Clarifying the Anatomy of Tetralogy of Fallot with S-shaped Ascending Aorta.

We recently encountered several cases of tetralogy of Fallot with an abnormally oriented S-shaped ascending aorta. In this retrospective study, we sought to clarify morphology of this unusual under-recognized variant. Databases were reviewed to identify all patients with tetralogy of Fallot having an S-shaped ascending aorta. Computed tomographic angiography was used for the assessment of cardiac morphology. Out of the 21 patients, 18 (86%) had a right aortic arch, 2 (9%) had a left aortic arch, and the remaining patient (5%) had a double aortic arch. Patients with a right aortic arch, compared to age and sex-matched patients with a right aortic arch but normally oriented ascending aorta, had lesser aortic override (29.3 ± 14% vs 54.8 ± 13.2%; p = 0.0001) and a wider ascending aorta (25.2 ± 6.9 vs 18.0 ± 3.2 mm; p = 0.0003). The S-shaped ascending aorta was located posteriorly, with a higher sterno-aortic distance (25.5 ± 7.7 vs 9.9 ± 4.5 mm; p = 0.0001). The ascending aorta among patients with tortuosity was longer (4.12 ± 1.7 vs 3.07 ± 0.82, p = 0.03) but with similar tortuosity index (1.22 ± 0.19 vs 1.15 ± 0.17, p = 0.23). Of the cases with right aortic arch and S-shaped ascending aorta, 16 (89%) had extrinsic compression of the right pulmonary artery (p = 0.0001), while 7 (39%) had crossed pulmonary arteries (p = 0.008), with no such findings among those with normally oriented ascending aorta. Tetralogy of Fallot with an S-shaped ascending aorta is a variant with lesser aortic override and a more posteriorly located ascending aorta. Compression of the right pulmonary artery and crossed pulmonary arteries is frequent in the presence of a right-sided aortic arch. These findings have important implications for optimal diagnosis and surgical repair.

[Right aortic arch with mirror image branching : a rare cause of dysphagia]. / L'image du mois. Arc aortique droit avec image en miroir : une cause rare de dysphagie.

We report the case of a 36-year-old female whose dysphagia revealed a congenital anomaly of the thoracic aorta: the right aortic arch with mirror image branching. This is a rare embryonic developmental anomaly where the aorta wraps around the right bronchus and the supra-aortic trunks emerge from the arch in the opposite order to normal. Most of the patients are asymptomatic unless there is a significant compression of mediastinal structures. Major compression of the esophagus or trachea, aneurysmal disease, dissection of the thoracic aorta, or the presence of a Kommerell diverticulum larger than 2 cm may require a surgical repair. There is no standard treatment and it must be adapted to the clinical presentation and the anatomic configuration of each patient. Our patient did not receive any treatment for her condition.

Nous rapportons le cas d'une patiente de 36 ans dont le tableau de dysphagie a permis de mettre en évidence une anomalie congénitale de l'aorte thoracique : l'arc aortique droit avec image en miroir. Il s'agit d'une anomalie de développement embryonnaire rare où l'aorte s'enroule autour de la bronche souche droite et où les troncs supra-aortiques émergent de la crosse dans l'ordre inverse et opposé à la normale. La grande majorité des patients est asymptomatique, à moins qu'il existe une compression des structures médiastinales. Une compression majeure de l'oesophage ou de la trachée, une maladie anévrismale, une dissection de l'aorte thoracique ou la présence d'un diverticule de Kommerell de plus de 2 cm peuvent justifier une sanction chirurgicale. Il n'y a pas de traitement standard et celui-ci doit être adapté à la présentation clinique et à la configuration anatomique du patient. Notre patiente n'a bénéficié d'aucun traitement pour son affection.

An unusual case of duplicated left brachiocephalic vein with right sided aortic arch and aberrant origin of LSCA in a patient undergoing implantable cardioverter defibrillator (ICD) implantation.

BACKGROUND: Anomalous left brachiocephalic vein (ALBCV) is a rare venous anomaly. Double Left brachiocephalic vein is the rarest type of ALBCV anomaly. CASE REPORT: Here we report a case of gentleman with post myocardial infarction ventricular tachycardia who underwent ICD implantation, where we could not place the lead initially through left side. CT angiography revealed presence of a duplicated circumaortic left BCV. It's cranial limb coursing normally anterior to arch and compressed at its confluence with RBCV and the caudal limb with a subaortic course draining into the RSVC. We report this first case of double LBCV along with right sided aortic arch and aberrant origin of LSCA arising from Kommerel's diverticulum. CONCLUSION: This case highlights that interventional cardiologists should be aware of these venous anomalies for proper planning and implantation of CIED successfully via transvenous approach.

The Impact of Prenatal Diagnosis on Clinical Outcomes of Isolated Vascular Rings From a Statewide Paediatric Cardiology Tertiary Service.

BACKGROUND: Vascular rings, including right aortic arch with aberrant left subclavian artery (RAA-ALSCA), double aortic arch (DAA) and pulmonary artery sling (PAS), are congenital anomalies that may cause airway and oesophageal compression. As prenatal detection has improved, literature comparing clinical outcomes of antenatally versus postnatally diagnosed cases continues to emerge. The aim is to define a statewide tertiary paediatric institution's clinical profile and outcomes of prenatal versus postnatally diagnosed isolated vascular rings. METHOD: A retrospective single-centre review of isolated RAA-ALSCA, DAA and PAS between 1 January 1999 and 31 December 2020 was conducted. Clinical characteristics, surgical and follow-up information were collected. Antenatal and postnatally diagnosed groups were compared. RESULTS: Out of 123 cases diagnosed with isolated vascular rings, 98 (79.7%) cases had RAA-ALSCA, 21 (17.1%) with DAA, 4 (3.3%) with PAS. The antenatal detection rate was 73.6% in the past decade; 20.3% had a genetic disorder, of which 48% had 22q11.21 microdeletion. Of prenatally diagnosed cases, 31.3% developed symptoms, commonly stridor and dysphagia, at a median age of 2.0 months (IQR 0.0-3.0), compared to a median age of diagnosis for the postnatal cohort of 9 months (IQR 1.0-40.7). Postnatally diagnosed cases were more likely to present with symptoms, primarily respiratory distress, than prenatally diagnosed cases (p=0.006). Fifty-nine (59) cases (50% antenatally diagnosed) required vascular ring division; 6.8% had residual symptoms following surgery. DISCUSSION: Antenatal diagnosis has improved and leads to better parental awareness and more timely, appropriate intervention. Postnatally diagnosed patients were older, more likely to be symptomatic, underwent more investigations and were commenced on more medications for symptom management prior to diagnosis. One in five cases of isolated vascular ring anomalies carried a genetic diagnosis, which has important implications on prenatal counselling and genetic testing.

Modified total arch replacement with the frozen elephant trunk technique for a right-sided aortic arch and aortic diverticulum in a teenager.

BACKGROUND: A right sided aortic arch (RAA) and an aortic diverticulum (AD) are a rare congenital anomaly associated with an aortic rupture and dissection. Recently, various methods for the surgical management have been described to treat RAAs and ADs. CASE REPORT: We describe a case of an RAA with a mirror image branching (RAMI) and AD in a teenager who complained of chest pain. We planned a total arch replacement with the frozen elephant trunk technique. Further, we needed to devise a way to reconnect the cerebral vessels because of his small arch anatomy and limited space in the mediastinum. We performed a right subclavian artery debranching, creating an anastomosis using one graft and diamond anastomosis, and performed the left innominate and right carotid anastomosis in an island fashion. This surgical approach could be a treatment option for young or small patients with RAMIs and ADs, whose arch anatomy is very small.

Surgical treatment of esophageal cancer with anomaly of the aortic arch and its branches.

BACKGROUND: Anomalies of the aortic arch and its branches rarely develop. The surgery for esophageal cancer may be challenging with the presence of these anomalies. This study is aimed to analyze the influence of these variations during the esophagectomy. METHODS: A total of 21 patients with aortic arch and brachiocephalic vessel variations were retrospectively identified from 2013 to 2019. Anomalies were distributed: 15 patients with left-sided aortic arch combined with aberrant right subclavian artery (LAA + ARSA), 2 right-sided aortic arch with mirror-image arch branches (RAA + MIAB), and 4 right-sided aortic arch combined with aberrant left subclavian artery (RAA + ALSA). Perioperative characteristics and long-term survival were analyzed. RESULTS: Tumors were mostly located in the upper and middle thorax (42.9% and 47.6%, respectively). Of the 15 patients with LAA + ARSA, 13 underwent McKeown esophagectomy and 2 via transhiatal approach. Left thoracotomy was performed on all 6 patients with RAA. The R0 resection rate was 90.5% (19/21). Recurrent laryngeal nerve (RLN) injury occurred in 2 patients. Two patients died within 30 days postoperatively. Lymph node yield was 23.6 ± 1.2, with a metastasis rate of 38.1% (8/21). The median follow-up time was 18 months. Recurrence occurred in 6 patients (11-35 months) and 4 patients died after recurrence with a median time to death of 21 months (8-47 months). CONCLUSION: For resectable esophageal cancer combined with aortic arch and its branches anomalies, satisfactory surgical results can be obtained under careful preoperative evaluation and reasonable surgical approach selection.

Association of variant arch anatomy with type B aortic dissection and hemodynamic mechanisms.

OBJECTIVE: Congenital aortic arch variations are more common in patients with thoracic aortic disease for reasons unknown. Additionally, little is understood about their relation to type B aortic dissections (TBAD) specifically. We investigated the prevalence of variant aortic arch anatomy in patients with TBAD compared with controls. To understand the implications of how variant aortic arch anatomy may contribute to degenerative aortic disease, we compared flow hemodynamics of three variations of aortic arches using four-dimensional flow magnetic resonance imaging (4D flow MRI). METHODS: Arch anatomy on computed tomography imaging was reviewed and compared between patients with TBAD and age/sex-matched controls free of aortic pathology. Arch variants were defined as follows: common origin of innominate and left common carotid artery (bovine arch), aberrant right subclavian artery, and right-sided aortic arch. Demographics, TBAD characteristics, and follow-up data were abstracted. Patients with TBAD with variant and conventional aortic arches were compared. Additionally, three matched healthy controls with conventional, bovine, and aberrant right subclavian artery arches underwent 4D flow MRI evaluation to assess if there were differences in flow patterns by arch type. Indices of regional hemodynamic wall sheer stress were compared. RESULTS: Computed tomography scans of 185 patients with TBAD (mean age, 58.1 ± 12.4 years; 72.4% males; 71.4% Caucasian) and 367 controls (mean age, 62.5 ± 13.4 years; 67% males; 77.9% Caucasian) were reviewed. Variant arch anatomy was more prevalent in patients with TBAD (40.5% vs 24.5%; P < .001). In patients with TBAD, there were no differences in the mean age of presentation and descending thoracic aorta diameter among those with variant or conventional arch anatomy. Patients with TBAD with variant arch anatomy had a higher percentage of dissection related thoracic aortic repairs (54.7% vs 33.6%; P = .004) with repairs occurring predominantly in the acute phase. 4D flow MRI demonstrated a higher systolic wall shear stress along the inner curve of the bovine arch compared with the conventional aberrant right subclavian artery arches. CONCLUSIONS: Variant aortic arch anatomy is significantly more prevalent in patients with TBAD. patients with TBAD with variant arch anatomy had a higher percentage of dissection-related aortic repair. Preliminary 4D flow MRI data show differences in hemodynamic flow patterns between variant and conventional arches. Studies of long-term outcomes based on arch anatomy may offer additional insight to TBAD genesis and possibly influence management decisions.

Video-assisted thoracoscopic right upper lobectomy in a patient with a right-sided aortic arch and Kommerell diverticulum.

BACKGROUND: It is a very rare condition for a patient to have right lung cancer and a right-sided aortic arch simultaneously. Right lobectomy under video-assisted thoracoscopic surgery (VATS) in such a patient is a challenging procedure that is seldom reported. We successfully performed a VATS right upper lobectomy in a 77-year-old female with a right-sided aortic arch and Kommerell diverticulum. CASE PRESENTATION: A 77-year-old woman was referred to our division for a mixed ground-glass opacity lesion in the right upper lung. A right-sided aortic arch with Kommerell diverticulum was identified by preoperative 3D CT reconstruction. A VATS right upper lobectomy with radical mediastinal lymph node dissection was performed, and the final histological staging was Ia3 (pT1cN0M0). The patient was discharged without any complications. CONCLUSIONS: We conclude that the video-assisted thoracic surgery can be safely performed in such conditions. It is difficult to determine the extent of upper mediastinal lymph node dissection in such cases.

The right-sided aortic arch with unusual course of bilateral recurrent laryngeal nerves: a report of rare variations.

We describe a rare case of the right-sided aortic arch, the unusual origin of the main arterial vessels and the unusual courses of bilateral recurrent laryngeal nerves in a Japanese cadaver. Chiefly, the right-sided aortic arch turned to the left side from the dorsal part of the trachea and esophagus, and Kommerell's diverticulum was found at the end of the arch. The right common carotid artery arose from the end part of the ascending aorta, but the left common carotid artery arose from the proximal portion of the ascending aorta. The right subclavian artery arose from the upper edge of the aortic arch, but the left one arose from the front wall at the upper side of the ligamentum arteriosum. The right recurrent laryngeal nerve hooked around the aortic arch (but not the right subclavian artery) dorsoventrally, and the left recurrent laryngeal nerve hooked around the ligamentum arteriosum and arose from the ventral side (but not dorsal) of the aortic arch. These variations are very rare, and understanding them is useful and important for clinical research.

Kommerell's diverticulum: A rare aortic arch anomaly.

Anomalies of the aortic arch associated with diverticulum are rare. We present a case of incidentally detected right-sided aortic arch with Kommerell's diverticulum and aberrant left subclavian artery.

Anomalous origin of the right pulmonary artery from the ascending aorta accompanied by absent pulmonary valve syndrome and right-sided aortic arch: a rare case in adult congenital heart disease.

We present a rare congenital heart disease in a 20-year-old man with anomalous origin of the right pulmonary artery from the ascending aorta, accompanied by absent pulmonary valve syndrome, and a right-sided aortic arch suspected initially in transthoracic echocardiography and subsequently confirmed by cardiac catheterisation and computed tomography angiography.

Right upper lobectomy with mediastinal dissection under uniportal video-assisted thoracoscopic surgery for lung cancer in a patient with a right-sided aortic arch: a case report.

BACKGROUND: A right-sided aortic arch is a rare congenital vascular structure variation. Right lobectomy is not commonly performed on patients with such a condition. Further, there are no reports on lobectomy under uniportal video-assisted thoracoscopic surgery (VATS) in this patient group. CASE PRESENTATION: A 67-year-old man with a right-sided aortic arch and Kommerell diverticulum underwent right upper lobectomy with mediastinal lymph node dissection under uniportal VATS for primary lung cancer. Due to the right descending aorta, which narrows the space of the dorsal hilum, handling of the stapler for stapling the right upper lobe bronchus from the uniport in the 6th intercostal space at the medial axillary line can be challenging. This issue was resolved by manipulating the staple over the azygos vein toward the inferior margin of the aortic arch. Via mediastinal lymphadenectomy, we found that the right recurrent laryngeal nerve branched from the right vagus nerve and hooked around the right-sided aortic arch. CONCLUSIONS: Right lobectomy with mediastinal lymph node dissection under uniportal VATS can be performed for lung cancer in patients with a right-sided aortic arch.

Interarm Blood Pressure Difference Revealing a Right-Sided Aortic Arch and Occluded Aberrant Left Subclavian Artery.

We present the case of a 33-year-old patient with a rare combination of a right-sided aortic arch and occluded asymptomatic aberrant left subclavian artery diagnosed after the coincidental finding of an interarm blood pressure difference. Because there were no symptoms of local compression or subclavian steal, conservative management was suggested.

Case Report: Successful Treatment of Aberrant Left Subclavian Artery With a Right-Sided Aortic Arch.

Right sided aortic arches with concomitant aberrant left subclavian arteries are exceedingly rare anatomical variants. We present a case of a 45 year old male that presented with symptoms consistent with dysphagia and known right sided aortic arch with an aberrant left subclavian artery. Though previous reports of repair have indeed been reported, we confirm that a hybrid approach to these anatomic variants remain feasible and with symptom resolution for patients.

Treatment of right-sided aortic arch aneurysms with aberrant left subclavian artery with Kommerell's diverticulum using the frozen elephant trunk technique.

OBJECTIVES: The ideal treatment for aneuryms of aberrant left subclavian arteries with Kommerell's diverticulum arising from right aortic arches remains open. METHODS: Between January 2015 and December 2020, 5 patients with aneurysms from a right-sided aortic arch with aberrant left subclavian artery and Kommerell's diverticulum underwent repair by using the frozen elephant trunk technique in 3 aortic centres. Patients' characteristics were retrospectively reviewed and the surgical procedure and outcomes are presented. RESULTS: The median age of the 2 male and 3 female patients was 59 (range from 49 to 63) years. The median operative times were as follows: surgery 405 min (range from 335 to 534), cardiopulmonary bypass time 244 min (range from 208 to 280) and aortic clamp time 120 min (from 71 to 184). The mean core temperature was 25.94°C (from 24 to 28). The intensive care unit stay was 4 days (range from 1 to 8) and the in-hospital stay 21 days (from 16 to 34). All patients were discharged and we observed no stroke or spinal cord ischaemia postoperatively. During the median follow-up time of 1003 days (range from 450 to 2306), 3 patients required subsequent thoracic endovascular distal stent graft extension. CONCLUSIONS: The frozen elephant trunk technique is a good treatment option for patients with aneuryms of an aberrant left subclavian artery with Kommerell's diverticulum arising from right aortic arches. Secondary stent graft extension is a frequently needed component of the treatment concept.

Hybrid management of type B aortic dissection in a patient with right-sided aortic arch and aberrant left subclavian artery.

This report describes a patient with a right-sided aortic arch, aberrant left subclavian artery and Kommerell diverticulum, who presented with aneurysmal degeneration of the aortic root to the descending aorta, in addition to an acute type B2-10 aortic dissection. He underwent hybrid treatment with a valve-sparing aortic root replacement, transverse arch replacement with reattachment of the right subclavian artery, bilateral common carotid arteries, and thoracic endovascular aneurysm repair with left subclavian artery embolization and a left common carotid to subclavian artery bypass.

Accidental central venous catheter cannulation into aberrant arterial anatomy requiring endovascular intervention.

Central venous catheter placement continues to be an extremely common procedure throughout hospital systems. Although ultrasound guidance can mitigate some placement risks, misplacement of lines into neighboring structures, such as arteries, remains an unfortunate complication. In this report, we will discuss an 83-year-old female with aberrant left subclavian artery and right sided arch, which provided for successful stent graft coverage of arterial injury secondary to accidental subclavian artery cannulation with a central venous catheter with preservation of the right common carotid artery and avoidance of a potentially morbid sternotomy.

Endovascular Repair of an Intrathoracic Subclavian Artery Aneurysm in a Patient With Dextrocardia.

This manuscript describes an endovascular repair of a symptomatic, large proximal left subclavian artery aneurysm in a patient with dextrocardia and right-sided aortic arch and absent bilateral internal carotid arteries. The patient had surgical reconstruction as an infant for congenital heart disease with Ventricular Septal Defect, bifid sternum . Given her previous surgical history, we declined an open operation and performed an endovascular repair with stent grafts to successfully repair the subclavian artery aneurysm. The patient had an uneventful postoperative course and follow-up ultrasonography demonstrated successful repair with preservation of flow through the left subclavian and vertebral arteries with resolution of her symptoms.

Endovascular Treatment Strategy for Acute Basilar Artery Occlusion with Unexpected Right-Sided Aortic Arch.

A right-sided aortic arch (RAA) is a rare variant of the aortic arch found. We present a case of endovascular treatment (EVT) for acute basilar artery (BA) occlusion with an RAA. A 70-year-old man developed acute ischemic stroke due to BA occlusion. During urgent EVT for BA occlusion with an RAA, which was observed unexpectedly, navigation of the catheter to the target vessel was achieved as a result of an early change in the access route. Flexible treatment strategies are warranted for cases of unexpected anatomical variant in the fight against time for emergent cases, such as acute ischemic stroke.

Hybrid repair for Kommerell's diverticulum and right aortic arch with aberrant right vertebral artery.

Kommerell's diverticulum (KD) is a rare aneurysm of the origin of an aberrant subclavian artery. Hybrid aortic arch repair for KD is being performed more often. We report hybrid arch repair for KD in a 63-year-old man with a right aortic arch and aberrant right vertebral artery, an extremely rare variant. We performed total arch replacement to completely reconstruct the five cervical arteries with elephant trunk to create an adequate landing zone, followed by second-stage endovascular stent-grafting from the ascending aorta to the proximal descending aorta.