Type B Interrupted Left Aortic Arch with Isolated Right Subclavian Artery

Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three per 1 million births. Type B interrupted aortic arch (interruption between the second carotid artery and the ipsilateral subclavian artery) is the most common of three major types (A, B, and C). We report an extremely rare finding: a case of left‐sided type B interrupted aortic arch with isolation of the right subclavian artery (origin from the right pulmonary artery).     

Hypoplastic right retro-oesophageal aortic arch: similarities to interrupted aortic arch.

At echocardiography a dysmorphic neonate was found to have a hypoplastic mitral valve, left ventricle, and ascending aorta with interruption of the aortic arch between the left common carotid and the left subclavian arteries--the left subclavian artery arose from a normal sized left descending aorta. Cardiac catheterisation and subsequent necropsy confirmed this arrangement but also showed a tortuous right retro-oesophageal aortic arch. This arch was severely hypoplastic distal to the right subclavian artery. These cardiovascular anomalies occurred in the child of a family in which other members showed the velo-cardio-facial syndrome, a condition known to be associated with right aortic arch. This aortic arch arrangement may be impossible to distinguish from simple aortic interruption by echocardiography alone.     

Isolation of the left common carotid or left innominate artery

Isolation of the left common carotid or left innominate artery from the aortic arch is rare. A six week malformed infant with a right aortic arch had isolation of a left innominate artery and connection to the pulmonary artery by a left ductus arteriosus. A right ductus arteriosus had been ligated. Another infant with a right aortic arch and ostium primum atrial septal defect was shown to have an aberrant left subclavian artery arising from the lower descending aorta. The left common carotid artery filled retrogradely and drained into the pulmonary artery by a left ductus arteriosus. This abnormality has not been reported before.     

Isolation of the left common carotid or left innominate artery.

Isolation of the left common carotid or left innominate artery from the aortic arch is rare. A six week malformed infant with a right aortic arch had isolation of a left innominate artery and connection to the pulmonary artery by a left ductus arteriosus. A right ductus arteriosus had been ligated. Another infant with a right aortic arch and ostium primum atrial septal defect was shown to have an aberrant left subclavian artery arising from the lower descending aorta. The left common carotid artery filled retrogradely and drained into the pulmonary artery by a left ductus arteriosus. This abnormality has not been reported before.     

Hypoplastic right retro-oesophageal aortic arch: similarities to interrupted aortic arch

At echocardiography a dysmorphic neonate was found to have a hypoplastic mitral valve, left ventricle, and ascending aorta with interruption of the aortic arch between the left common carotid and the left subclavian arteries--the left subclavian artery arose from a normal sized left descending aorta. Cardiac catheterisation and subsequent necropsy confirmed this arrangement but also showed a tortuous right retro-oesophageal aortic arch. This arch was severely hypoplastic distal to the right subclavian artery. These cardiovascular anomalies occurred in the child of a family in which other members showed the velo-cardio-facial syndrome, a condition known to be associated with right aortic arch. This aortic arch arrangement may be impossible to distinguish from simple aortic interruption by echocardiography alone.     

Arteria lusoria: Developmental anatomy, clinical, radiological and surgical aspects

The left aortic arch with an aberrant right subclavian artery, or arteria lusoria, is the most common aortic arch anomaly, occuring in 0.5-2.5% of individuals. Four vessels arise sequentially from the aortic arch: the right common carotid artery, the left common carotid artery, the left subclavian artery and the aberrant right subclavian artery, which crosses upwards and to the right in the posterior mediastinum. It results from a disruption in the complex remodelling of the paired branchial arches, typically of the right dorsal aorta distal to the sixth cervical intersegmental artery. The diagnosis and differentiation of arch anomalies is based on findings at chest radiography in association with those at esophagography. It is usually asymptomatic. When symptomatic, it produces dysphagia lusoria or dyspnea and chronic coughing. Treatment is indicated for symptomatic relief of dysphagia lusoria and for prevention of complications due to aneurysmal dilatation.     

Aortic anomaly with atypical coarctation A report of three cases presenting coarctation between the origin of the left carotid and the left subclavian artery

Three cases of faulty development of the aorta are presented. In Case 1 the aortic arch between the origin of the left common carotid and the left subclavian artery was tortuous, narrow and situated high in the thorax. Two aneurysms were located in this portion. The left vertebral and left internal mammary arteries were not demonstrated. The narrowed part of the aorta was resected under hypothermia with a good result.

Case 2 showed a pronounced coarctation between the left common carotid artery and the left subclavian artery. This latter vessel was filled almost completely via an ample collateral network. The left common carotid artery had an anomalous origin and the right subclavian artery showed a slight coarctation in the proximal part; a poststenotic dilatation was distal to this.

Case 3 showed an aortic arch situated high in the thorax. The bending of the aortic arch was very sharp, producing a functional coarctation with differences in the blood pressures in the right and left arm; there was no anatomic coarctation in the ordinary sense.

Two cases were corrected by surgery; the former by resection of the tortuous part and end to end anastomosis, the latter by incision and removal of the membrane.

The different theories concerning the development of coarctation of the aorta are discussed. It is suggested that the cases presented might best be explained by faulty development.     

Right aortic arch with ruptured aneurysm of anomalous left subclavian artery

An aneurysm in the anomalous left subclavian artery developed in an elderly woman with a right aortic arch and an aberrant left subclavian artery (left ligamentum arteriosum connected to the left common carotid artery). The aneurysm gradually enlarged, eventually rupturing with exsanguination. A progressive increase in the size of this aneurysm was associated with symptoms and anatomic findings consistent with the subclavian “steal” syndrome.     

Cervical origin of the right subclavian artery in aortic arch interruption: pathogenesis and significance

In 14 of 21 infant hearts (66%) with aortic arch interruption between the left common carotid and left subclavian arteries (type B of Celoria and Patton), the right subclavian artery (SA) arose anomalously. In 9 (43%) it arose from the descending aorta and in 1 heart from the right pulmonary artery (5%). In 4 hearts (19%), it arose high in the neck from the right common carotid artery as a trifurcation with the internal and external carotid arteries, an anomaly not previously reported. In these latter cases, the SA descended down the neck to enter the right arm, with the right recurrent laryngeal nerve coursing around its take-off. Anomalous origin of the right SA is common in type B aortic arch interruption, and we believe this to be the result of a strong tendency for both fourth aortic arches to disappear early in development. If involution occurs in embryos of less than 14 mm crown-rump length, before the right ductus caroticus and dorsal segment of the right sixth arch have become attenuated, 3 alternate routes are available to the embryo to form a right SA.     

Transesophageal Echocardiographic Diagnosis of Right-Sided Aortic Arch

We present the transesophageal echocardiographic findings in two adult patients with right-sided aortic arch: one without dissection and the other with traumatic aortic injury (dissection). In both patients, the branching pattern was the left common carotid artery and then the right common carotid artery, followed by the right and left subclavian arteries. The technique for the diagnosis of this anomaly and the identification of adjacent vascular structures using contrast echocardiography is described. Three-dimensional reconstruction of the aortic arch also was performed in both patients.     

Left common carotid artery arising from the pulmonary artery in a patient with DiGeorge syndrome.

A female infant, born at 33 weeks' gestation with tetralogy of Fallot, died of severe perinatal asphyxia 6 hours after birth. Necropsy disclosed two associated vascular anomalies: a right aortic arch with a left common carotid artery arising from the pulmonary artery (isolated left common carotid artery) and an aberrant left subclavian artery arising from the descending aorta. Agenesis of the thymus and parathyroid gland was also found, suggesting that the child also had DiGeorge syndrome. Origin of the left common carotid artery from the pulmonary artery is exceedingly rare. When planning surgical treatment it is important to be aware of the possibility of this anomaly occurring in association with congenital heart disease, particularly in the presence of tetralogy of Fallot, right sided aortic arch, or DiGeorge syndrome.     

Surgical management in paediatric patients with left abnormal subclavian artery and right aortic arch

Left abnormal subclavian artery and right aortic arch is described as a rare cause of dyspnoea and dysphagia in paediatric patients. The optimal surgical management of such cases is not clearly established.We propose a single-stage repair by transection of the patent ductus arteriosus or ligamentum arteriosum and an additional transection of the left abnormal subclavian artery with reimplantation into the common carotid artery.     

Atresia of the aortic arch, with a collateral artery from the right subclavian artery supplying the descending aorta

An infant of 2 months presented with absence of the femoral pulses, albeit with no signs of cardiac failure. The mother was known to have ingested Valproate during pregnancy. Echocardiography showed the aortic arch to be interrupted between the left common carotid and left subclavian arteries, so-called type B interruption, in the setting of an intact ventricular septum. Angiography, and multislice computed tomography, revealed the descending aorta to be supplied by a collateral artery originating from the right subclavian artery. Corrective surgery was successfully performed, but revealed an atretic segment of the arch at the site of interruption of flow.     

Obstructed coarctation in a right aortic arch in an adult female

Coarctation of a right aortic arch is rare congenital anomaly. We report a rare case of a 24-year-old female with coarctation of the right aortic arch with aberrant left subclavian artery between the right common carotid and right subclavian arteries. The coarctation progressed into complete obstruction as the interruption of the aorta in adulthood. To prevent cerebral complications and progression to heart failure, surgical procedure was selected. Extraanatomical bypass grafting between the ascending and descending aorta was successfully performed using cardiopulmonary bypass. Some patients diagnosed with interruption of the aortic arch in adulthood might be displaying progression of undiagnosed coarctation, as our in case. Three-dimensional computed tomography was useful to detect the obstructive lesion and to determine the surgical approach and methods.     

Isolation of the Left Subclavian Artery in D-Transposition of the Great Arteries with Right Aortic Arch

Abnormal branching of the aorta associated with the right aortic arch (RAA) has been reported as isolation of left subclavian artery (ILSA), isolation of left common carotid artery, isolation of brachiocephalic artery. ILSA is a rare aortic branch anomaly that originates in the left subclavian artery from the pulmonary artery via ductus arteriosus. Several reports have described ILSA associated with 22q11.2 deletion syndrome and tetralogy of Fallot. Here, we present a very unusual case of RAA with ILSA associated with D-transposition of the great arteries and inferior vena cava interrupted with azygos continuation.     

Unusual vertebral artery origins: examples and related pathology.

Anomalies of the vertebral arteries are uncommon, but important to recognize in the diagnosis and catheter based evaluation and treatment of patients suffering cerebrovascular disease. This article illustrates our experience with such anomalies. These include the vertebral artery arising as the fourth and most distal branch of the aortic arch, as a right subclavian artery branch arising distal to the right thyrocervical trunk, as a right common carotid artery branch in a patient with an aberrant right subclavian artery, and a case of left vertebral artery proximal duplication, with both aortic and left subclavian vertebral arteries present in the same patient; the latter join to form a single distal cervical vertebral artery.     

Aberrant left subclavian artery in tetralogy of Fallot

The anatomy of the aberrant left subclavian artery in a right-sided aortic arch is different in patients with tetralogy of Fallot than in persons with a normal heart. In all eight patients with tetralogy of Fallot and aberrant left subclavian artery in this series, the left subclavian artery arose directly from the distal aortic arch. In contrast, normal persons with right aortic arch and aberrant left subclavian artery invariably have an aortic diverticulum from which the left subclavian artery and the left ductus arteriosus originate. The possible different embryologic events responsible for these two forms of aberrant left subclavian artery are discussed.     

头臂血管成形术治疗成人主动脉弓及头臂血管发育不良

目的:总结并探索主动脉弓及头臂血管发育不良的手术治疗方式。方法:2009年2月至2011年12月,北京安贞医院大血管中心对4例主动脉弓及头臂血管发育不良的患者行主动脉弓成形术治疗,手术均采用右侧腋动脉和股动脉及右心房插管建立体外循环,在深低温停循环选择性脑灌注下进行,沿主动脉弓长轴靠近头臂血管根部横行切开主动脉弓,切口两端达正常段,再纵形剖开3枝头臂血管开口及近段(狭窄段)前壁,分别将无名动脉和左颈总动脉、左颈总动脉和左锁骨下动脉相邻血管壁连续缝合在一起,将3枝血管融合成一个共同腔的后壁,再用人工血管片作为前壁补片加宽主动脉弓及头臂血管共同腔,以完成对发育不良的主动脉弓和头臂血管的手术矫治。结果:患者术后均恢复顺利,复查结果显示主动脉弓及头臂血管形态良好,上下肢压差<20 mmHg(1 mmHg=0.133 kPa)。结论:将发育不良的头臂血管近段剖开融合成一个共同腔,再用人工血管片加宽可理想地矫正主动脉弓及头臂血管发育不良。     

Symptomatic aberrant retro-esophageal subclavian artery: considerations about the surgical approach, management and results

Ten case histories of patients with symptomatic aberrant retro-esophageal subclavian artery are presented. The diagnosis was made on a clinical basis in 8 cases because of symptoms of tracheo-esophageal obstruction, and confirmed by barium meal. All patients were investigated by angiography. Six patients with right abnormal retro-esophageal subclavian artery (arteria lusoria) and its mirror image were treated by transection of the aberrant subclavian artery and reimplantation into the common carotid artery through a single midline sternal splitting incision which gave ample access. Three patients with left abnormal subclavian artery (right aortic arch) were operated by transection of the patent ductus arteriosus or ligamentum arteriosum through a left lateral thoracotomy. After a follow-up of from one to 10 years (mean 6.4 years), 9 patients are free of symptoms like dysphagia, dyspnea or subclavian steal and normal pulses are present on both upper extremities in 8 cases.     

Interruption of the aortic arch associated with deletion of chromosome 22q11 is associated with a subarterial and doubly committed ventricular septal defect in Japanese patients.

OBJECTIVES: The purpose of this study was to clarify the clinical characteristics of interruption of the aortic arch associated with chromosome 22q11deletion. BACKGROUND: About half of patients with interruption of the aortic arch between the left common carotid and the left subclavian artery have deletion of chromosome 22q11. METHODS: In total, 20 patients with interruption of the aortic arch were studied with fluorescence in situ hybridization using peripheral lymphocytes and a DiGeorge syndrome chromosomal probe (Oncor N25). Cardiovascular anomalies in these patients were diagnosed by cross-sectional echocardiography and angiocardiography, and were confirmed at intracardiac repair. RESULTS: Of 13 patients with interruption between the left common carotid artery and the left subclavian artery, seven had the deletion. All 7 also showed thymic hypoplasia and hypocalcemia, together with a nasal voice and peculiar facies. Six of the seven patients had complete deficiency of the muscular outlet septum, with the defect extending to the perimembranous area. Such complete absence of the muscular outlet septum was not present in any of the patients without the deletion. CONCLUSIONS: Interruption of the aortic arch between the left common carotid and the left subclavian artery, absence of the thymus, and complete absence of the muscular outlet septum, were characteristic in Japanese patients with interruption of the aortic arch associated with deletion of chromosome 22q11.