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.     

Persistent Left Fifth Aortic Arch Associated with Tetralogy of Fallot

A case of persistent left fifth aortic arch, forming a congenital ``double-lumen' aortic arch, has been diagnosed on angiocardiography during life. It appeared as an unusual vascular structure running inferiorly and parallel to the ``real' aortic arch from the innominate artery to the left subclavian artery superior to the pulmonary artery. This anomaly was found in the setting of tetralogy of Fallot, an association never described before, with patent ductus arteriosus (previously reported in most cases). The left aortic arch in this case was not a source of pulmonary circulation, as described in previous cases with pulmonary atresia and ventricular septal defect, but was a systemic-to-systemic connection without functional relevance.     

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 Anomalies Associated with Chromosome 22q11 Deletion (CATCH 22)

Chromosome 22q11 deletion or CATCH 22 is associated with DiGeorge syndrome, conotruncal anomaly face syndrome, and velocardiofacial syndrome. Associated congenital heart diseases include tetralogy of Fallot, truncus arteriosus, and ventricular septal defect. Associated anomalies of the aortic arch, aortic branches, ductus arteriosus, and pulmonary arteries are more frequent in patients with the deletion than in those without the deletion. Associated anomalies include right aortic arch, cervical aorta, aberrant origin or isolation of the subclavian artery, the absence of the ductus arteriosus, major aortopulmonary collateral arteries, isolation of the left pulmonary artery, and vascular ring formed by the right aortic arch, retroesophageal aortic arch, and left descending aorta.     

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.     

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.     

Anomalous Origin of One Pulmonary Artery from the Innominate Artery: A Report of Two Cases

Two children with an anomalous origin of one pulmonary artery from the innominate artery are reported. One was a 15-month-old boy presenting with respiratory distress. He had a right aortic arch and his left pulmonary artery originated from the innominate artery. The other was a 1-month-old girl presenting with congestive heart failure. She had a left aortic arch and her right pulmonary artery originated from the innominate artery. An understanding of the embryological pathogenesis of these anomalies has significant therapeutic implications.     

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).     

An Isolated Left Common Carotid Artery from the Main Pulmonary Artery: Possible Malseptation of the Truncoaortic Sac

An isolated left common carotid artery (LCA) is an extremely rare condition with only four reported cases. In each case, the isolated carotid artery connects to the right or left pulmonary artery via the ductus arteriosus and the embryologic basis for the abnormalities is believed to reflect an error in the development of the branchial arches. We present a case of an isolated LCA connecting to the main pulmonary artery in association with a right aortic arch and an anomalous origin of the left subclavian artery from the descending aorta. The left ligamentus arteriosus was identified separately. This may represent a disturbance in the septation of the truncoaortic sac secondary to abnormal migration of neural crest cells rather than a pure developmental anomaly of the branchial arches.     

Stenting of Ductus Arteriosus in a Neonate with Truncus Arteriosus and Interrupted Aortic Arch Associated with a Right Aortic Arch

This report describes a case in which successful stenting of ductus arteriosus (DA) was performed for a 27-day-old boy with truncus arteriosus (TA) and interrupted aortic arch (IAA). The patent DA was associated with a right aortic arch. During the balloon catheter crossing of the ductus, the DA and descending aorta shifted toward the left side, making appropriate stent placement difficult. Additionally, the DA was longer than in previously reported cases with left aortic arch, thus requiring a longer stent. This experience suggests that DA stenting in neonates with TA and IAA averts surgical repair during the early neonatal period.     

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.     

Patterns of Right Aortic Arch and Mirror-Image Branching of the Brachiocephalic Vessels Without Associated Anomalies

It is unusual to have a right aortic arch with mirror-image branching of the brachiocephalic vessels and no associated congenital cardiac anomalies. Pathogenetic factors of this anomaly, and associated development of the derivatives of the pharyngeal arches, have not been explored extensively. Eleven patients with a mirror-image right aortic arch and no intracardiac anomalies have undergone evaluation at our institution since 1987. Of these, 4 had stenosis (n= 2) or atresia (n= 2) of the proximal left pulmonary artery. These included 2 with bilateral ductus arteriosus. Six patients had a vascular ring formed by a ductus arteriosus or ligamentum arteriosum from the descending aorta to the left pulmonary artery. Six patients were evaluated for deletion within the DiGeorge critical region of chromosome 22q11. All 6 of the patients with a vascular ring underwent division of the ductus arteriosus or ligamentum through a left lateral thoracotomy. None of the patients with stenosis or atresia of the LPA have undergone intervention. Two of the 6 patients who underwent evaluation for chromosome 22q11 deletion were found to have the deletion. At a median follow-up of 7 years (15 months to 12 years), the patients who underwent division of the ring are all alive and asymptomatic. Two of these patients have undergone repeat magnetic resonance imaging, 1 of whom had mild residual tracheal compression. A right aortic arch with mirror-image branching of the brachiocephalic vessels and no associated intracardiac anomalies is extremely uncommon. We have observed two basic patterns of this lesion: (1) with associated abnormalities of the proximal left pulmonary artery, (2) with a vascular ring formed by a ductus arteriosus or ligamentum from the descending aorta to the left pulmonary artery. Pathogenetic considerations are explored, with a focus on flow-related vascular development and the role of deletions within chromosome 22q11.     

Single arterial trunk arising from the aortic arch associated with coarctation of the aorta

A 6-day-old baby with a single arterial trunk arising from the aortic arch is reported. The baby had coarctation of aorta with patent ductus arteriosus and ostium primum atrial septal defect. A review of the literature in English reveals no other case reported of an infant with this association of anomlies.     

Single arterial trunk arising from the aortic arch

A seven month old female having a single arterial trunk arising from the aortic arch is presented. The baby also had severe coarctation of the aorta and a patent ductus arteriosus. This anomaly cannot be explained by Edwards' embryonic double aortic arch model. However, it can be explained by persistence of the fifth branchial arch as the definitive aortic arch.     

Right aortic arch with isolation of the left subclavian artery

A 5-year-old boy with cyanotic heart disease and weak pulses in the left arm is described. Cardiac catheterization and cineangiography confirmed the diagnosis of tetralogy of Fallot and right aortic arch with isolation of the left subclavian artery. In addition to the aortogram and right ventricular cineangiography, pulmonary angiography was performed, demonstrating that blood did not reach the left subclavian artery through a left ductus arteriosus.     

Coarctation of the Aorta in the Right Aortic Arch with Left Aberrant Innominate Artery

Right aortic arch with an aberrant left innominate artery is a rare combination. We report a unique case of coarctation of the aorta in a patient with right aortic arch and aberrant left innominate artery. The diverticulum of Kommerell gave rise to the left innominate artery and a left ligamentum arteriosum, completing a vascular ring. This unusual case raises interesting points regarding the development of coarctation in the context of a right-sided arch and the approach to management of these patients.     

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.     

Aortic Arch Complex Anomalies: 20-Year Experience with Symptoms,Diagnosis, Associated Cardiac Defects,and Surgical Repair

To better understand the clinical presentation and surgical management of children with aortic arch complex anomalies (``vascular rings'), a retrospective study was performed on patients who presented to Children's National Medical Center between the years 1969 and 1989. A total of 59 patients were identified ranging in age at admission from birth to 14 years, of whom 35 (59%) were male and 24 (41%) female. Patients were classified into four major subtypes based on their surgical anatomy, with 29 (49%) patients having right aortic arch and left ductus/ligamentum arteriosus, 21 (36%) double aortic arch, 6 (10%) anomalous left pulmonary artery, and 3 (5%) arch artery anomaly (no ring); 19 patients (32%) had associated cardiac defects. The mean (±SD) age at onset of symptoms was 4.6 ± 14.0 months, and the age at surgical repair was 18 ± 34 months. There were no intraoperative mortalities, but two late deaths occurred. Three (5%) patients had a surgical complication. In contrast to previous studies, the incidence of nonring lesions was lower and associated cardiac defects higher. Forty-nine percent of patients had symptoms present at birth, and patients with associated cardiac disease did not present earlier than those without. In patients with right aortic arch and left ductus/ligamentum arteriosus, few had an anomalous left subclavian artery. Finally, equal dominance of the arches was most frequent in patients with double aortic arch. Aortic arch complex anomalies present symptomatically in a variety of ways, and noninvasive methods are used to identify the specific lesion and associated cardiac defects. Surgical repair is associated with low or no mortality in patients with uncomplicated aortic arch complex anomalies.     

Absent pulmonary artery

Summary Cardiac catheterization was performed in nine patients with unilateral absence of a pulmonary artery. Aortography revealed a diverticulum of the innominate artery in five of six patients in whom the aortic arch and absent pulmonary artery were on opposite sides. It is suggested that the diverticulum indicates fetal systemic blood supply to the affected lung through the distal part of the sixth aortic arch, which—consisting of ductal tissue—obliterated after birth, leaving the diverticulum where the ductus joins the innominate artery.