Innovative Surgical Repair of a Rare Dextro-Transposition of the Great Arteries

In a remarkable case at Children’s Hospital Los Angeles, a specialized surgical team successfully repaired a rare and complex variation of dextro-transposition of the great arteries (D-TGA) in a critically ill newborn. Transposition of the great vessels is a congenital heart defect where the pulmonary artery and aorta are switched, disrupting normal blood flow. Typically, the arteries are in their standard positions, but in this infant, the arteries were also levo-malposed, adding a significant layer of complexity to the procedure.

The newborn arrived at CHLA just hours after birth, requiring immediate intensive care and mechanical ventilation. The surgical team faced unique anatomical challenges: the aorta was positioned in front and to the left rather than its usual location, and both the right coronary artery and the left anterior descending artery originated from a single, anterior, and rightward opening. These anomalies made standard arterial switch techniques insufficient.

The team performed an arterial switch operation within the first two days of life, led by renowned congenital heart surgeon Dr. Luke Wiggins. The procedure demanded innovative techniques, including the use of a J-shaped trapdoor incision in the neoaortic root. This approach provided extra flexibility, allowing precise transfer of the coronary arteries while preventing twisting or compression, which are critical for maintaining proper blood flow.

Additionally, the team opted against the common LeCompte maneuver to avoid risks of coronary artery compression. Instead, they positioned the pulmonary arteries behind the aorta, ensuring ample space and protection for the transferred coronary arteries.

Postoperative recovery was successful; the infant was discharged home 18 days after the surgery. The procedure’s success highlights that even with rare anatomical variations, complex congenital heart defects can be effectively treated at high-volume specialized centers. The comprehensive team effort, encompassing surgeons, cardiologists, anesthesiologists, and intensive care staff, played a vital role in achieving these excellent outcomes.

This case underscores the importance of surgical expertise and teamwork in managing complex neonatal heart conditions. It also emphasizes that individualized, innovative surgical approaches can overcome unique anatomical challenges, offering long-term hope for affected infants.

For more detailed insights, the case was published in the World Journal for Pediatric and Congenital Heart Surgery.