Extremely late presentation of children with transposition the great arteries

Area of Science:

• Pediatric Cardiology
• Congenital Heart Surgery
• Cardiac Physiology

Background:

• Transposition of the great arteries (TGA) with intact ventricular septum is a critical congenital heart defect.
• Late presentation of TGA necessitates complex surgical interventions.
• Arterial switch operation (ASO) is the gold standard for TGA repair.

Observation:

• Three cases of extremely late presenting TGA with intact ventricular septum underwent primary arterial switch operation.
• Patients included a 7-year-old female, a 3-year-old male, and a 6-year-old female.
• Two patients were discharged on postoperative days 9 and 11, respectively.

Findings:

• One patient developed severe hemodynamic instability 12 hours post-surgery, succumbing to left ventricular failure and pulmonary hypertension.
• Specific criteria for primary ASO include left ventricular mass index >35 g·m⁻² and left ventricular posterior wall >4 mm.
• Two survivors did not require extracorporeal membrane oxygenation or nitric oxide support.

Implications:

• Careful patient selection based on ventricular criteria is crucial for successful primary ASO in late-presenting TGA.
• Intraoperative or postoperative circulatory support may improve outcomes in select high-risk patients.
• Further research is needed to optimize surgical strategies for late-presenting TGA to reduce mortality.