. The family also meets with a social worker for psychosocial support and to learn about resources in their own community. Abby will also continue to see her cardiologist each year as she grows.
For Tara and Adam, the CHOP program means peace of mind. “The sooner they catch certain things, the more treatable they are and the better quality of life she’ll have,” says Tara.
For any parent, watching a child born with a severe heart defect grow and thrive is a joy. For Tara, the process has been bittersweet. Back in 1980, Tara’s younger sister Erin was born with double inlet left ventricle, the same congenital heart condition as Abby. But Erin did not live to see her fourth birthday. When Tara learned that her own baby had the same diagnosis, she was devastated. But Abby, happy and thriving today, embodies the incredible advances in cardiac care since Erin’s death. “I look at the progress research has made over the last 30 years right in the face every day,” Tara says. And as Abby makes her own progress, CHOP will be with her every step of the way.
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