The Challenge of Pediatric Heart Transplantation in a Failing Fontan Patient With Dextrocardia and Heterotaxy Syndrome

Patients with dextrocardia, heterotaxy syndrome, and single ventricle physiology typically undergo multiple staged palliative operations culminating in a Fontan circulation. Despite these palliative procedures, heart transplantation may become the inevitable final option.We report the case of a 12-year-old female with dextrocardia, heterotaxy syndrome with right atrial isomerism, and single ventricle physiology who had previously undergone staged Fontan palliation. Due to progressive Fontan failure and clinical deterioration, she was evaluated and listed for OHT.She underwent successful OHT without intraoperative complications. Her postoperative course was uneventful, and she showed significant clinical improvement during follow-up.This case highlights that heart transplantation is a viable and life-saving treatment option for patients with complex congenital heart disease following Fontan failure. Although transplantation in patients with dextrocardia poses technical challenges, it can be performed safely with favorable outcomes.