Waters Peadar S, Mitchell David P, Tawfick Wael, Hynes Niamh, Sultan Sherif
Western Vascular Institute, University College Hospital, Galway, Ireland.
Int J Surg Case Rep. 2013;4(1):130-3. doi: 10.1016/j.ijscr.2012.10.012. Epub 2012 Oct 29.
Aortic coarctation is a congenital defect which rarely presents in adulthood but results in significant morbidity and mortality. Endovascular techniques present novel therapeutic options for managing this anomaly with comparable results to traditional open surgical repair.
We convey a case of postductal aortic coarctation in a symptomatic 31-year-old woman, undetected, despite 27 years history of congenital bicuspid valve with aortic incompetence. We staged a successful endovascular repair following presentation with hypertension, abdominal pain, and worsening lower limb claudication. This lady recovered well, eventually giving birth to twins after a successful caesarean section delivery. The anatomical, clinical and technical features of the case are appraised.
Aortic coarctation is classified anatomically as preductal (infantile) or postductal (adult) and the morphological spectrum of abnormality ranges from a discrete stenosis distal to the left subclavian to a hypoplastic transverse arch and aortic isthmus presenting in infancy. Its incidence is 0.2-0.6 per 100 live births. Traditional open surgical repair of aortic coarctation is associated with high morbidity and mortality. Advances in endovascular techniques with their associated shorter hospital time and lower costs, reduced short term morbidity and mortality mean that they are likely to become mainstays of treatment.
The presentation of aortic coarctation for the first time in an adult is extremely unusual. Error and delayed diagnosis can never be fully exempted in the practice of medicine even with the most advanced diagnostic tools. Follow-up is mandatory by trans-oesophageal echo in all endovascular patients.
主动脉缩窄是一种先天性缺陷，在成年期很少出现，但会导致严重的发病率和死亡率。血管内技术为处理这种异常情况提供了新的治疗选择，其结果与传统的开放手术修复相当。
我们讲述了一名31岁有症状女性的动脉导管后主动脉缩窄病例，尽管她有27年先天性二叶式主动脉瓣伴主动脉瓣关闭不全的病史，但此前一直未被发现。在出现高血压、腹痛和下肢间歇性跛行加重后，我们成功进行了分期血管内修复。这位女士恢复良好，最终在剖宫产成功分娩后产下一对双胞胎。对该病例的解剖学、临床和技术特征进行了评估。
主动脉缩窄在解剖学上分为动脉导管前型（婴儿型）或动脉导管后型（成人型），异常的形态学范围从左锁骨下动脉远端的局限性狭窄到婴儿期出现的发育不全的横弓和主动脉峡部。其发病率为每100例活产中有0.2 - 0.6例。传统的主动脉缩窄开放手术修复与高发病率和死亡率相关。血管内技术的进步及其相关的住院时间缩短和成本降低、短期发病率和死亡率降低，意味着它们可能会成为主要的治疗方法。
主动脉缩窄首次在成人中出现极为罕见。即使使用最先进的诊断工具，在医学实践中也永远无法完全避免误诊和诊断延迟。所有接受血管内治疗的患者都必须通过经食管超声进行随访。
