| Sumario: | Objective and Aims: Pediatric heart transplantation (HTx) for children with single-ventricle (SV) physiology remains a complex and high-risk procedure. This study aimed to retrospectively evaluate long-term outcomes in patients with SV undergoing primary HTx versus HTx after failed staged palliation. Methods: Between 1991 and 2025, 22 pediatric patients (aged ≤ 18 years) with SV physiology underwent orthotopic HTx. Thirteen received primary HTx (Group 1), and nine had HTx after failed staged palliation (Group 2), including two post-Norwood, three post-Glenn, and four post-Fontan procedures. Results: The 10-year survival rate was 71.6%, with similar re-transplant-free survival. The median transplant age was 0.22 years (interquartile range [IQR], 0.08–0.49) for Group 1 and 15.1 years (IQR, 3.0–16.5) for Group 2. Posttransplant extracorporeal membrane oxygenation was needed in three patients in Group 1 and six patients in Group 2. Coronary vasculopathy (grades I–III) occurred in eight patients in Group 1 and 5 patients in Group 2; one patient in each group underwent re-HTx. Cytomegalovirus positivity was found in three and one patients; Epstein–Barr virus positivity in six and two patients (Groups 1 and 2, respectively). Rejection (grades 0–II) occurred in 7 and 2 patients, respectively. Posttransplant lymphatic disorder developed in four patients (Group 1). Immunosuppression was changed in 10 patients (Group 1) and 6 patients (Group 2). Three patients in Group 1 required pacemakers. Conclusions: HTx is feasible for pediatric SV patients. Primary Tx in infancy may involve shorter operative times, whereas HTx after failed palliation, especially post-Fontan, is more complex and associated with higher morbidity. Early referral and tailored treatment are essential. Multicenter prospective studies are needed to improve patient selection and address donor availability.
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