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Katie Foley

Triple Challenge: Tetralogy of Fallot, Pulmonary Atresia and Collateral Vessels

Katie Foley is one tough cookie. That’s not a typical way to describe a baby. But with multiple heart surgeries in her first six months successfully behind her, Katie is far from typical.

The third child of P.J. and Bill Foley, Katie was 6 pounds 1 ounce when she was born in 2005. Her dusky coloration was the first tip to doctors that something might be seriously wrong.

Rushed to Children’s Hospital of Pittsburgh of UPMC within hours of her birth, she was quickly diagnosed with tetralogy of Fallot (TOF), a complex condition involving four specific defects that conspire to limit her body’s ability to use life-supporting oxygen. Those defects include a hole between the right and left chambers of the heart known as a ventricular septal defect (VSD), a thickened right ventricle muscle wall and an enlarged aorta, positioned over the VSD. The fourth condition of TOF is a restriction of blood flow from the right ventricle to the lungs due to a narrowed pulmonary valve. But in Katie’s case, the condition was much more severe – the tract was blocked completely, a condition known as pulmonary atresia.

In addition to TOF with pulmonary atresia, Katie has what doctors call collateral blood vessels – extra vessels from the aorta to her lungs that grew during her development. "The collaterals are irregular, convoluted vessels that supply blood to her lungs because the normal pathway was blocked. But these high-pressure vessels are often not big enough to supply the lungs as babies grow," explained Katie’s cardiologist, Linda Russo, MD, FAAP.

Doctors at Children’s used all the tools at their disposal to get a clear picture inside of Katie’s tiny body – electrocardiograms, echocardiography and ventilation-perfusion (VQ) scans, which determine blood flow to each lung. When Katie was 6 days old, she had a cardiac catheterization, allowing a closer look at the structures inside.

Catheterization confirmed what doctors were hoping for – on the other side of the blocked valve she had a true, albeit small, main pulmonary artery exiting her heart and branching, like a "T," to her right and left lungs. This information set the course for the next stage of her treatment – creating normal pulmonary blood flow, which in turn would help the pulmonary arteries to grow and promote normal oxygenation.

One Step at a Time

On Katie’s seventh day of life, surgery was performed by Victor O. Morell, MD, chief of the Pediatric Cardiothoracic Surgery Division, to open the pulmonary valve, enlarging it with a graft to promote a more normal flow into her pulmonary arteries. Dr. Morell and his team judged that closure of the VSD would have to wait until later so as not to create back-pressure issues within Katie’s heart due to her small pulmonary arteries. Also her collaterals would have to remain until they were sure that the pulmonary arteries would grow big enough to handle the entire blood flow.

When Katie was nearly 3 months old, Children’s Cardiac Catheterization Lab took the next major step to rehabilitate her pulmonary artery system – balloon angioplasty. Under the guiding hand of Jacqueline Kreutzer, MD, director of Interventional Cardiology, a catheter was once again inserted via a leg vein up into the heart and beyond, into the narrow branching structures of Katie's pulmonary arteries. As five of the most restrictive narrow spaces were encountered, a balloon at the tip of the catheter was inflated, tripling the diameter of millimeter-thin arteries to promote blood flow. Dr. Kreutzer repeated the treatment two months later, meticulously addressing another 10 sites with balloon expansion and adding a single stent in a seven-hour procedure.

The second procedure was "a huge leap forward in terms of increasing the blood flow to her lungs and allowing both of them to share equally in the oxygenation of her blood," said Mr. Foley.

The Fight Continues, With Hope

Not that many years ago, babies born with a condition like Katie's did not survive the newborn period. And, while Katie’s cardiopulmonary system is still a work-in-progress, she is "completely asymptomatic today" said Dr. Russo. Her mother describes Katie as "outgoing with a wicked little sense of humor." Her father says her "resiliency is nothing short of remarkable."

Through multiple complications, Katie’s battled hard and always comes away smiling. She no longer requires all of the 13 medications she initially needed as a newborn. Eventually doctors will need to address the hole in her heart. Left alone over a protracted period of years, her collateral vessels could also be a problem leading to lung damage because of the high blood pressure coming off of the aorta. So they remain a concern and may eventually be closed off with small coils placed by catheter.

Helping to give the family hope for Katie’s future is the success of Mr. Foley’s niece, another TOF survivor. Born in 1984 in New Mexico, she too was brought to Children’s Hospital of Pittsburgh because of its reputation in cardiac care and the pioneering work then being conducted in infant heart surgeries. "She’s thrived, led a successful life and is now an emergency room nurse in Albuquerque," said Mr. Foley. "We certainly have all the confidence that this is the place you can go to get world-class care – you see the people from around the world who bring sick children here because they know this is a place where miracles can happen."
 

 
Last Update
February 19, 2014
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Last Update
February 19, 2014
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