When the Patient with TGA Has Pulmonary Stenosis

Johns Hopkins pediatric cardiac surgeon Luca Vricella had seen too many patients like the one before him. Born with transposition of the great arteries (TGA), in which the aorta is connected to the right ventricle and the pulmonary artery to the left—opposite a normal heart’s anatomy—the infant was severely cyanotic due to the lack of normal blood flow and oxygen to the lungs. Vricella knew the standard approach for TGA, the Rastelli procedure, has resulted in good outcomes with low mortality. However, Vricella also knew that one-third of infants with TGA, including his patient, are also born with pulmonary stenosis and would likely need additional surgeries later in life.

“The Rastelli procedure requires an artificial conduit that unfortunately does not grow with the child,” says Vricella. “Long term, these patients can have problems that require surgical reintervention of the left ventricular outflow track, which carries blood and oxygen to the great arteries.”

Vricella knew of another surgical option for this patient, one developed by cardiac surgeon Hisashi Nikaidoh, which has been shown to avoid the need for an artificial conduit and additional surgeries. However, the Nikaidoh approach is a more complicated and technically challenging surgery, Vricella notes, and one that unfortunately not many pediatric cardiac surgeons have training or experience in performing. A year ago, Vricella decided he wanted to be one of them and reached out to Nikaidoh at another children’s hospital.

“There I learned firsthand his approach has the advantage of realigning the left ventricle with the aorta in a different way with less potential for obstruction, restoring normality and avoiding reintervention long term,” says Vricella.

Now that a patient with both TGA and pulmonary stenosis had found Vricella, he offered the Nikaidoh approach to the family. They agreed. To ensure the best possible outcome, Vricella conducted a week-long training exercise with fellow pediatric cardiac surgeon Narutoshi Hibino and the entire cardiac surgery team to prepare them for the procedure. Then he went a step further and invited Nikaidoh to be a consulting surgeon during the operation. Nikaidoh obliged.

“Dr. Nikaidoh made this a true educational experience across our unit—he presented a lecture on the potential pitfalls of the procedure and looked over our shoulders during the surgery, coaching us on the operation he created,” says Vricella. “Dr. Nikaidoh is a true gentleman and scholar, one of the unsung heroes in our field.”

The outcome of the five-hour surgery?

“He’s a healthy pink child with a perfect repair,” says Vricella. “Needless to say, this experience opens our door to patients with both transposition of the great arteries and pulmonary stenosis.”