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Introduction: Children with congenital heart disease are at increased risk for stroke due to large vessel occlusion. Mechanical thrombectomy is standard of care for treatment of large vessel occlusion in adults; however, appropriate protocols for children, particularly those with complex cardiovascular anatomy and hemodynamics, are not well established.Methods: We report 3 cases of children with known cardiac disease who presented with acute stroke symptoms and underwent thrombectomy between May-July 2017. Baseline characteristics, stroke presentation, endovascular treatment, complications and outcomes of each case were reviewed, along with the cardiac anatomy and cardiopulmonary status of each patient.Results: Case details are reported in Table 1. All three patients had an M1 occlusion and minimal to absent early infarct signs on initial CT. Thrombolysis in Cerebral Infarction (TICI) score of 2C or 3 was achieved and small final stroke burden sustained in all cases (Figure 1). Cardiopulmonary status caused significant complications leading to death for one patient, and necessitated alterations in standard neuroprotective measures for another.Conclusion: Children with genetic or congenital cardiac disease represent a significant subset of pediatric stroke patients amenable to endovascular therapy. Our cases highlight complications which may arise when treating these anatomically complex and often hemodynamically tenuous patients. These children represent an important subgroup for whom specific endovascular pathways should be developed.