Rare pulmonary defect prompts parents’ nationwide search for answers

With no blood flow to his right lung, infant Carter Johnson was diagnosed with a rare condition called absent right pulmonary artery. His parents turned to Stanford Children’s Health for help.

Carter Johnson underwent two surgeries at Packard Children’s Hospital. 
Courtesy of the Johnson family

Carter Johnson came into the world on Jan. 9, 2018. According to his parents, Kelly and Malcolm Johnson, he was perfect in every way. But it wasn’t long before red flags went up, and one of the couple’s happiest days became shadowed with worry and fear.

Something wasn’t quite right. Carter’s color was off and he was turning gray, prompting the care team at his local hospital in Maryland to whisk him to the neonatal intensive care unit for tests. When their efforts failed to provide answers, the family was sent to a regional hospital for further examination. That’s when they discovered there was no blood flow to Carter’s right lung. He was diagnosed with a rare condition called absent right pulmonary artery.

Time was of the essence. Typically, this condition is associated with multiple congenital heart defects, prompting additional echocardiograms, more tests and a rapid search for answers. In Carter’s case, his doctors determined that the uncommon malformation was isolated. But risks were high, as Carter’s test results revealed pulmonary hypertension, and other complications could be imminent. Carter needed help. 

Searching for the best treatment

Kelly and Malcolm were told by the regional hospital that the best course of treatment for Carter’s condition was surgery but that he was not a candidate. This prompted his parents to start a nationwide search. Malcolm went into research mode and delved into pages of medical journals. He also sought second and third opinions from the nation’s leading children’s hospitals. All the results of his research pointed to the same treatment plan of placing a shunt to initiate blood flow to the right lung. This plan would require repeat surgeries throughout Carter’s life to up-size the shunt as he grew. One night, Malcolm was on his laptop and came across the Pulmonary Artery Reconstruction program at Lucile Packard Children’s Hospital Stanford. He shook Kelly awake and told her, “I think I found it.”

“I’m stubborn by nature,” he said, laughing. “I just felt there was something else for Carter.”

The next day, Kelly and Malcolm were on the phone with Jennifer Shek, a nurse practitioner in cardiothoracic surgery at Stanford Children’s Health. She told them Carter was a promising candidate for the program. The surgical team was led by Frank Hanley, MD, professor of cardiothoracic surgery and chief of pediatric cardiac surgery, and Doff McElhinney, MD, professor of cardiothoracic surgery and of pediatrics. Stanford Children’s Health has been recognized worldwide for using innovative approaches to surgical management of complex pulmonary artery procedures. The team revealed that Carter did, in fact, have a pulmonary artery, but it was in the wrong place. The doctors diagnosed Carter with discontinuous pulmonary artery resulting from a ductal origin. Their unique surgical approach to Carter’s case would restore his lung without requiring any long-term treatments. Kelly and Malcolm could barely believe their ears. 

Exceeding all expectations

Any parent who’s had to watch their child be taken away for surgery will tell you it’s one of the toughest things you can go through. But Kelly and Malcolm said the care they received at Packard Children’s helped them get through Carter’s surgery. “Everyone exceeded our expectations. We didn’t have one negative interaction,” Kelly said. “We received constant updates on Carter’s status, and we really felt empowered in how we played a role in his care.”

Carter had two repairs in all. In his first surgery, a shunt was implanted to establish blood flow to his right lung. The second surgery, which happened five months later, in November 2018, was the final artery construction in which Hanley removed the shunt and connected the grown pulmonary artery. During the five months between the two surgeries, Carter’s pulmonary artery grew to normal size, surpassing the doctors’ expectations.

Today, Carter is back home in Maryland and doing well. His hometown medical care team — from his pediatrician to his cardiologist — have communicated frequently with the Stanford team to ensure the boy’s care is fully coordinated.

“Dr. Hanley and his team have delivered on their promise that Carter will live a normal life,” Kelly said. “Dr. Hanley told us Carter will be repaired and he will run, play baseball and have no restrictions. As parents, that was our greatest hope.”



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