Pseudocoarctation of the aorta associated with left vertebral artery hypoplasia

Introduction: Pseudocoarctation of the aorta is a relatively rare anomaly, defined as an elongation of the aortic arch, with kinking or buckling of the aorta at the level of the ligament arteriosus and with narrowing of the aortic isthmus without a pressure gradient across the lesion and without formation of collateral circulation. Vertebral artery hypoplasia is a congenital variation of the posterior circulation, defined as a vessel with a diameter in the entire course of less than 2 mm, respectively less than 3 mm, or with a side difference equal or greater than 1:1.7, according to different authors. Methods: We report the case of a 51 years old woman, with a history of cardiovascular risk factors (ex-smoker, hypertension, dyslipidemia, diabetes mellitus type 2, obesity) who was admitted for headaches, dizziness, vertigo, short episodes of blurred vision and hearing disturbances (tinnitus and hypoacusis) and left arm paresthesia. The physical examination revealed a difference in blood pressure between arms (right – 150/100 mm Hg; left – 110/80 mm Hg) and a murmur in the left supraclavicular area. Results: The electrocardiogram was normal and the chest X-ray revealed elongation of the aortic arch. On transthoracic echocardiography we found a turbulent flow at the junction between the aortic arch and the descending aorta, with increased velocities at this level (2,64 m/s). Magnetic resonance angiography showed a reduction of the aortic diameter in the isthmic region. The aortic arch angiography showed an area of significant kinking at the junction between the aortic arch and the descending aorta, without a pressure gradient between the two segments. The left subclavian artery emerged from the abnormal segment of the aorta and had prominent tortuosity in the proximal region, without presenting stenosis. Also, the left vertebral artery seemed hypoplasic. Conclusions: We attribute the neurological symptoms described by our patient to vertebrobasilar insufficiency caused by the hypoplastic left vertebral artery. Concerning the difference in blood pressure between the arms and the left arm sensitivity disturbances, we hypothesize that they are the consequence of left subclavian artery emergence at the level of the pseudocoarctation, combined with important tortuosity and kinking of its proximal segment.

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