Journal
INTERACTIVE CARDIOVASCULAR AND THORACIC SURGERY
Volume 33, Issue 5, Pages 819-821Publisher
OXFORD UNIV PRESS
DOI: 10.1093/icvts/ivab171
Keywords
Hunter syndrome; Aortic root dilatation; Aortic root replacement
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Hunter syndrome is a rare disease that can lead to glycosaminoglycan accumulation in tissues, affecting multiple organs. Prognosis is mainly influenced by cardiac and respiratory failures. Patients with Hunter syndrome may have a risk of aortic root dilatation, necessitating periodic monitoring with diagnosis based on indexed measures.
Hunter syndrome is a rare disease leading to glycosaminoglycan accumulation in tissues. Multiple organs are involved, but prognosis is mainly conditioned by cardiac and respiratory failures. Cardiac valvular impairment is quite common but aortic root dilatation is rarely described. This article covers a case of surgical root replacement due to aortic valve insufficiency and aortic root dilatation documented with magnetic resonance and computed tomography angiographies. Anatomic pathology reported both aortic valve and aorta with mucoid overload and elastic fibre depletion. These patients do have a risk of aortic root dilatation, which justifies periodic monitoring. Diagnosis must be made using indexed measures.
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