Endovascular repair of a right-sided descending thoracic aortic aneurysm with a right-sided aortic arch and aberrant left subclavian artery

Aneurysms involving a right-sided aortic arch and a right-sided descending thoracic aorta with an aberrant origin of the left subclavian artery are rare. We describe the successful surgical repair of this vascular anomaly by the combined use of a left carotid to subclavian artery bypass followed by endovascular stent-graft placement to exclude the aortic aneurysm. We also review the literature associated with this particular anatomic presentation.     

RIGHT AORTIC ARCH WITH ABERRANT LEFT SUBCLAVIAN ARTERY: ANEURYSMAL DILATATION CAUSING SYMPTOMATIC COMPRESSION OF THE RIGHT MAIN BRONCHUS IN AN ADULT

A 60 year old woman presented with a cough, nocturnal stridor and dysphagia. Bronchoscopy showed tight compression of the right main bronchus. Digital subtraction angiography (DSA) and a computed tomographic (CT) scan showed the presence of a right-sided aortic arch with aberrant left subclavian artery. The distal right arch and proximal right-sided descending thoracic aorta were aneurysmal and were responsible for this compression. Surgical relief was accomplished by dividing the aberrant left subclavian artery and replacing the aneurysm with a vascular graft.     

Vertical vascular ring around right mainstem bronchus.

An 18-year-old male patient was referred with an atypical form of vascular ring consisting of a right aortic arch, a right descending aorta, a persistent right ligamentum arteriosum, and a dilated right pulmonary artery in the setting of discontinuous pulmonary arteries with a single functional right lung. The initial presentation was that of pneumonia of the right lung and later evaluation revealed the right mainstem bronchus was squeezed between the descending aorta and the dilated right pulmonary artery. Surgical exploration disclosed the right-sided ligamentum arteriosum was running under the right mainstem bronchus and hereby was completing a vertical vascular ring. Decompression was achieved through surgical and bronchoscopic treatment.     

One-stage repair with separated cardiopulmonary bypass for coarctation of the aorta with left aortic arch and right thoracic descending aorta

We treated a 7-day-old neonate with aortic coarctation, left aortic arch, right-side descending aorta, aberrant right subclavian artery, and outlet ventricular septal defect. Surgical one-stage repair consisting of coarctation repair by extended direct anastomosis, patch closure of the ventricular septal defect, and translocation of the right subclavian artery was performed under a separated (upper and lower body) perfusion with ascending and thoracic descending aortic cannulation. The postoperative course was complicated with massive chylous effusion, which was successfully treated by thoracic duct ligation.     

Kommerell's diverticulum and right-sided aortic arch: a cohort study and review of the literature

We report four consecutive cases of Kommerell's aneurysm of an aberrant left subclavian artery in patients with a right-sided aortic arch and the results of a systematic review of the literature. In our cohort of patients, three had an aneurysm limited to the origin of the aberrant subclavian artery, causing dysphagia and cough, and one had an aneurysm involving also the distal arch and the entire descending thoracic aorta, causing compression of the right main-stem bronchus. A left subclavian-to-carotid transposition was performed in association with the intrathoracic procedure, and a right thoracotomy was used in all patients. One of the patients underwent surgery with deep hypothermia and circulatory arrest, and the others with the adjunct of a left-heart bypass. The repair was accomplished with an interposition graft in two patients and with endoaneurysmorrhaphy in the others. The postoperative course was complicated by respiratory failure and prolonged ventilation in one patient, and one patient died because of severe pulmonary emboli. The survivors are alive and well at a follow-up of 1 to 3 years. Only 32 cases of right-sided aortic arch with an aneurysm of the aberrant subclavian artery have been reported: 12 were associated with aortic dissection, and 2 presented with rupture. Surgical repair was accomplished in 29 patients. A number of operative strategies were described: right thoracotomy, bilateral thoracotomy, left thoracotomy with sternotomy, sternotomy with right thoracotomy, and left thoracotomy. In only 12 cases was the subclavian artery reconstructed. We believe that a right thoracotomy provides good exposure and avoids the morbidity associated with bilateral thoracotomy or sternotomy and thoracotomy. We feel that a left subclavian-to-carotid transposition completed before the thoracic approach revascularizes the subclavian distribution without increasing the complexity of the intrathoracic procedure.     

Surgical repair for aortic dissection accompanying a right-sided aortic arch

Aortic anomaly in which a right-sided aortic arch associated with Kommerell's diverticulum and aberrant left subclavian artery is rare. The present report describes a patient with type-B aortic dissection accompanying aortic anomalies consisting of right-sided aortic arch and the left common carotid and left subclavian artery arising from Kommerell's diverticulum. As dissecting aortic aneurysm diameter increased rapidly, Single-stage surgical repair of extensive thoracic aorta was performed through median sternotomy and right posterolateral fifth intercostal thoracotomy, yielding favorable results. Our surgical procedures are discussed.     

Surgery of the dissecting aneurysm involving a right aortic arch

A dissecting aneurysm in association with a right aortic arch is extremely rare. However, a 50-year-old male was diagnosed as having a dissecting aneurysm (DeBakey IIIa) with a right aortic arch, right descending aorta and an aberrant retro-esophageal left subclavian artery. A graft replacement of the right descending aorta was successfully performed under right thoracotomy and partial cardiopulmonary bypass. Precise anatomical definition and proper surgical procedure permitted a successful surgical result.     

Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair

There is uncertainty regarding the best method of repair of interrupted aortic arch. The question is whether to perform primary definitive repair of this anomaly plus the associated defects versus arch repair only and palliation of the intracardiac defects, usually by pulmonary artery banding. Since 1976, 16 infants with interrupted aortic arch have been treated surgically. They were seen at 5.2 +/- 3 days of age and weighed 3.2 +/- 0.7 kg. The interruption occurred between the left carotid and left subclavian arteries (type B) in 9 and between the left subclavian artery and the descending aorta in 7 (type A). Isolated ventricular septal defect (VSD) was the only associated anomaly in 7 and aortopulmonary window, in 4. Two patients had truncus arteriosus type 1. Three had transposition of the great arteries: 1 with VSD and 2 with single ventricle. Prior to 1980, our policy was to palliate all patients. Between 1976 and 1980, 4 infants underwent left thoracotomy with arch repair plus pulmonary artery banding (3, VSD; 7, transposition of the great vessels and single ventricle) with only 1 (25%) survivor. Because of this high mortality, 8 patients with interrupted aortic arch and VSD or aortopulmonary window, seen since 1980, received complete repair with median sternotomy, end-to-end arch anastomosis, and closure of the VSD or aortopulmonary window utilizing profound hypothermia and circulatory arrest. All 8 survived.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hypoplastic circumflex retroesophageal right-sided cervical aortic arch with unusual vascular arrangement and severe coarctation

We report the case of a 12-year-old boy with a hypoplastic retroesophageal circumflex right-sided cervical aortic arch and coarctation. After the incidental finding of a heart murmur when the boy was 9 years old, cardiac magnetic resonance showed a right-sided cervical aortic arch, hypoplastic transverse arch, and separate origin of the left common carotid, right common carotid, right vertebral, and right subclavian arteries. The left subclavian artery arose from the proximal descending aorta next to the coarctation. An extra-anatomical ascending to descending aorta tube graft was inserted through a right lateral thoracotomy with good results.     

Repair of coarctation in a right-sided circumflex retroesophageal aortic arch

We describe an unusual case of a 3-month-old infant with normal cardiac situs and coarctation of the aorta occurring proximal to the right subclavian artery in the presence of a right-sided retroesophageal circumflex aortic arch and aberrant left subclavian artery. Preoperative evaluation with magnetic resonance imaging and conventional aortography led us to surgically approach this lesion through a right thoracotomy with a successful outcome.     

Aortic dissection in the presence of an aberrant right subclavian artery

The presence of an aberrant right subclavian artery arising from the proximal descending aorta is a relatively common anomaly of the aortic arch. These vessels may be involved in aortic dissections, either as the site of the primary intimal tear or as a dissected aortic branch, subject to flow limitation and future aneurysmal degeneration. In this report, we review our experience in treating these lesions in the endovascular era. In two cases, the dissected aorta was treated with an endovascular approach, whereas in two others, proximal open surgical repair was followed by distal stent grafting. The ostium of the aberrant vessel was covered in all cases. Preservation of antegrade flow into the right subclavian was obtained via extra-anatomic bypass. Creation of end-to-side bypasses as opposed to transpositions allowed transbrachial endovascular access to the aberrant right subclavian artery with the purpose of embolization while preserving the right vertebral artery. The hybrid approaches described here effectively address the aortic dissection and degenerative changes associated with an aberrant right subclavian artery while minimizing the need for extensive open repair.     

Single-stage repair of interrupted aortic arch with simultaneous coronary artery bypass grafting without cardiopulmonary bypass in an adult

Interrupted aortic arch is a rare congenital cardiac defect. There are extremely few cases in adults that are reported in the pertinent medical literature. However, single-stage repair of interrupted aortic arch combined with coronary artery disease has been rarely reported. We describe the management of interrupted aortic arch associated with coronary artery disease through single-stage surgical procedure without cardiopulmonary bypass in a 56-year-old woman. The vascular prosthesis was anastomosed between the aortic arch just distal to the origin of the left subclavian artery and the descending thoracic aorta. Then a venous bypass was subsequently formed between the left anterior descending coronary artery, the circumflex artery, and the descending aorta.     

Gleichzeitige Durchführung von Debranching und endovaskulärer Versorgung eines rupturierten Aortenaneurysmas bei aberrierender A. subclavia rechts

A 62-year-old female patient with a known aberrant right subclavian artery (ARSA) and previous endovascular repair of Stanford type-B aortic dissection presented for follow-up. CT revealed a contained rupture of the proximal descending aorta with a maximum diameter of 80 mm involving the aortic arch and the origin of the ARSA. Combined debranching of the supra-aortic vessels via median sternotomy and endovascular obliteration of the thoracic aortic aneurysm was performed. The aberrant ARSA was ligated. Postoperative imaging showed excellent results without endoleak. The postoperative course confirmed tolerance of ARSA ligation. A hybrid approach to the proximal descending aorta is favorable even in cases of aberrant right subclavian artery. A team approach and appropriate planning is essential for success. The English full-text version is available at SpringerLink (under“Suppplemental”).     

Endovascular repair of thoracic aortic aneurysm associated with right-sided aortic arch: report of two cases

Right-sided aortic arch (RAA) is a rare congenital disorder. We describe herein two cases of thoracic aortic aneurysm with a right aortic arch and right-sided descending aorta treated with thoracic endovascular aortic repair (TEVAR). In one case, a 70-year-old man with Edwards type 1 RAA underwent TEVAR using a Relay stent-graft (Bolton Medical, Barcelona, Spain). In another case, a 72-year-old woman with Edwards type 3 RAA underwent TEVAR using a Kawasumi Najuta stent-graft (Kawasumi Laboratories, Inc., Tokyo, Japan) with the “buffalo horn chimney technique”, our original method for left subclavian artery flow preservation. The postoperative courses were uneventful. Postoperative computed tomography showed complete exclusion of the aneurysm without endoleakage. Compared to conventional open surgical repair, TEVAR is challenging in patients with a RAA and right-sided descending aorta. However, our results showed that TEVAR might be feasible and a treatment option even in a patient with a RAA and right-sided descending aorta.     

Undiagnosed aberrant right subclavian artery: pitfall in aortic arch surgery

Selected cerebral perfusion as brain protection via right subclavian artery during aortic arch aneurysm repair adds safety, but may be jeopardized by aortic arch anomalies not readily recognized preoperatively. We describe a case of transverse aortic arch aneurysm repair where an undiagnosed aberrant right subclavian artery was cannulated for selective brain protection.     

Interrupted aortic arch and aortic atresia with circle of Willis-dependent coronary perfusion

We present a case of type B interrupted aortic arch and aortic atresia. This combination is usually incompatible with life. However, the presence of an aberrant right subclavian artery and enough blood flow through the circle of Willis allowed perfusion of the coronary arteries through retrograde carotid and aortic blood flow. A two-ventricular repair was successfully undertaken.     

Endovascular repair of a ruptured thoracic aortic dissection with a right sided aortic arch: A case report

BackgroundEmergency treatment of complex aortic pathology is challenging in the setting of a right-sided aortic arch. We report the successful treatment of a ruptured thoracic aortic aneurysm (TAA) in the setting of a Stanford type B aortic dissection (TBAD) and right-sided aortic arch.Presentation of caseThe patient is a 66-year-old male with chronic kidney disease (CKD) admitted with right sided chest pain and hypotension. Computed tomography angiography (CTA) revealed a 5 cm ruptured TAA in the setting of a TBAD and right-sided aortic arch. The TBAD began just distal to the right common carotid artery and involved the origin of the left subclavian artery (SCA). Using a totally percutaneous approach, a conformable Gore^® TAG^® thoracic endoprosthesis was placed in proximal descending thoracic aorta covering the left SCA. Aside from progression of his pre-existing CKD, the patient had an uneventful recovery. CTA one-month post-procedure revealed a type IB endoleak with degeneration of the distal descending thoracic aorta. To exclude the endoleak, the repair was extended distally using a Medtronic Valiant^® thoracic stent graft. The left subclavian artery was subsequently coil embolized to treat an additional retrograde endoleak. The patient has done well with no further evidence of endoleak or aneurysm expansion.ConclusionRight-sided aortic arch presents challenges in the emergency setting. CTA and post-processing reconstructions are very helpful. While the endoleaks prompted additional interventions, the end result was excellent. This case displays the importance of careful attention to detail and follow-up in these complicated patients.     

Interrupted left aortic arch and isolated right subclavian artery from the right pulmonary artery

We report a 10-day-old newborn, weighing 2.9 kg with an interrupted left aortic arch type B, a large subarterial ventricular septal defect and a right ductus connecting the right pulmonary artery to an isolated right subclavian artery. The patient underwent successful total surgical repair and the isolated right subclavian artery was ligated. He was discharged from hospital without complication and maintains excellent perfusion to the right arm via collaterals.