Congenital kinking of aorta – A rare aortic arch anomaly

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Congenital kinking of aorta or pseudocoarctation is a rare anomaly of the aortic arch with elongation and kinking of aorta at the level of ligamentum arteriosum. This anomaly is not a true coarctation as there is no significant hemodynamic obstruction or pressure gradient across the lesion. We report two cases with this anomaly diagnosed during the evaluation for incidental finding of murmur. Both patients had flow gradient across the kinked segment of aorta by echocardiography but did not require any intervention. Long term complication is aneurysmal dilatation and rupture beyond the kinked segment. Computed tomography of the aorta is a simple noninvasive diagnostic modality for the definitive diagnosis. Surgical repair should be performed for all symptomatic individuals. Regular periodic follow up is mandatory for all asymptomatic patients. Keywords: Aortic arch syndromes, Aortic coarctation, Congenital heart defects, Echocardiography, Thoracic aorta