Modified Nikaidoh procedure with double-root translocation in a 1-year-old boy

Area of Science:

• Pediatric Cardiac Surgery
• Congenital Heart Disease Repair
• Cardiovascular Surgery

Background:

• Transposition of the great arteries (TGA) is a critical congenital heart defect requiring complex surgical correction.
• Left ventricular outflow tract (LVOT) obstruction, often associated with TGA, presents significant surgical challenges.
• Bicuspid pulmonary valves with stenosis require careful management during TGA repair.

Observation:

• A 1-year-old boy with TGA, VSD, and severe LVOT obstruction (moderate pulmonary stenosis, z-score -4.4) underwent a modified Nikaidoh procedure.
• The procedure utilized a valve-spared pulmonary root with double root translocation.
• Postoperative assessment included echocardiography to evaluate repair effectiveness.

Findings:

• Successful biventricular repair was achieved, resolving LVOT obstruction and aortic valve regurgitation.
• Mild pulmonary stenosis and regurgitation were noted postoperatively.
• No reintervention was necessary during a 6-year follow-up period.
• Annular growth of the pulmonary valve was observed, indicating favorable long-term development.

Implications:

• The modified Nikaidoh procedure offers a viable option for complex TGA with LVOT obstruction, particularly when valve sparing is desired.
• Valve-sparing techniques in congenital heart surgery can lead to favorable long-term outcomes and reduce the need for reoperations.
• This approach highlights the potential for excellent functional recovery and growth in pediatric patients with complex cardiac anomalies.