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Surgical pulmonary artery reconstruction in patients with Arterial Tortuosity Syndrome (ATS) has excellent outcomes. In this study, we report our late outcomes after more than a decade of experience with such complex interventions. We conducted a retrospective review of 33 ATS patients who underwent pulmonary artery reconstruction. The mean pre-operative right ventricular/left ventricular pressure (RVp/LVp) ratio was 1.19 ± 0.2. Our surgical approach included either a single-stage complete repair through a median sternotomy (17 patients