特纳综合征经皮主动脉狭窄支架置入术成功1例并文献复习

Q4 Medicine Revista Romana de Pediatrie Pub Date : 2021-09-30 DOI:10.37897/rjp.2021.3.6
D. Toma, Dorottya Miklósi, A. Sglimbea, L. Hadadi, M. Pop, Andreea Cerghit-Paler, Amalia Făgărășan, R. Togănel, L. Gozar
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引用次数: 0

摘要

特纳综合征(TS)是影响女性最常见的染色体异常,高达50%的患者有心脏异常。虽然缩窄是最常见的心脏畸形之一,但由于相关的主动脉病变和主动脉夹层的风险,这些患者的治疗方案一直存在争议。除了文献中发现的相互矛盾的数据外,关于TS患者主动脉缩窄支架置入的安全性和实用性,我们提出了一名儿童年龄的患者,他成功地接受了手术,没有围手术期和短期并发症。除了演示本身,我们的目的是回顾和总结关于这个主题的文献中可用的数据。综上所述,我们强调微创介入治疗的作用,并希望强调对这一患者群体进行进一步、更大规模的研究和指南的必要性。鉴于相关的主动脉病变,应采取一切预防措施,以避免在支架植入过程中主动脉夹层。虽然我们的患者的发展是有利的,但在文献中发现的数据有些矛盾,需要密切随访以帮助评估长期并发症的风险。
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Successful percutaneous stenting of coarctation of the aorta in Turner syndrome – a case report and literature review
Turner syndrome (TS) is the most common chromosomal abnormality affecting females and cardiac abnormalities have been described in up to 50% of patients. Although coarctation represents one of the most frequent cardiac malformation, treatment options in these patients represent an area of debate, due to associated aortopathy and risk for aortic dissection. In addition to the contradictory data found in the literature, regarding the safety profile and utility of stenting of coarctation of the aorta in TS patients, we present the case of a patient of pediatric age, who successfully underwent the procedure, being free from periprocedural and short-term complications. Beside the presentation itself, we aimed to review and summarize the data available in the literature regarding this topic. As a conclusion, we emphasize the role of minimally invasive interventional therapy and wish to underline the need of further, larger scale studies and guidelines in this patient group. Given the related aortopathy, all preventive measures should be undertaken to avoid aortic dissection during stent implantation in this vulnerable population. Although the evolution of our patient was favorable, data found in the literature is somewhat contradictory and a close follow-up is indicated to help evaluate the risk of long-term complications.
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CiteScore
0.10
自引率
0.00%
发文量
15
审稿时长
4 weeks
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