Malakan Rad Elaheh, Pouraliakbar Hamid Reza
Department of Pediatric Cardiology, Children's Hospital Medical Center (Pediatric Center of Excellence), Tehran University of Medical Sciences, Tehran, Iran.
Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran.
Ann Pediatr Cardiol. 2019 May-Aug;12(2):117-129. doi: 10.4103/apc.APC_74_18.
Isolation of the left brachiocephalic artery (ILBA) is an extremely rare anomaly of aortic arch with diverse manifestations in the neurologic system, heart, and left upper arm. This anomaly is defined as the absence of connection of the left brachiocephalic artery (LBA) to aortic arch and connection of LBA to pulmonary artery (PA) through a patent arterial duct (PAD). However, this definition is not inclusive of all cases. Not only are there inconsistencies in the definition and terminology of this aortic arch anomaly but also there is no classification for this anomaly despite its heterogeneous nature in terms of anatomy, clinical presentation and prognosis. We performed a 52-year comprehensive literature review in the period between 1966 and 2018. Our inclusion criteria were any manuscript that included a case report or case series, with confirmed diagnosis of ILBA. All quantitative data were analyzed using descriptive analysis by SPSS version 21 (IBM SPSS Statistics, USA). Results were presented as mean ± standard deviation and median. Based on the presence or absence of connection of LBA to PA and the number of sources of steal from the LBA, we classified ILBA into three types: single-steal type with no connection of LBA to PA and single source of blood flow steal from LBA through the left subclavian artery (LSCA), double-steal type with connection of LBA to PA through PAD and two sources of steal through LSCA and arterial duct (AD), and triple-steal type with bilateral PADs and therefore, three sources of blood flow steal from LBA including the LSCA and the double ADs. Patients with single-steal type have the best prognosis and present latest with symptoms of cerebrovascular insufficiency or left arm claudication. The oldest reported patient was 69 years of age with symptoms of dizziness and near syncope. No death was reported in these patients. Double-steal type is the most common type and is often associated with genetic syndromes and/or extracardiac anomalies. Triple-steal type is the rarest type with the earliest presentation and worst prognosis. The oldest reported patient was 60 days of age. All reported cases had cardiac symptoms, pulmonary overcirculation, pulmonary hypertension, and fatal outcome.
左头臂干动脉孤立（ILBA）是一种极为罕见的主动脉弓异常，在神经系统、心脏和左上臂有多种表现。这种异常被定义为左头臂干动脉（LBA）与主动脉弓无连接，且LBA通过开放的动脉导管（PAD）与肺动脉（PA）相连。然而，这个定义并不涵盖所有病例。这种主动脉弓异常不仅在定义和术语上存在不一致，而且尽管其在解剖结构、临床表现和预后方面具有异质性，但却没有针对该异常的分类。我们在1966年至2018年期间进行了为期52年的全面文献综述。我们的纳入标准是任何包含病例报告或病例系列且确诊为ILBA 的手稿。所有定量数据均使用SPSS 21版（美国IBM SPSS Statistics）进行描述性分析。结果以均值±标准差和中位数表示。根据LBA与PA是否连接以及LBA盗血来源的数量，我们将ILBA分为三种类型：单盗血型，LBA与PA无连接，通过左锁骨下动脉（LSCA）从LBA有单一血流盗血来源；双盗血型，LBA通过PAD与PA连接，通过LSCA和动脉导管（AD）有两个盗血来源；三盗血型，有双侧PAD，因此从LBA有三个血流盗血来源，包括LSCA和双侧AD。单盗血型患者预后最佳，出现脑血管供血不足或左臂间歇性跛行症状最晚。报告的年龄最大的患者为69岁，有头晕和接近晕厥的症状。这些患者中未报告死亡病例。双盗血型是最常见的类型，常与遗传综合征和/或心外异常相关。三盗血型是最罕见的类型，发病最早且预后最差。报告的年龄最大的患者为60天。所有报告病例均有心脏症状、肺循环过度、肺动脉高压和致命结局。
