Pulmonary valve

Jimmy Kimmel’s son: What is Tetralogy of Fallot?

In a rare emotional monologue on his ABC show, Jimmy Kimmel opened up about the birth of his son and the scary diagnosis he received just hours after birth.

USA TODAY

Raise your hand if you had never heard of Tetralogy of Fallot with pulmonary atresia before Monday’s episode of ABC’s Jimmy Kimmel Live.

Awareness of this serious congenital heart defect got a big bump after the late-night host shared the story of his newborn son’s diagnosis and open-heart surgery.

Kimmel choked up as he told the audience how, a few hours after baby Billy’s relatively trouble-free delivery on April 21, a “very attentive” nurse detected a heart murmur (which is somewhat common in newborns) and observed that his skin appeared purple (which was not normal).

“They determined he wasn’t getting enough oxygen into his blood,” Kimmel recounted, “which, as far as I understand, is most likely one of two things: either his heart or his lungs.”

A chest X-ray revealed that Billy’s lungs were fine, “which meant his heart wasn’t.”

Later that night, a pediatric cardiologist diagnosed Billy with Tetralogy of Fallot (TOF) with pulmonary atresia, a severe variety of a combo pack of four related congenital heart defects named for Étienne-Louis Arthur Fallot, the French doctor who identified the disease’s four defining traits in 1888.

TOF affects one in 2,500 newborns; the pulmonary atresia variety affects less than 20% of that number.

Children with TOF have all four cardiac defects in varying degrees but in Billy’s case, the two most serious problems are a completely obstructed pulmonary valve or artery (atresia) and a hole between his left and right ventricles (ventricular septal defect).

“The pulmonary valve is the aspect that needs immediate attention,” explains Dr. Nicolas Madsen, an assistant professor of pediatric cardiology at Cincinnati Children’s Hospital and vice-chair of the Medical Advisory Board for the Pediatric Congenital Heart Association.

“Without flow through the pulmonary valve, there’s only one other way for blood to get into the lungs, and that’s through a blood vessel called the ductus arteriosus that’s wide open during pregnancy to allow blood to skip the lungs, since the placenta provides all the oxygen. But once you’re born and need all of the blood going to the lungs, that connection between the…