Abstract:
Coarctation of the aorta (CoA) with arterial tortuosity syndrome (ATS) is a rare hereditary condition characterized by elongation and tortuosity of the medium- and large-sized arteries, pulmonary hypertension, pulmonary artery stenosis, and constriction or narrowing of the aorta. Considering the non-specific indications and the overlap of ATS with other disorders of the connective tissue such as Williams Beuren syndrome (elastin), Marfan syndrome (fibrillin-1) and Ehlers Danlos syndrome (Type III collagen), it is vital to keep a high diagnostic suspicion in the analysis of the condition. We therefore report on a rare occurrence of coarctation of the aorta with arterial tortuosity syndrome in a teenage female who initially presented with severe resistant hypertension, providing additional literature to aid in the differential diagnosis of COA with ATS. Cinematic rendering delivered a markedly enhanced view of the condition, allowing for greater accuracy in the diagnosis.
摘要:
主动脉缩窄(CoA)伴动脉迂曲综合征(ATS)是一种罕见的遗传性疾病,其特征是中型和大型动脉的伸长和迂曲,肺动脉高压,肺动脉狭窄,主动脉收缩或变窄。考虑到非特异性适应症以及ATS与其他结缔组织疾病如WilliamsBeuren综合征(弹性蛋白)的重叠,马凡综合征(纤丝蛋白-1)和EhlersDanlos综合征(III型胶原),在分析病情时保持高度的诊断怀疑是至关重要的。因此,我们报告了在最初患有严重顽固性高血压的青少年女性中罕见的主动脉缩窄伴动脉曲折综合征,提供更多文献以帮助鉴别诊断COA与ATS。电影渲染提供了一个显著增强的视图的条件,允许更准确的诊断。