Right Aortic Arch with Isolation of the Left Subclavian Artery and Bilateral Patent Ductus Arterioses

Of the right aortic arch anomalies, a right arch with isolation of the left subclavian artery is the least common. In a neonate who had a right arch with isolation of the left subclavian artery and bilateral patent ductus arterioses, pulmonary artery steal phenomenon developed after ligation of the right ductus. We performed division of the left ductus and reimplantation of the left subclavian artery into the left common carotid artery.     

MDCT angiography of isolated right subclavian artery

Isolation of a subclavian artery is an uncommon congenital anomaly of the aortic arch in which one subclavian artery loses its connection with the aorta and originates from the homolateral pulmonary artery by way of a ductus arteriosus. Isolation of the left subclavian artery in patients with a right aortic arch is well known. However, isolated right subclavian artery with a left-sided aortic arch is an extremely rare condition. In this report, we present multidetector computed tomographic (MDCT) angiography findings of an isolated right subclavian artery associated with a common carotid trunk and an anomalous origin and proximal interruption of the left pulmonary artery.     

Congenital Pulmonary Steal Phenomenon Associated with Tetralogy of Fallot, Right Aortic Arch, and Isolation of the Left Subclavian Artery

Isolation of the left subclavian artery has been reported in cases of tetralogy of Fallot with a right aortic arch. As the isolated left subclavian artery is supplied by the left vertebral artery (in which the blood flow direction is inverted), this anomaly is usually responsible for a congenital subclavian steal phenomenon. In our case the isolated left subclavian artery was connected by a patent ductus arteriosus to the main pulmonary artery. Therefore the left vertebral artery did not supply the distal left subclavian artery but supplied the main pulmonary artery (congenital pulmonary artery steal).     

Right Aortic Arch with Aberrant Left Subclavian Artery and Aneurysm of Right Subclavian Artery. Report of a Case

A case of 12-year-old boy with the right aortic arch, aberrant left subclavian artery, left ligamentum arteriosus and congenital aneurysm of right subclavian artery which was excised, was reported.     

Magnetic Resonance Imaging of a Distorted Left Subclavian Artery Course: An Important Clue to an Unusual Type of Double Aortic Arch

The objective of this study was to determine if distortion of the left subclavian artery course distinguishes double aortic arch with atretic left dorsal aorta from right aortic arch with mirror image branching. We performed a retrospective case series at a tertiary care center. Twenty-six patients undergoing magnetic resonance imaging for suspicion of a vascular ring were identified, 6 of whom had an atretic left dorsal aorta confirmed by surgical inspection. Six patients with the diagnosis of right aortic arch with mirror image branching were identified for comparison. The course of the left subclavian artery was assessed using surface-rendered magnetic resonance angiography (MRA) and axial fast spin echo images. All patients with double aortic arch had clinical symptoms suggestive of esophageal or tracheal compression. Six patients had double aortic arch, 3 of whom had an atretic left dorsal aorta. In these 3 patients, the branching patterns on MRA mimicked right aortic arch mirror image branching except for the distortion of the initial course of the left subclavian artery. Surgical observation confirmed the presence of an atretic left dorsal aorta that resulted in tension on the left subclavian artery pulling it posteriorly and inferiorly and completing the vascular ring. Patients with right aortic arch mirror image branching demonstrated no such subclavian artery distortion, and these patients did not have clinical symptoms suggestive of a vascular ring. Our results demonstrate that left subclavian artery distortion due to traction by an atretic left arch is an important diagnostic finding in the evaluation 6 patients with suspected vascular rings.     

An isolated left subclavian artery supplied by a collateral artery from the abdominal aorta

An isolated left subclavian artery is a rare anomaly. We report a 9-month-old boy with an isolated left subclavian artery associated with tetralogy of Fallot and the right aortic arch. MRI and angiography show that the blood supply through the left subclavian artery was maintained by a large tortuous collateral artery from the abdominal aorta. This type of collateral artery structure is unique.     

Subclavian Steal Syndrome in Anomalous Connection of the Left Subclavian Artery to the Pulmonary Artery in D-Transposition of the Great Arteries

Several reports have documented the occurrence of an isolated left subclavian artery in association with both tetralogy of Fallot and double-outlet right ventricle. In certain cases a congenital subclavian or pulmonary artery steal syndrome exists in which the left subclavian artery is connected to the main pulmonary artery via a ductus arteriosus. We describe a subclavian steal syndrome secondary to anomalous origin of the left subclavian artery from the pulmonary artery in d-transposition of the great arteries in a patient with Spondylocostal dysostosis (SCD). Cardiac anomalies are rare in SCD and this constellation of findings have not previously been described.     

Rare types of aortic arch anomalies

Summary Rare types of aortic arch malformations include isolation of the right or left subclavian arteries in conjunction with a left or right aortic arch respectively. Isolation of the innominate or carotid arteries and an aberrant left innominate artery may exist in association with a right aortic arch. Other anomalies are persistent fifth aortic arch, subclavian artery as the first branch of the aortic arch, pulmonary artery, and ductus arteriosus sling. The angiocardiographic features and embryology of each of these malformations are discussed.     

Faulty origin of the right subclavian artery from the pulmonary artery: a rare cause of subclavian steal syndrome in childhood

A congenital subclavian steal syndrome may be caused by coarctation or interruption of the aortic isthmus or by isolation of a subclavian artery. We describe a patient with D-transposition of the great arteries, a left aortic arch, and isolation of the right subclavian artery which originated from the right pulmonary artery via a right ductus arteriosus. A subclavian steal syndrome was demonstrated noninvasively by echocardiography and doppler sonography of the cerebral arteries. We recommend routine cerebral doppler sonography for all infants with congenital heart disease and unilaterally weak brachial pulses. Since the long term outcome of the congenital subclavian steal syndrome is uncertain the aberrant subclavian artery should be reimplanted at the time of corrective cardiac surgery.     

Dysphagia Lusoria Caused by Aberrant Right Subclavian Artery, Kommerell’s Diverticulum, Legamentum Ring, Right Descending Aorta, and Absent Left Pulmonary Artery: A Report of a Unique Vascular Congenital Disease Undetected until Adulthood and a Review of the Literature

An active otherwise healthy and middle-aged woman presented with left supraclavicular pulsation, right upper extremity claudication, and mild dysphagia. Evaluation revealed an aberrant right subclavian artery, Kommerell’s diverticulum with aneurysmal degeneration, legamentum arteriosum completing vascular ring, and absent left pulmonary artery with multiple collateral supply to the left lung. She underwent successful surgical repair via right thoracotomy, including division of the vascular ring, resection of the diverticulum and aneurysm, and finally reimplantation of the right subclavian artery to the aortic arch. Her symptoms resolved completely, and she was able to resume normal activities.     

MRI diagnosis of isolated origin of the left subclavian artery from the left pulmonary artery

Right aortic arch with isolation of the left subclavian artery is a rare disorder. In this entity, the left subclavian artery has its origin from the pulmonary artery via the ductus arteriosus. We report an infant with an unbalanced atrioventricular septal defect, right aortic arch, bilateral ductus arteriosi and an isolated left subclavian artery. This infant also had chromosome 22q11 deletion. The origin and course of the isolated subclavian artery were well-demonstrated using contrast-enhanced 3-D magnetic resonance angiography. This non-invasive diagnostic modality can accurately reveal detailed mediastinal vascular anatomy in a young infant and allows diagnosis of aberrant vascular connections.     

Isolation of the Left Subclavian Artery-Origin from the Left Pulmonary Artery by Way of Ductus Arteriosus: Multidetector Row Computed Tomographic Angiographic Imaging

Right aortic arch with isolation of the left subclavian artery is a rare anomaly. It has been reported to occur with conotruncal anomalies and may be associated with 22q11 deletion. Multidetector-row computed tomographic angiogram images of a 15-year-old African boy with Tetralogy of Fallot who had right aortic arch and isolated left subclavian artery arising from the left pulmonary artery by way of ductus arteriosus are presented.     

Aortic arch interruption presenting with absence of all limb pulses.

Three patients with aortic arch anomalies presented with severe heart failure, acidaemia, and poor or absent upper and lower limb pulses. Prominent carotid arterial pulsations were detected which distinguished the condition clinically from aortic atresia. In 2 patients with a left aortic arch, interruption was distal to the left common carotid artery and there was an aberrant right subclavian. In the 3rd patient with a right aortic arch, interruption was distal to the right common carotid and there was an aberrant left subclavian artery. By dilating the narrowed ductus, prostaglandin E2 infusion corrected the acidosis. Successful surgical correction was achieved in one patient by direct anastomosis of descending aorta to ascending aorta and the base of the left common carotid artery.     

Right Pulmonary Agenesis Converts an Incomplete Vascular Ring Into a Near-Complete Vascular Ring

A unique combination of pulmonary agenesis and anatomic left aortic arch with aberrant right subclavian artery was identified in two patients. Because of the right pulmonary agenesis, there is rotation of mediastinal contents, especially the aortic arch, into the right chest, converting an incomplete vascular ring to a near-complete vascular ring. The anterior portion of the ring is formed by the ascending aorta and aortic arch, whereas the posterior portion is formed by the aberrant right subclavian artery. Subclavian artery reimplantation and aortopexy effectively relieve the anterior and posterior tracheoesophageal compressive forces.     

MR imaging of isolated right subclavian artery

We report the MRI findings in a 3-week-old boy with D-transposition of the great arteries and an abnormal origin of the right subclavian artery from the pulmonary artery. This anomaly of the subclavian arteries is called isolation. It is infrequent in patients with a right aortic arch, but exceedingly rare in those with a left aortic arch. This is a unique report of the MRI findings in this congenital abnormality of the aortic arch.     

Right circumflex retro-oesophageal aortic arch with coarctation of a high-positioned right arch

We present a rare case of right circumflex retro-oesophageal aortic arch with coarctation of a high-positioned right arch. A 7-month-old boy presented with a cardiac murmur. Cardiac situs was normal and there was no evidence of an intracardiac shunt or patent ductus arteriosus. MR aortography revealed a right aortic arch that was high-positioned, tortuous and narrowed. This right aortic arch crossed the midline behind the oesophagus and continued as a left-sided descending aorta. The left common carotid and subclavian arteries arose from a large branching vascular structure that derived from the top of the left-sided descending aorta. The right common carotid artery arose from the ascending aorta. The proximal portion of the right common carotid artery showed very severe stenosis and poststenotic dilatation. The right subclavian artery originated distal to the narrowed and tortuous segment of the aortic arch.     

Reconstruction of an Isolated Left Subclavian Artery in a Patient with a Right Aortic Arch and Tetralogy of Fallot

An isolated left subclavian artery (ILSA) is very rarely seen in patients with a right aortic arch. This report describes the case of a 2-year-old boy who underwent ILSA reconstruction during repair of the associated tetralogy of Fallot to prevent future subclavian steal syndrome after surgery.     

THE OCCURRENCE OF TWO VASCULAR RINGS IN THE SAME INFANT

A fatal case of an infant with a combination of two vascular rings is described. One ring consisted of a right-sided aortic arch, aberrant left subclavian artery and persistent ductus arteriosus, the other of an anomalous left pulmonary artery branched off from the right pulmonary artery. The importance of a properly performed roentgen examination of the oesophagus is stressed.     

Takayasu arteritis as a cause of arterial hypertension. Case report and literature review

We report a 16-year-old girl in whom Takayasu arteritis (TA) was manifested mainly by severe arterial hypertension on her right arm, which was detected during a routine examination at school. Her systolic blood pressure on the right arm was significantly higher than that on the left one. There was also a pressure difference between the right arm and legs. The pulse of the left external carotid artery and that of the left radial artery was absent. Vascular bruits over interscapular and right supra- and subclavian areas were heard on auscultation. The diagnosis of TA was confirmed by a spiral computed tomography angiography, which showed a thickened thoracic aortic wall and narrowing of its lumen. In addition, complete occlusion of the left common carotid artery and the left subclavian artery was observed. Conclusion: The rarity of the disorder and the heterogeneous nature of its clinical manifestation predispose to a late diagnosis and delayed treatment. Our report highlights the fact that the condition can and does occur in a pediatric population in Europe and hence must be considered in patients presenting with suggestive symptoms and signs, especially in young patients with unexplained hypertension. Clinical suspicion and proper imaging are crucial for the correct diagnosis and management of patients with TA. A brief review of literature completes this report.