Phoenix Children’s a lifesaver for Chandler man

Adam Smith didn’t know much about his heart defects until recently.

He knew he was born a “blue baby” and underwent multiple heart surgeries in his first few weeks of life, but his parents didn’t talk much about it.

The topic was simply too painful. Even today – and even though their son is a healthy and happy 35-year-old man – they struggle to look back at that scary and painful time.

“I always knew there was a problem with my heart, but I didn’t know the specifics. I just knew I had to be careful,” said Smith. “With my parents’ help, I enjoyed a pretty normal childhood, even if I did have to take extra precautions in sports and visited the doctor’s office a lot more often than my friends.”

When Smith was born in June 1985, he was diagnosed with pulmonary atresia, a rare and life-threatening birth defect in which the blood vessel that controls blood flow from the heart to the lungs is absent.

This led to a dangerously low oxygen level in his bloodstream. As a result, at 1 day old, he underwent his first open-heart surgery to place a shunt from a blood vessel off his aorta to his left pulmonary artery.

This life-saving operation worked initially, but a week later the shunt clotted off and his oxygen stared to drop again. He was rushed back into surgery for another shunt, this time to the right pulmonary artery.

The procedure was successful. Once he recovered, his circulation improved enough that he could go home with his mom and dad, who would need to monitor him every minute of every day.

Despite their meticulous care, his parents received more bad news shortly after his first birthday. Their baby’s oxygen was dropping again and another operation would be needed.

At 14 months – and at barely 10 pounds – he had grown enough for surgeons to perform an open-heart operation to close the large hole in the bottom chambers and put in a durable pulmonary valve to normalize his oxygen levels.

The best news: this repair should last him through childhood. His parents were incredibly relieved. Thanks to his talented surgeons, their son could finally move past his “blue baby” syndrome.

“Even though my parents struggled to talk about it, they were painstaking in their records,” he said. “As an adult, I look through the albums and read my mother’s inscriptions, and I just can’t imagine what it was like for them. My first year of life truly was touch-and-go. They didn’t know whether I would live through it.”

Smith doesn’t remember those days, but his memory of his fifth open-heart surgery – at age 26 – is sharp as ever.

“I had to get an artificial pulmonic valve implanted,” said Smith. “I have a long and complicated medical history. I can’t believe I know what all of these procedures mean, but I’ve become something of an expert when it comes to my heart.”

Those five surgeries saved Smith’s life, but he experienced different struggles as an adult. Indeed, while Smith is an expert with his own health problems, he has struggled to find doctors with expertise in caring for grown ups with heart defects.

That’s because most adult cardiologists are trained to treat acquired heart disease, which is caused by high blood pressure, diabetes, high cholesterol and smoking.

Care for these patients is dramatically different from treatment of congenital issues like pulmonary atresia, which are birth defects in the architecture of the heart.

“Patients like Adam get lost in the system,” said Wayne J. Franklin, MD, FACC, co-director of Phoenix Children’s Heart Center and associate director of Adult Congenital Heart Disease at Phoenix Children’s. “Adults with CHD are a growing population, but there just aren’t enough cardiologists with the right training and expertise to treat them.”

The Adult Congenital Heart Association reports there are more than two million adults living with CHD. That means there are more adults than children with heart defects.

To raise the standard of care for these patients, ACHA created an accreditation program that requires high-quality care for health systems that serve adults with CHD.

Late last year, Phoenix Children’s was the first health system in Arizona – and one of just a handful in the Southwest – to earn ACHA’s accreditation, which distinguishes the Heart Center and its clinicians as leading experts in the treatment of adult CHD.

Patients like Smith are thrilled to have access to this level of care.

“When I met Dr. Franklin, I was filled with relief,” he said. “My wife and I had moved around a lot for school and work, and I was so anxious to find a doctor who understood what I’d been through and knew how to help me stay healthy.”

Smith met his wife, Pam, when she was a medical student studying to become a pediatric endocrinologist. They bounced from Pennsylvania to Georgia to Missouri.

When Pam was offered a job at Phoenix Children’s, they moved to Arizona. She met Franklin while at work.

“It was serendipitous,” said Adam. “My health issues have been a considerable source of stress for us. Finding Dr. Franklin reinforced that Phoenix was the right place for our family.”

“It is such an honor to take care of ACHD patients,” said Dr. Franklin. “The accreditation process was rigorous and lengthy, but patients like Adam make it absolutely worthwhile.”

Franklin began working toward accreditation when he moved to Phoenix from Texas in late 2018. Criteria include expertise in adult CHD heart surgery, cardiac critical care, pregnancy and reproductive health, heart failure, and adult CHD nursing as well as written policies that direct patient care and provider education.

Phoenix Children’s Adult CHD program sees more than 1,000 adult patients each year and performs more than 50 adult CHD heart surgeries annually.

The program is part of Phoenix Children’s Heart Center, recognized by U.S. News & World Report as a nationally ranked specialty program, which offers the full array of cardiac care for patients – from fetal life through adulthood.

While Pam builds her career as a pediatric endocrinologist, Adam works as the volunteer manager for the Ronald McDonald House. The two have a daughter, 5-year-old Lilly, who also receives care at Phoenix Children’s for osteogenesis imperfecta, also known as brittle bone disease.

“It’s pretty cool that we all have the same hospital to call home,” said Smith. “We’re just so grateful.”

To learn more about Phoenix Children’s adult CHD program, visit

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