Rare Causes of Arterial Hypertension and Thoracic Aortic Aneurysms-A Case-Based Review.

ABSTRACT: Thoracic aortic aneurysms may result in dissection with fatal consequences if undetected. A young male patient with no relevant familial history, after having been investigated for hypertension, was diagnosed with an ascending aortic aneurysm involving the aortic root and the proximal tubular segment, associated with a septal atrial defect. The patient underwent a Bentall surgery protocol without complications. Clinical examination revealed dorso-lumbar scoliosis and no other signs of underlying connective tissue disease. Microscopic examination revealed strikingly severe medial degeneration of the aorta, with areas of deep disorganization of the medial musculo-elastic structural units and mucoid material deposition. Genetic testing found a variant of unknown significance the PRKG1 gene en

SUBMITTER: Encica S 

PROVIDER: S-EPMC8001303 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature