Arterial tortuosity syndrome is a rare connective tissue disorder characterised by elongation, tortuosity, stenosis and aneurysms of the large and middle-sized arteries. The symptomatology is correlated to the artery affected by the pathology with correlated stenosis. We describe our hybrid surgical procedure in the treatment of a case of kinking of the pulmonary branches with significant gradient and hypertension. Aortic arch and supraaortic vessels presented various deviousness without hemodynamic alterations.
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