Coarctation of the aorta (CoA) with arterial tortuosity syndrome (ATS) is a rare hereditary condition characterized by elongation and tortuosity of the medium- and large-sized arteries, pulmonary hypertension, pulmonary artery stenosis, and constriction or narrowing of the aorta. Considering the non-specific indications and the overlap of ATS with other disorders of the connective tissue such as Williams Beuren syndrome (elastin), Marfan syndrome (fibrillin-1) and Ehlers Danlos syndrome (Type III collagen), it is vital to keep a high diagnostic suspicion in the analysis of the condition. We therefore report on a rare occurrence of coarctation of the aorta with arterial tortuosity syndrome in a teenage female who initially presented with severe resistant hypertension, providing additional literature to aid in the differential diagnosis of COA with ATS.
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