Adult interrupted aortic arch revealed by resistant arterial hypertension

IF 18 Q4 Medicine
A. Boutaled , M. Tabat , Y. Mekouar , G. Bennani , A. Drighil , G. Lembarki
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引用次数: 0

Abstract

Introduction

Interrupted aortic arch (IAA) is a rare congenital heart condition where there is a complete discontinuation between the ascending and descending aorta. The association with a patent ductus arteriosus or developed arterial collateral allows survival until adulthood in extremely rare cases. Transthoracic echocardiography is helpful for the diagnosis, but the exact interruption site and collateral vessels are more accurately highlighted by computed tomography angiography (CTA) and magnetic resonance imaging (MRI).

Results/Expected results

A 50 year-old-patient with a history of hypertension was admitted for severe high blood pressure. The main functional symptom that prompted his consultation was a grade II NYHA dyspnea evolving for few weeks. Physical examination found a blood pressure of 190/110 mmHg at the right arm, 120/70 mmHg at the left arm, and 110/70 mmHg at lower limbs. Cardiac and pulmonary auscultation were normal. The electrocardiogram showed left ventricular hypertrophy. Transthoracic echocardiography revealed severe left ventricular hypertrophy with good left ventricular function and dilatation of the ascending aorta measured at 47 mm. The suprasternal notch view suggested a type B IAA. CTA confirmed the diagnosis, exhibiting an IAA located between the left carotid and left subclavian artery with an arterial collateral network emerging from descending branch of the transverse cervical artery (Figure 1).

Conclusion/Perspectives

Adult forms of IAA are a rare and complex manifestation of a congenital heart condition. They present significant challenges due to the severity of symptoms and high operative risk involved in treatment. An accurate and timely diagnosis is essential and can be achieved through a combination of echocardiography and advanced imaging techniques such as CTA or MRI angiography. A multidisciplinary approach involving a team of specialists is crucial for optimizing the management of these patients.

成人主动脉弓中断表现为顽固性高血压
主动脉弓中断(IAA)是一种罕见的先天性心脏病,在升主动脉和降主动脉之间存在完全中断。合并动脉导管未闭或动脉侧枝发育的病例极少存活至成年。经胸超声心动图有助于诊断,但计算机断层血管造影(CTA)和磁共振成像(MRI)更准确地显示确切的中断部位和侧支血管。结果/预期结果患者50岁,有高血压病史,因严重高血压入院。促使他就诊的主要功能症状是持续数周的II级NYHA呼吸困难。体检发现右臂血压190/110 mmHg,左臂血压120/70 mmHg,下肢血压110/70 mmHg。心肺听诊正常。心电图显示左室肥厚。经胸超声心动图显示左心室严重肥厚,左心室功能良好,升主动脉扩张47mm。胸骨上切迹显示为B型IAA。CTA证实了诊断,显示IAA位于左颈动脉和左锁骨下动脉之间,颈横动脉降支出现动脉侧支网络(图1)。结论/观点成人形式的IAA是先天性心脏病罕见且复杂的表现。由于症状的严重性和治疗的高手术风险,它们提出了重大的挑战。准确和及时的诊断是必不可少的,可以通过超声心动图和先进的成像技术如CTA或MRI血管造影相结合来实现。涉及专家团队的多学科方法对于优化这些患者的管理至关重要。
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来源期刊
Archives of Cardiovascular Diseases Supplements
Archives of Cardiovascular Diseases Supplements CARDIAC & CARDIOVASCULAR SYSTEMS-
自引率
0.00%
发文量
508
期刊介绍: Archives of Cardiovascular Diseases Supplements is the official journal of the French Society of Cardiology. The journal publishes original peer-reviewed clinical and research articles, epidemiological studies, new methodological clinical approaches, review articles, editorials, and Images in cardiovascular medicine. The topics covered include coronary artery and valve diseases, interventional and pediatric cardiology, cardiovascular surgery, cardiomyopathy and heart failure, arrhythmias and stimulation, cardiovascular imaging, vascular medicine and hypertension, epidemiology and risk factors, and large multicenter studies. Additionally, Archives of Cardiovascular Diseases also publishes abstracts of papers presented at the annual sessions of the Journées Européennes de la Société Française de Cardiologie and the guidelines edited by the French Society of Cardiology.
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