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.

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.

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.

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.

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.

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.

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.

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.

Debranching Thoracic Endovascular Aortic Repair for Kommerell's Diverticulum with Right-Sided Aortic Arch / 日本心臓血管外科学会雑誌

We report a case of debranching thoracic endovascular aortic repair for Kommerell's diverticulum with right-sided aortic arch in 78-year-old women. The computed tomography (CT) demonstrated Kommerell's diverticulum with a right-sided aortic arch and the trachea and esophagus were compressed by the diverticulum. The diverticulum had a maximum diameter of 32 mm, and surgical intervention was chosen because of the aneurysmal change and the possibility of rupture. We performed endovascular aortic repair for Kommerell's diverticulum with a right-sided aortic arch because of low lung function and low frailty. The patient was discharged on the 21st postoperative day. There was no evidence of aortic event during 2 years follow up.

Two Cases of Descending Aortic Diverticulum Associated with the Right-Sided Aortic Arch / 日本心臓血管外科学会雑誌

Case 1 is a 70-year-old male. He has a history of cholelithiasis and left inguinal hernia. A preoperative examination of the inguinal hernia showed the enlargement of the mediastinal shadow, and he was referred to our department. A close examination revealed a right-sided aortic arch, a right descending aorta, and a descending aortic diverticulum. No subjective symptoms, intracardiac malformations, or other cardiovascular diseases were observed. The surgery was scheduled for descending aorta replacement including a diverticulum with right posterior lateral 4th intercostal thoracotomy and lower body partial extracorporeal circulation. However, due to aortic intima injury at the proximal end, hypothermic cerebral circulatory arrest and proximal anastomosis were performed by the open proximal method. There was no problem with the postoperative course, and he was discharged 19 days after surgery. Case 2 is a 51-year-old female. Born in China, she has lived in Japan for 15 years. No notable history. An abnormal shadow was shown on chest Xp performed in a medical examination, and aortic malformation was suspected on chest CT. She was referred to our department. The diagnosis was right-sided aortic arch, right descending aorta, aberrant left subclavian artery, and Kommerell diverticulum. There were no subjective symptoms and no intracardiac malformations. The operation was a two-stage operation. As the initial surgery, median sternotomy was performed, total arch replacement with intrathoracic reconstruction of the left subclavian artery, and open stent graft insertion, and the Kommerell diverticulum was covered with an open stent graft. We did not treat the diverticulum because it was located on the dorsal side. At 15 days after surgery, we performed embolization of the origin of the left subclavian artery from the Kommerell diverticulum. There was no problem with the postoperative course, and she was discharged 19 days after the initial surgery.

Right-sided aortic arch with complex anomalies presented with transient ischemic attack.

Background: The right-sided aortic arch (RAA) is an uncommon anatomical anomaly found in <0.1% of the adult population. In this article, we report a case of RAA anomaly with an aberrant left subclavian artery (ALSA) and Kommerell's diverticulum associated with aneurysmal dilation of the ascending aorta, left carotid artery (CCA) stenosis, and pancake kidney presented with a transient ischemic attack (TIA). To the best of our knowledge, this is the first case in the literature that discusses such associations, especially in a symptomatic patient with neurological rather than tracheaesophageal symptoms and in the absence of the steal phenomenon. Case Description: A 52-year-old male, with a history of recurrent multiple TIAs, presented immediately after the onset of blurred vision and left-sided weakness. The initial diagnostic cerebral angiogram revealed a left CCA stenosis of <30%, with normal posterior circulation vasculature. The diagnosis of RAA was made with computed tomography angiography (CTA) of the thoracic and abdominal aorta, which revealed Type 2 RAA, with ALSA, which had a bullous dilatation at its origin that suggests Kommerell's diverticulum. Another two findings on CTA were a persistent left-sided superior vena cava that ended in the coronary sinus and a single pelvic fused renal mass (Pancake kidney). Conclusion: We presented an extremely rare case of RAA with ALSA associated with a group of extra rare anomalies. Understanding the anatomical variants of RAA and its characteristics is critical to improving the management and follow-up of patients with such anomalies.

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.

Arteria Lusoria With Right-Sided Aortic Arch and Atrial Septal Defect Associated With Extensive Thrombosis and Paradoxical Embolism: A Case Report.

In this report, we present an incidental finding of a rare combination of an Aberrant Right Subclavian Artery (ARSA), or arteria lusoria, with right-sided aortic arch and atrial septal defect associated with extensive thrombosis and paradoxical embolism causing acute stroke in an octogenarian woman with COPD presenting with acute hypercapnic respiratory failure. We also discuss the various surgical approaches for management and conservative treatment alternatives in non-surgical candidates (as in this case). We believe that this is the first reported case of these combined rare anomalies in an asymptomatic patient to the best of our knowledge.

Right-sided aortic arch with Kommerell's diverticulum: a case report of a rare cause of type B dissection.

Background: A right-sided aortic arch (RAArch) is present in approximately 0.1% of the population. A Kommerell's diverticulum (KD), a remnant of the dorsal aortic arch usually refers to an aneurysmal aortic enlargement at the origin of an aberrant left subclavian artery (ALSA) and is associated with an increased risk of aortic dissection. Case summary: A 59-year-old female smoker with a history of hypertension and hypercholesterolaemia presented with a 24-hour history of sudden-onset and severe stabbing chest pain radiating to the interscapular region. Physical examination was normal except for bilateral basal crepitations. Computed tomography angiography (CTA) showed a type B aortic dissection in a RAArch with an ALSA arising from KD with a peri-aortic haematoma and haemothorax without any active contrast extravasation. After medical stabilization, a semi-urgent hybrid repair was performed with a right carotid-subclavian bypass, thoracic endovascular aortic repair (TEVAR), a plug in the left subclavian artery, and left carotid-subclavian bypass due to severe ischaemia of the left arm. The postoperative CTA showed patent bypasses, aortic remodelling, and a minimal type IIa endoleak at the level of the ALSA. Discussion: In patients with a type B dissection and KD, hybrid repair including TEVAR is feasible after careful pre-operative assessment of the patient's unique anatomy and may reduce post-surgical morbidity and mortality compared to open surgery. Prophylactic repair may be considered in patients with an asymptomatic RAArch and KD.

Successful treatment of patent ductus arteriosus accompanying right-sided aortic arch and aberrant left subclavian artery: a case report.

Background: The coexistence of a right-sided aortic arch (RAA), an aberrant left subclavian artery (ALSA), and a patent ductus arteriosus (PDA) is a rarely seen vascular ring anomaly. There is currently no general guideline consensus on the management and follow-up of this congenital defect, posing a challenge to the clinicians. At this point, the heart team plays a critical role in the management of the disease. Case summary: In the present case, a 25-year-old male patient was presented to the outpatient clinic with dyspnoea and fatigue. A transthoracic echocardiography revealed PDA with a left-to-right shunt. To evaluate the anatomy thoroughly, a thoracic computed tomographic angiography was performed and showed PDA accompanying ALSA and RAA. The patient was evaluated by the Heart Team, and a percutaneous closure of PDA was recommended due to signs of left ventricular volume overload. The closure was successfully performed with Amplatzer vascular plug II. At follow-up, the patient was free of symptoms. Discussion: Clinicians should be aware of the potential concomitant lesions during the diagnostic work-up. In selected patients, percutaneous closure of PDA may be the first-line therapy in experienced centres.

The dilemma after an unforeseen aortic arch anomalies at thoracoscopic repair of esophageal atresia: Is curtailing surgery still a necessity?

BACKGROUND AND OBJECTIVE: There are several forms of relevant epi-aortic branching anomalies, and perhaps that is why different views as to the best approach have been reported. To help resolve this dilemma, we examined the unforeseen arch anomalies found at thoracoscopic repair of esophageal atresia and the outcomes. METHODS: In a retrospective cohort, all consecutive patients who were thoracoscopically approached for esophageal atresia over a 5-year period with unforeseen aortic/epi-aortic branching were identified and grouped. Thoracoscopic views, operative interventions, and outcomes were studied. RESULTS: A total of 121 neonates were thoracoscopically approached for EA, of whom 18 cases with aberrant aortic architecture were selected. Four (3%) cases were diagnosed on a preoperative echocardiography as a right-sided aortic arch, whereas unforeseen anomalous anatomies were reported in 14 cases (11.6%): left aortic arch with an aberrant right subclavian artery (ARSA) (n = 10), right-sided aortic arch with an aberrant left subclavian artery (ALSA) (n = 3), and mirror-image right arch (n = 1). Single postoperative mortality was reported among the group with left arch and ARSA (10%), whereas all the cases with right arch and ALSA died. CONCLUSIONS: In all, 11.6% of the studied series exhibited unexpected aberrant aortic architecture, with higher complication rates in comparison to the typical thoracoscopic repairs. For EA with left aortic arch and ARSA, the primary esophageal surgery could safely be completed. Meanwhile, curtailing surgery-after ligating the TEF-to get advanced imaging is still advised for both groups with the right arch due to the significant existence of vascular rings.