Luke Wiggins, MD, congenital heart surgeon with the Heart Institute at Children's Hospital Los Angeles. Credit: Children's Hospital Los Angeles
When a critically ill baby with dextro-transposition of the great arteries (D-TGA) was transferred to Children's Hospital Los Angeles last year—just hours after birth—the Heart Institute team saw right away that the case was anything but routine.
As in all cases of D-TGA, the baby's pulmonary artery and aorta were switched. But their positions were also levo-malposed, making the surgical repair more complex. What's more, the coronary arteries originated from one location, not two—and were in a difficult spot that would make the surgery much more challenging.
With the baby in critical condition and needing full mechanical ventilation, the team performed an arterial switch operation the next day—the baby's second day of life. CHLA congenital heart surgeon Luke Wiggins, MD, led the team and recently published a case report on the successful surgery in the World Journal for Pediatric and Congenital Heart Surgery.
Dr. Wiggins shares how the team succeeded with this complex case—particularly the challenging coronary artery transfer part of the operation.
What were the challenges of this baby's unique anatomy?
Multiple challenges made this much more complex than a standard arterial switch. One of them was that the great arteries were levo-malposed. In typical D-TGA, the aorta is in front and to the right of the pulmonary artery. In this case, the aorta was in front and to the left.
That meant that the arteries were farther away from where we needed to connect them. So they were more at risk of being stretched or compressed during the repair.
The biggest challenge, though, was the rare coronary artery pattern. Normally, the coronary arteries originate from two nearby places at the base of the aorta, which makes it easier to transfer them during the arterial switch.
In this case, though, the right coronary artery and the left anterior descending artery both came from a single opening, which was more anterior and to the right. We had to move those arteries to the new aorta, but the starting point was far from where they needed to be attached, and it was a unique angle.
The baby also had other defects, including a ventricular septal defect, a patent ductus arteriosus, and a patent foramen ovale.
How critical was the coronary artery transfer? How did the team approach this?
The coronary transfer is a big key to success for the arterial switch. In this case, we had to use some alternative techniques to work around the baby's unique anatomy.
One of the important things we did was to use a J-shaped medial-based trapdoor incision in the neoaortic root. This flap—of native vessel tissue—gave us more room and flexibility to move the coronary arteries and gently angle them into place without any twisting or compression, which is critical for ensuring good blood flow.
We also chose not to do the standard LeCompte maneuver (moving the pulmonary arteries in front of the aorta) because it would have risked compressing or interfering with these unusually placed coronary arteries. Keeping the pulmonary arteries behind the new aorta gave them more space and protection.
How did the baby do after surgery?
The baby did great and went home 18 days after surgery. Transposition of the great vessels is one of the most rewarding heart defects to take care of because a successful operation essentially restores normal cardiac anatomy and does not require further surgery. Following a good anatomic repair, the long-term outlook for these babies is fantastic.
What are the key takeaways from this case?
The biggest takeaway is that, even with rare coronary patterns and unique vessel positions, you can safely perform a full anatomic repair. But babies should be treated at a high-volume center like CHLA with extensive experience in neonatal cardiac surgery. At CHLA, we do more than 800 heart surgeries annually on average, and more than half of our neonatal heart surgeries fall into the most complex categories.
Surgical expertise is also just one piece of the puzzle. Our whole Heart Institute team—surgeons, cardiologists, echocardiographers, Cardiothoracic Intensive Care Unit physicians and nurses, pediatric cardiac anesthesiologists, and more—all worked together to make this case a success. It's the experience of the whole team that leads to these excellent outcomes for babies with complex congenital heart disease.
More information: Mark B. Lewis et al, A Coronary Challenge: Management of Dextro-Transposition With Levo-Malposed Great Vessels, World Journal for Pediatric and Congenital Heart Surgery (2025). DOI: 10.1177/21501351251322158
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