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主动脉缩窄的诊断、影像学检查及临床管理

Diagnosis, imaging and clinical management of aortic coarctation.

作者信息

Dijkema Elles J, Leiner Tim, Grotenhuis Heynric B

机构信息

Department of Pediatric Cardiology, Wilhelmina Children's Hospital, The University of Utrecht, Utrecht, The Netherlands.

Department of Radiology, University Medical Centre Utrecht, Utrecht, The Netherlands.

出版信息

Heart. 2017 Aug;103(15):1148-1155. doi: 10.1136/heartjnl-2017-311173. Epub 2017 Apr 4.

Abstract

Coarctation of the aorta (CoA ) is a well-known congenital heart disease (CHD) , which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic screening, prenatal diagnosis of C o A remains difficult. Most patients with CoA present in infancy with absent, delayed or reduced femoral pulses, a supine arm-leg blood pressure gradient (> 20 mm Hg), or a murmur due to rapid blood flow across the CoA or associated lesions (BAV). Transthoracic echocardiography is the primary imaging modality for suspected CoA. However, cardiac magnetic resonance imaging is the preferred advanced imaging modality for non-invasive diagnosis and follow-up of CoA. Adequate and timely diagnosis of CoA is crucial for good prognosis, as early treatment is associated with lower risks of long-term morbidity and mortality. Numerous surgical and transcatheter treatment strategies have been reported for CoA. Surgical resection is the treatment of choice in neonates, infants and young children. In older children (> 25 kg) and adults, transcatheter treatment is the treatment of choice. In the current era, patients with CoA continue to have a reduced life expectancy and an increased risk of cardiovascular sequelae later in life, despite adequate relief of the aortic stenosis. Intensive and adequate follow-up of the left ventricular function, valvular function, blood pressure and the anatomy of the heart and the aorta are , therefore, critical in the management of CoA. This review provides an overview of the current state-of-the-art clinical diagnosis, diagnostic imaging algori thms, treatment and follow-up of patients with CoA.

摘要

主动脉缩窄(CoA)是一种众所周知的先天性心脏病(CHD),常与其他几种心脏和血管异常相关,如二叶式主动脉瓣(BAV)、室间隔缺损、动脉导管未闭和主动脉弓发育不全。尽管进行了超声心动图筛查,但CoA的产前诊断仍然困难。大多数CoA患者在婴儿期出现股动脉搏动缺失、延迟或减弱,仰卧位时上肢与下肢血压梯度(>20 mmHg),或因CoA或相关病变(BAV)处血流速度加快而出现杂音。经胸超声心动图是疑似CoA的主要影像学检查方法。然而,心脏磁共振成像是非侵入性诊断和CoA随访的首选高级影像学检查方法。CoA的充分及时诊断对良好预后至关重要,因为早期治疗与较低的长期发病率和死亡率风险相关。已有许多针对CoA的手术和经导管治疗策略的报道。手术切除是新生儿、婴儿和年幼儿童的首选治疗方法。对于年龄较大的儿童(>25 kg)和成人,经导管治疗是首选治疗方法。在当今时代,尽管主动脉缩窄得到了充分缓解,但CoA患者的预期寿命仍然缩短,晚年发生心血管后遗症的风险增加。因此,对左心室功能、瓣膜功能、血压以及心脏和主动脉解剖结构进行强化和充分的随访,对于CoA的管理至关重要。本综述概述了CoA患者当前的临床诊断、诊断成像算法、治疗及随访的最新情况。

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