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Background: Two-stage surgery including right ventricular outflow tract (RVOT) stenting with subsequent total surgical repair (TSG) has been suggested as a promising curative option in infants with tetralogy of Fallot (ToF) having comorbidities such as low body weight. However, data on clinical outcomes of such approach and tissue response to RVOT stenting in underweight infants are scarce.Methods: We recruited 16 underweight (<3 kg; average weight, 2.2 ± 0.4 and 4.7 ± 0.9 kg at the time of RVOT stenting and TSG, respectively) infants (1-3 months of age, average 28.2 ± 4.3 and 100.2 ± 22.3 days at the time of RVOT stenting and TSG, respectively) with ToF and performed RVOT stenting with the subsequent TSG. Excised stents were embedded into epoxy resin and stained by toluidine blue and basic fuchsin.Results: Fifteen infants had a favorable clinical outcome, probably due to the rapid increase in the body weight, blood oxygen saturation, and left ventricular end-diastolic volume to body surface area ratio indicative of improved pulmonary perfusion. Histological analysis revealed an endothelial cell monolayer at the stent surface with notable neovascularization of stented tissues, which could potentially explain the abovementioned clinical and echocardiography improvements. The only death occurred immediately after RVOT stenting and was caused by a massive subdural hematoma, possibly provoked by grade 2 intraventricular hemorrhage 12 days before the stenting.Conclusions: We confirm RVOT stenting with the subsequent TSG as a safe and efficient surgical approach for the treatment of underweight children with ToF. [ABSTRACT FROM AUTHOR]