Atrioventricular Canal Defect: Madeline's Story

Madeline is an active, happy 9-year-old. She loves school, playing soccer, singing and dancing in musical theater, and spending time with her friends. She’s a delight to her parents, who look back to when she was born and give thanks to the medical team at CHOP who saved her life.

Madeline In a routine ultrasound 19 weeks into their pregnancy, Karen and Brian McAndrews learned that their daughter would be born with a heart problem.

To get a more accurate diagnosis, they made an appointment at Children’s Hospital of Philadelphia (CHOP). At the Center for Fetal Diagnosis and Treatment, they met Jack Rychik, MD, Director of the Fetal Heart Program, who reviewed the images and explained to them that their daughter’s heart condition was serious.

She had an atrioventricular canal defect: a hole in the center of her heart. She would need surgery soon after birth in order to survive. Madeline’s heart was also unbalanced, with the left ventricle smaller than the right. If it continued to develop that way, she would be born with hypoplastic left heart syndrome (HLHS), an even more serious problem that would require a series of three operations to correct.

An emotional journey

Karen and Brian were stunned by the news, as any parents would be. They asked all of the difficult questions parents ask when they get a frightening medical diagnosis ­— “How did this happen? Why is this happening to us? Will our baby survive or lead a normal life?”

They did research on the conditions and sought out other families who had been through this before them. Hearing the stories of other children who had heart surgery and were now doing well comforted them.

“I was searching for hope,” says Karen. “I prayed for guidance and strength. Ultimately, I found comfort in believing that we had been chosen to raise a special child."

Madeline was born in October 2007 at Pennsylvania Hospital, with a team of CHOP doctors on hand to care for Madeline immediately after delivery.

The CHOP team confirmed that the hole in the center of her heart was large, and Madeline would need surgery within a few days. They also found another problem, a narrowing of the aorta (coarctation of the aorta). This narrowing restricted blood flow through the heart and would need to be repaired. To their happy surprise, they found that Madeline’s left ventricle looked large enough to sustain her, so the set of three staged procedures to repair that problem wouldn’t be needed.

Madeline was immediately transported to CHOP’s Cardiac Intensive Care Unit for special care before her surgery. Karen stayed behind at Pennsylvania Hospital to recover from her c-section. Brian spent the next three days going back and forth between the two hospitals until Karen could join him to be with Madeline at CHOP.

Today, babies like Madeline who will require specialized care before, during or after birth have the option of delivering in CHOP’s Garbose Family Special Delivery Unit, the first birth facility in a pediatric hospital designed for healthy mothers carrying babies with known birth defects. Moms can stay close, and babies are treated immediately without having to be transported.

When Madeline was only 5 days old, Thomas Spray, MD, operated on her heart to fix her aorta. Eight months later, she was back for a second surgery to repair the atrioventricular canal defect.

Achieving milestones

Madeline recovered quickly from her surgeries as a young child and has not needed additional medical interventions since. She hit all the normal developmental milestones and even began walking early.

“She was never a child who would just sit,” Karen shared. “At music class when she was just 1 year old, she was doing laps around the room, singing and dancing.”

Every September, she comes back to CHOP to see Dr. Rychik and the Cardiac Center outpatient team for her annual check-up to ensure that her heart continues to grow normally.

“We truly look forward to our visit every September,” says Karen. “Every time I walk through those doors, I feel like I’m getting a warm hug. Madeline wouldn't be alive today if it weren't for the phenomenal care she received from the amazingly caring and compassionate staff at CHOP. We will be forever indebted to them for this amazing gift they have given us."

Support for single ventricle care and research

About the Single Ventricle Survivorship Clinic

The McAndrews, together with Madeline's grandfather, Robert G. Klimasewski, showed their appreciation to CHOP's Cardiac Intensive Care Unit with a generous philanthropic gift to the Single Ventricle Care and Research Fund. They continue to give back to the doctors who helped them and Madeline through their support for the Single Ventricle Survivorship Clinic, part of the Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program.

Founded by Dr. Rychik, who is still Madeline's primary cardiologist, the program's multidisciplinary team of specialists provide long-term care for single ventricle survivors. An important part of the team’s work is active research focused on improving outcomes and finding better solutions for the health complications that often afflict these patients as they grow older.

"We are grateful to CHOP for the excellent care and compassion showed to Madeline and our family during extremely terrifying times," Klimasewski says. "The outcome was beyond expectations. We can't do surgery, advise on cardiac issues or prescribe meds. But we can assist CHOP and, in particular Dr. Rychik, to advance his ability to help others similarly affected like Madeline through a meaningful contribution."

The contributions made by Madeline’s family are more than an investment in the future of medicine — they are a celebration of life and an investment in better futures for all single ventricle patients.

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