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.     

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.     

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.     

Interrupted aortic arch: Natural history and operative results

Summary The chances of survival for patients with interruption of the aortic arch depend mainly on early recognition and medical treatment before symptoms of cardiogenic shock appear, and specifically on early operative repair after the exact diagnosis has been established. Accurate angiography with visualization of the interrupted aortic arch and delineation of associated cardiac malformations is of prime importance for the surgical management and prognosis. Among our 36 patients with interrupted aortic arch, 27 were operated on. The operative mortality in 22 patients with associated ventricular septal defect (VSD) and persistent ductus arteriosus (PDA) was reduced from 43% (in seven patients seen up until 1979) to 7% (in 15 patients seen since 1980). One patient with interrupted aortic arch type C and another infant with associated truncus arteriosus were successfully corrected on day 9 and day 17 of life, respectively. In the patients with associated VSD and PDA as well as in the one patient with associated truncus arteriosus, the primary correction—direct anastomosis of the interrupted segments without prosthesis and VSD closure and in the case with truncus, the additional positioning of a valve-bearing allograft conduit from the right ventricle to the pulmonary artery—has been more successful than a two-step approach with initial pulmonary artery banding.     

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.     

Vascular Rings: Presentation,Imaging Strategies,Treatment, and Outcome

This study aimed to evaluate the presenting symptoms, the effectiveness of imaging methods, and the surgical treatment of vascular rings. Data for 44 patients (32 enrolled prospectively, 12 reviewed retrospectively) over a 10-year period in a tertiary referral center were analyzed. These patients comprised 25 patients with a left aortic arch and an aberrant right subclavian artery, 13 patients with a right aortic arch and a left subclavian artery originating from Kommerell’s diverticulum, 1 patient with a right aortic arch and an aberrant left subclavian artery, 3 patients with a double aortic arch, and 2 patients with a pulmonary sling. Respiratory symptoms were found in 25 patients and dysphagia in 6 patients. Atypical symptoms such as reflex apnea, cyanosis, syncope episodes, and exercise-induced wheezing were noted in five patients. Associated congenital heart defects were detected in 41% of the patients. The diagnostic yield was 95.23% for barium esophagography, 54.54% for echocardiography, and 66.66% for computed tomography. The anatomy could be correctly identified by magnetic resonance imaging (MRI) in 97.43% and by angiography in 90.5% of the patients. Of the 30 patients who underwent surgery, 80% were completely relieved of symptoms during a mean follow-up period of 25 ± 33.5 months. Vascular rings should not be overlooked in infants with atypical symptoms. The authors’ diagnostic procedure of choice is MRI because it is superior to angiography for delineating the relationship between abnormal vascular structures, trachea, and esophagus.     

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.     

Follow-up of surgical correction of vascular anomalies causing tracheobronchial compression

Summary Between January 1977 and January 1990, 44 patients with symptomatic vascular rings/slings required surgical intervention at this center. Nineteen patients had double aortic arch (group I); 13 patients had vascular ring consisting of right aortic arch, anomalous origin of the left subclavian artery, and ligamentum arteriosus (group II); eight patients had innominate artery compression (group III); and four patients had pulmonary artery sling (group IV). Three patients had complex congenital heart defect and died secondary to it and are excluded from the study. Follow-up was obtained on 31 patients (76%). The follow-up period ranged from 0.4 years to 10.9 years, with a mean of 3.6 years. Seventy percent of the overall group were asymptomatic, and 30% of patients continued to have upper and lower respiratory symptoms on late follow-up. One third of patients in groups I and II, who underwent surgical repair, continue to have symptoms. Patients who have persistent symptoms should be further evaluated with PFTs, MRI, and bronchoscopy, and may benefit from aortopexy. Patients with innominate artery compression and pulmonary artery sling do well soon after surgery with relief of most of their symptoms.     

Magnetic resonance imaging in tracheal compression by the innominate artery

Tracheal compression by the innominate artery is frequently suspected in infants with noisy breathing and no obvious aortic arch anomalies or pulmonary artery sling. However the diagnosis is difficult to confirm. The findings after using magnetic resonance imaging in two infants with tracheal compression syndrome are presented.     

Magnetic resonance imaging in tracheal compression by the innominate artery.

Tracheal compression by the innominate artery is frequently suspected in infants with noisy breathing and no obvious aortic arch anomalies or pulmonary artery sling. However the diagnosis is difficult to confirm. The findings after using magnetic resonance imaging in two infants with tracheal compression syndrome are presented.     

Situs Inversus Totalis and Corrected Transposition of the Great Arteries {I,D,D} in Association with a Previously Unreported Vascular Ring

A 3-month-old girl with ``noisy breathing' was found to have situs inversus totalis, corrected transposition of the great arteries {I,D,D}, and a vascular ring. The ring was composed of a left aortic arch with normal branching pattern and a right ligamentum arteriosum that extended from a diverticulum off the descending aorta and coursed retroesophageal and to the right to join the pulmonary artery. There was no circumflex component of the aorta or aberrant subclavian artery. The descending aorta was left sided. Compression of the esophagus and trachea was noted on contrast esophagram, magnetic resonance imaging (MRI), and at the time of surgery to divide the vascular ring. In association with her corrected transposition, the patient also was shown to have a mild Ebstein's deformity of the right-sided (systemic) atrioventricular valve and electrocardiographic evidence of Wolfe–Parkinson–White syndrome. The combination of situs inversus totalis, corrected transposition of the great arteries {I,D,D}, and an aortic arch anomaly has not been previously reported. In addition, the aortic arch anomaly suggested by MRI imaging and confirmed at surgery has previously only been postulated to exist but to our knowledge never reported.     

Tetralogy of Fallot with Right Aortic Arch and Isolation of the Left Innominate Artery from a Left-Sided Patent Ductus Arteriosus

Isolation of the left innominate artery arising from the ductus arteriosus is a rare clinical entity with only nine cases being reported in the literature [5, 7, 8]. We describe a case consisting of tetralogy of Fallot, right aortic arch, and isolation of the left innominate artery arising from a left-sided ductus arteriosus which, to the best of our knowledge, has not been previously reported. The embryology of the aortic arch anomaly is reviewed.     

Pulmonary Artery Diverticulum: An Angiographic Marker for Williams Syndrome

We evaluated the presence of pulmonary artery diverticulum in patients with Williams syndrome in comparison with other conditions causing peripheral pulmonary artery stenosis (PPS). Angiographic characteristics of patients with a definitive diagnosis of Williams syndrome, by fluorescence in situ hybridization, between 1990 and 2008 were reviewed. These data were compared with those diagnosed with those for patients with PPS without Williams syndrome. Differentiating morphological features on angiography were compared between the groups, along with demographic and echocardiographic data. Twelve patients with a chromosomal diagnosis of Williams syndrome who underwent cardiac catheterization were identified. Seven were male. Eleven patients (91%) had supravalvar aortic stenosis and nine (81%) had PPS. Pulmonary valve stenosis was seen in two patients. Eight patients who were negative for Williams syndrome and had PPS were identified during the same period. Two had Alagille syndrome and one had Noonan syndrome. Mean age at catheterization was 5 years in the Williams group versus 8 years in the non-Williams group. Pulmonary artery diverticulum involving the main pulmonary artery was documented in all patients with Williams syndrome, while none of the patients in the other group had it. It originated at the bifurcation of the pulmonary artery in all. In conclusion, the angiographic appearance of a diverticulum as an extension of the main pulmonary artery is a consistent finding in patients with Williams syndrome. Compared to the classically described findings of supravalvar aortic stenosis or PPS, pulmonary artery diverticulum can be considered as a pathognomonic feature of Williams syndrome.     

Two-dimensional echocardiographic visualization of the aortic arch by right parasternal scanning in neonates and infants

Summary Echocardiography has previously been of limited use in visualizing the aorta in neonates and infants. Using a new technique—right parasternal scanning—the aortic arch and brachiocephalic vessels were studied in 50 children under 18 months of age. Complete visualization was accomplished in 47 patients including those 1) under 1,000 grams, 2) with indwelling endotracheal tubes, 3) who had D- or L-transposition, or 4) with right or double aortic arch. Inadequate studies were related to bilateral pneumothorax (2 neonates) and lack of cooperation (1 infant). Close correlation was obtained between images of the aorta by echocardiography and by angiography. Two-dimensional echocardiography can now visualize the aortic arch in small infants and children. In conjunction with another new technique—simultaneous Doppler flow-detection—coarctation of the aorta can be completely evaluated noninvasively in children of all ages including neonates. Presented, in part, at the World Congress of Pediatric Cardiology, June 2–6, 1980, London, England     

Right Aortic Arch with Retroesophageal Left Aberrant Innominate Artery

Right aortic arch with a left retroesophageal innominate artery (type D double aortic arch) is rare. The diagnosis is made by aortography. The present case is the first known patient to undergo a magnetic resonance imaging study that outlined the anomaly clearly.     

Isolated Innominate Artery in 22q11 Microdeletion

A patient with an isolated left innominate artery (with a right-sided cervical aortic arch) is described. This is the first report of such an anomaly associated with chromosome 22q11 microdeletion. The abnormality represents an interruption in the primitive aortic arch that is atypical for this chromosome deletion.     

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.     

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.