Tetralogy of Fallot: Aidan's Story

Aidan had open heart surgery as a 3-day-old and brain surgery as a 5-week-old. He was 8 weeks old when his parents, Maria and Charles, could finally bring him home.

aidan Home was nearly a two-hour drive from The Children’s Hospital of Philadelphia, where numerous teams had helped Aidan through difficult months. His parents knew they would make the drive many more times — and felt grateful.

“I am so happy that we happen to live this close to such an amazing place,” Maria says. “There are so many families coming from so far away. I’m grateful that we have such a great center specializing in children’s care so close by.”

The diagnosis: Tetralogy of Fallot (TOF)

Maria was 20 weeks pregnant when an ultrasound showed an irregularity in her baby’s heart.

A maternal-fetal medicine specialist near their home in Lancaster, PA, diagnosed Maria's unborn baby with tetralogy of Fallot (TOF), a congenital heart defect that refers to a combination of related heart defects, including a ventricular septal defect (VSD), a hole between the lower chambers, and other irregularities such as pulmonary stenosis, a narrowing of the pulmonary valve that affects blood flow from the heart to the lungs.

Choosing CHOP

After researching their options, Maria and Charles decided on the Cardiac Center at CHOP. The Fetal Heart Program at the Cardiac Center, led by Jack Rychik, MD, specializes in the diagnosis, evaluation and management of congenital heart disease, such as TOF, prior to a baby's birth. It is one of the largest such programs in the country with a team of fetal cardiologists, nurses, sonographers and social workers who specialize in heart disease in unborn babies.

After the fetal echocardiography, pediatric cardiologist Shobha Natarajan, MD, confirmed that the baby did have tetralogy of Fallot. She explained the heart defect and the open heart surgery that would be necessary soon after the baby was born.

Maria and Charles, though upset, were relieved to hear that if their baby was otherwise healthy, a full recovery was possible.

Over the next four months, Maria and Charles continued to visit the Cardiac Center for checkups.

Dr. Natarajan and the Fetal Heart Program team planned for the baby’s arrival — Maria would deliver in the Garbose Family Special Delivery Unit (SDU) at CHOP. This first-of-its-kind delivery unit, for babies with known birth defects, provides access to world-class care for mother and baby in one location. It is on the same floor as cardiac operating rooms, catheterization labs and the Cardiac Intensive Care Unit.

The Cardiac Center at CHOP is like a hospital within a hospital. Cardiac patient units and the operative and imaging suite take up nearly the entire sixth floor. The care team of nearly 600 includes three cardiothoracic surgeons, more than 50 cardiologists, more than 250 cardiac nurses, a team of anesthesiologists, child life therapists, social workers and many more specialists, all dedicated to the care of children with congenital heart defects.

Delivery day in the SDU

Aidan was delivered at 9:30 a.m. on Feb. 15, 2010. Babies born in the SDU are tended to by a highly specialized team of obstetricians, anesthesiologists, neonatologists, cardiologists, advanced practice nurses, OB and neonatal nurses and social workers. Mother and baby have immediate access to a level of skill and technology that is among the highest in the world.

“Delivering at CHOP gave us confidence,” Maria recalls. “We knew that whatever care the baby needed would be close by.”

After he was delivered, Maria and Charles were able to touch Aidan before a team took him to an adjacent room to stabilize him. At 4 p.m., Maria was able to visit her son in the Tabas Family Cardiac Intensive Care Unit down the hall.

“I was able to hold him,” she recalls. “He had wires and things but the nurses made it work. They’re wonderful. We shed a lot of tears.”

Surgery to treat TOF

Three days later, Aidan had open heart surgery to treat his tetralogy of Fallot. Thomas Spray, MD, chief of Cardiothoracic Surgery at CHOP, closed the hole in Aidan’s heart and opened up the right ventricular outflow tract. The surgery went well.

However, a few days later, Aidan developed a pleural effusion, an accumulation of fluid in the lung cavity that can occur as a complication of surgery. He would have to remain in the Hospital.

At 1 month of age, cardiologists noticed signs of hydrocephalus, a build-up of spinal fluid in the brain. At CHOP, our Cardiac patients have immediate access to top-ranked pediatric specialists including Neurosurgery, which Aiden would need immediately for a shunt placement.

On March 26, neurosurgeon Leslie Sutton, MD, placed a shunt (a one-way valve) to drain spinal fluid from Aidan’s brain to his stomach. Aidan will have the shunt for his whole life.

Because their hearts function differently and their blood-oxygen levels may be lower than normal, children with heart defects can react differently to anesthesia. CHOP is one of the only pediatric institutions with an anesthesia team specifically for cardiac patients. This team was with Aidan during brain surgery.

Recovery after surgery

Aidan's recovery went well and on April 12, he came home. Aidan is now a happy 14-month-old.

While he came home on seven medications, now he takes none. 

He visits Dr. Natarajan, his primary cardiologist, every six months. Dr. Natarajan has been an important part of Aidan’s life since before he was born and will be for many years, providing vital follow-up to ensure Aidan’s heart continues to function well.

Maria will never forget Dr. Natarajan’s visits to the delivery unit in the week after Aidan was born.

“She would stop by my room, late at night and in the morning, to check on me and say hello,” Maria recalls.

I felt like she was always there for us.

Fear transformed into joy: the future

Aidan is also part of the Cardiac Kids Developmental Follow-up Program. Children with complex heart defects, such as TOF, have a higher likelihood of neurodevelopmental problems, compared to children without complex heart defects. These may include learning disabilities, ADHD and speech and language issues. The Cardiac Kids Program includes a team of cardiologists, neurologists, developmental pediatricians, nurses, and physical and occupational therapists and other specialists who screen and evaluate at-risk patients and help coordinate care if needed.

So far Aidan doesn’t have any delays. With help from CHOP, Maria and Charles' initial fear has been transformed into joy at their son’s good health.

“He is like any normal little kid,” Maria says. “His shirts cover his chest scars and his hair covers his shunt. Nobody would ever know anything different.”

Originally posted: April 11
Updated: September 2013

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