Right-Sided Aortic Arch: A Computed Tomography Angiography Investigation, A Systematic Review with Meta-Analysis.

Background/Objectives: The right-sided aortic arch (RAA) is an uncommon variation of the aortic arch (AA), characterized by the aorta crossing over the right main bronchus. In the RAA, the descending aorta can be found on either the right or left side of the spine. The current study comprises a comprehensive retrospective computed tomography angiography (CTA) investigation into the prevalence of the RAA within the Greek population. Additionally, we will conduct a systematic review and meta-analysis to elucidate both common and rare morphological variants of the RAA. This research is significant as it sheds light on the prevalence and characteristics of the RAA in a specific population, providing valuable insights for clinical practice. Methods: Two hundred CTAs were meticulously investigated for the presence of a RAA. In addition, the PubMed, Google Scholar, and Scopus online databases were thoroughly searched for studies referring to the AA morphology. The R programming language and RStudio were used for the pooled prevalence meta-analysis, while several subgroup analyses were conducted. Results: Original study: A unique case of 200 CTAs (0.5%) was identified with an uncommon morphology. The following branches emanated from the RAA under the sequence: the right subclavian artery (RSA), the right common carotid artery (RCCA), the left common carotid artery (LCCA), and the left vertebral artery (LVA) in common origin with the aberrant left subclavian artery (ALSA). The ALSA originated from a diverticulum (of Kommerell) and followed a retroesophageal course. Systematic Review and Meta-Analysis: Sixty-two studies (72,187 total cases) met the inclusion criteria. The pooled prevalence of the RAA with a mirror-image morphology was estimated at 0.07%, and the RAA with an ALSA was estimated at <0.01%. Conclusions: AA anomalies, specifically the RAA, raise clinical interest due to their coexistence with developmental heart anomalies and possible interventional complications. Congenital heart anomalies, such as the Tetralogy of Fallot and patent foramen ovale, coexisted with RAA mirror-image morphology.

A case of right aortic arch type II combined with Kommerell's diverticulum and left innominate vein beneath the aortic arch.

The combination of the right aortic arch and aberrant left subclavian artery (ALSA) with Kommerell's diverticulum (KD) is rare to coexist with the left innominate vein (LINV) beneath the aortic arch. It escalates the surgical risk undoubtedly and increases the difficulty of clinical procedures. We report one case diagnosed by Ultrasound and Computed Tomography Angiography (CTA).

Right-Sided Aortic Arch With Isolated Left Subclavian Artery, Subclavian Steal Syndrome, and Right Bronchial Compression: A Case Report.

A right-sided aortic arch with an isolated left subclavian artery represents a rare anatomical variant, posing diagnostic challenges and clinical complexities. Here, we present a case of a 14-year-old male presenting with respiratory symptoms, unveiling a right-sided aortic arch with an isolated left subclavian artery. Through detailed clinical evaluation, radiographic imaging, and diagnostic modalities including chest radiography, computed tomography angiography, ultrasound, and time-of-flight magnetic resonance angiography, the anatomical features and associated complications were delineated. The discussion encompasses embryological underpinnings, clinical manifestations, and therapeutic considerations, shedding light on the rarity and clinical implications of this anomaly.

Case Report: Right aortic arch with isolation of left brachiocephalic artery and ventricular septal defect.

Right aortic arch with isolation of left brachiocephalic artery is a rare congenital aortic arch anomaly. Herein, we reported a case of this rare anomaly with ventricular septal defect in a 9-month-old infant. We successfully reconstructed the islolated left brachiocephalic artery and repaired the ventricular septal defect in one stage.

Navigating the Unexpected: Right Aortic Arch Unveiled During Left Heart Catheterization.

A right aortic arch (RAA) is an extremely rare congenital anomaly with seven identified variants. While most variants are asymptomatic, those with a vascular ring can be associated with severe symptoms. We present an incidental RAA finding during left heart catheterization (LHC) in a 68-year-old female with multiple morbidities presented with worsening angina. Her echocardiogram was significant for inferolateral wall akinesia, prompting LHC. The procedure was challenging with an unexpected course of the guide wire distally behind the cardiac shadow. Pressure tracing confirmed arterial access and contrast injection revealed RAA. A subsequent aortic computed tomography angiography (CTA) confirmed RAA with mirror-image branching. Abnormal origin and angle of aortic arch branches pose challenges in choosing the proper access. We used the right radial artery approach, but the left radial approach may be superior in providing a more proximal access and avoiding the abnormal origin of the right subclavian artery (RSA). Choosing the appropriate angiographic view is also of utmost importance, and the right anterior oblique view provided better visualization in our case. Aortic arch anomalies are confirmed by a CTA or magnetic resonance angiography (MRA) of the aorta. This case underscores the importance of identifying the aortic arch anomalies and the imposed challenges during the LHC.

Prenatal echocardiography diagnosis of a novel combination of bilateral ductus arteriosus and cardiovascular anomalies: a case report and literature review.

Background: Bilateral ductus arteriosus (BDA) is a relatively rare vascular malformation. According to the double arch theory, BDA is formed when the distal ends of the sixth pairs of primitive arches on the left and right sides have not regressed. We describe a fetus with prenatal echocardiographic findings of BDA and right aortic arch mirror-image branching (RAA-MIB) combined with congenital heart disease. Furthermore, to gain a deeper understanding of the embryological mechanism of BDA, we review the literature on all combinations of BDA present in 40 fetuses/infants. Case summary: A 22-year-old female patient underwent fetal echocardiography at 23 weeks of gestation. Both the two-dimensional (2D) grayscale image and color Doppler flow imaging (CDFI) revealed dextro-transposition of the great arteries combined with a ventricular septal defect and RAA-MIB. The following scan revealed a rare vascular ring, which was identified as BDA extending from the confluent of the left pulmonary artery and right pulmonary artery, completely encircling the trachea to form an "O"-shaped vascular ring before finally converging into the descending aorta. A persistent left superior vena cava was also observed. We subsequently used four-dimensional (4D) color Doppler imaging with the spatiotemporal image correlation (STIC) HD live flow and STIC HD live flow silhouette mode to clearly display ventricular arterial connectivity and the direction of vessel travel. Adjusting the image quality and display angle is very important when applying STIC. The 4D images confirmed our diagnosis. After multidisciplinary counseling and discussion with her family, this female patient decided to terminate the pregnancy. Conclusion: Our review of the literature summarized nine combinations classified into three types of BDA and aortic arch pathology. However, our case differs because it is a novel combination of intracardiac structural abnormalities and vascular rings in a fetus. Prenatal ultrasound diagnosis of BDA is important and requires a combination of 2D grayscale, CDFI, and STIC images to assist in scanning.

Advances in Minimally Invasive Procedures of the Thoracic Cavity.

Significant advances in veterinary minimally invasive surgeries and procedures have occurred in the past 10 years. These advances have been allowed due to continual research into optimizing working space through one-lung ventilation techniques and carbon dioxide insufflation. Additionally, minimally invasive surgery enthusiasts have joined forces with interventionalists and, in many cases, physicians to push the boundaries, minimize pain, suffering, and time away from owners with advances in a variety of procedures. Several larger multi-institutional retrospective studies on various disease processes allow veterinarians and owners to understand that minimally invasive approaches allow for outcomes comparable to traditional open surgery and, in some cases, may now be considered the standard of care in canine and feline patients.

Dysplastic aberrant left subclavian artery originating from a thoracic intersegmental artery associated with a right aortic arch.

PURPOSE: A right aortic arch (RAA) is a rare vascular anomaly that often coexists with an aberrant left subclavian artery (ALSA). Due to the rarity of RAA, the development of an ALSA is not well understood. METHOD: We describe a case in which a 58-year-old man who was scheduled to undergo posterior decompression and fusion surgery for thoracic ossification of the posterior longitudinal ligament from Th1 to Th3 was found to have a RAA and an ALSA. RESULTS: Preoperative computed tomography angiography demonstrated a RAA and an ALSA. The ALSA was extremely tortuous and ran in the paraspinal muscles behind the thoracic laminae, which meant it was in the surgical field. The ALSA arose from the descending aorta and bifurcated into the left segmental arteries of Th1 and Th2, and also bifurcated into the left vertebral artery, which had a normal subsequent course. The dysplastic ALSA was considered to have developed from the thoracic intersegmental artery. Based on preoperative examination findings, we performed spinal surgery without vessel injury. CONCLUSION: We report a rare case of a dysplastic ALSA that developed from the thoracic intersegmental artery with a RAA. The knowledge of this anomaly provides safety in spinal surgery of the cervicothoracic junction.

Associated Anomalies and Outcome in Patients with Prenatal Diagnosis of Aortic Arch Anomalies as Aberrant Right Subclavian Artery, Right Aortic Arch and Double Aortic Arch.

We aimed to evaluate retrospectively associated anomalies and outcome in prenatal aortic arch anomalies (AAAs). We included ninety patients with aberrant right subclavian artery (ARSA), right aortic arch (RAA) with mirror image branching (RAA-mirror) or aberrant left subclavian artery (RAA-ALSA) and double aortic arch (DAA) between 2011 and 2020. In total, 19/90 (21.1%) had chromosomal anomalies, the highest rate being within the ARSA subgroup (17/46, 37%). All (13/13) of the RAA-mirror subgroup, 10/27 (37.0%) of RAA-ALSA, 13/46 (28.3%) of ARSA and 0/4 within the DAA subgroup had additional intracardiac anomaly. The rate of extracardiac anomalies was 30.7% in RAA-mirror, 28.3% in ARSA, 25.0% in DAA and 22.2% in the RAA-ALSA subgroup. A total of 42/90 (46.7%) had isolated AAAs: three (7.1%) with chromosomal anomalies, all trisomy 21 (3/26, 11.5%) within the ARSA subgroup. Out of 90, 19 (21.1%) were lost to follow-up (FU). Two (2.2%) intrauterine deaths occurred, and six (6.7%) with chromosomal anomalies terminated their pregnancy. In total, 63 (70.0%) were liveborn, 3/63 (4.8%) with severe comorbidity had compassionate care and 3/60 (5.0%) were lost to FU. The survival rate in the intention-to-treat cohort was 53/57 (93%). Forty-one (77.4%) presented with vascular ring/sling, two (4.9%) with RAA-ALSA developed symptoms and one (2.4%) needed an operation. We conclude that intervention due to vascular ring is rarely necessary. NIPT could be useful in isolated ARSA cases without higher a priori risk for trisomy 21 and after exclusion of other anomalies.

[Right-sided upper lobectomy in a patient with right aortic arch: a case report and literature review]. / Verkhnyaya lobektomiya sprava u patsienta s pravostoronnei dugoi aorty: sluchai iz praktiki s kratkim obzorom literatury.

A right aortic arch is an anomaly of prenatal development characterized by location of aortic arch to the right from tracheal-esophageal complex. This variant of anatomy is usually asymptomatic and diagnosed accidentally. We performed open upper lobectomy for cancer of the upper lobe of the right lung in a patient with aortic arch dextraposition. Classical right-sided upper lobectomy in patients with right aortic arch is associated with certain difficulties. The most difficult objective is total excision of lymph nodes because trachea is hidden under aortic arch. A specific complication may be postoperative hoarseness associated with iatrogenic damage to the right recurrent laryngeal nerve.

Right-sided aortic arch with isolation of the left innominate artery and hypoplasia of the left internal carotid artery.

PURPOSE: Here, we report a case of the right-sided aortic arch with isolation of the left innominate artery and hypoplasia of the left internal carotid artery. METHODS: A 42-year-old male patient underwent a whole-body computed tomography angiography (CTA) examination upon the clinical suspicion of vasculitis. RESULTS: CTA revealed a right-sided aortic arch with the isolation of the left innominate artery and hypoplasia of the left internal carotid artery. CONCLUSION: The right-sided aortic arch, with the isolation of the left innominate artery, is a scarce vascular variation that may occur with other cardiovascular anomalies such as ventricular septal defect. It can be asymptomatic or can present with symptoms of subclavian steal syndrome. Although its association with the agenesis of the left internal carotid artery has been reported, its association with the hypoplasia of the left internal carotid artery has not been reported previously to the best of our knowledge.

Perioperative and Anesthetic Considerations in Vascular Rings and Slings.

Vascular rings represent an increasingly prevalent and diverse set of congenital malformations in which the aortic arch and its primary branches encircle and constrict the esophagus and trachea. Perioperative management varies significantly based on the type of lesion, its associated comorbidities, and the compromise of adjacent structures. Multiple review articles have been published describing the scope of vascular rings and relevant concerns from a surgical perspective. This review seeks to discuss the perioperative implications and recommendations of such pathology from the perspective of an anesthesia provider.

Increased Pulmonary-Aortic Interspace in Fetal Right Aortic Arch: A Matched Case-Control Study.

INTRODUCTION: The prenatal detection rate of a right aortic arch (RAA) has increased with the implementation of the three-vessel view (3VV) to the second-trimester anomaly scan formed by the pulmonary artery (PA), aorta (Ao), and superior vena cava (SVC). We examined the value of measuring the distance between PA and Ao in the 3VV in cases with an RAA. METHODS: We conducted a case-control study in which fetuses with an isolated RAA were matched to 3 healthy controls. Using 3VV images, the distances between PA, Ao, and SVC were measured and the ratio between PA to Ao (PAAo) distance and Ao to SVC (AoSVC) distance was calculated. RESULTS: Fifty-four RAA cases and 162 matched controls were included. The mean absolute distance PAAo was 3.1 mm in cases and 1.8 mm in controls (p < 0.001), and the mean PAAo/AoSVC ratio was 2.9 and 1.4, respectively (p < 0.001). The ROC curve of PAAo/AoSVC ratio showed a cut-off point of 1.9 with sensitivity and specificity over 87% for the diagnosis of RAA. CONCLUSIONS: The pulmonary-aortic interspace and the PAAo/AoSVC ratio were significantly larger for RAA cases as compared to controls. If an increased pulmonary-aortic interspace is observed, a PAAo/AoSVC of ≥1.9 can be helpful in the diagnosis of an RAA.

Surgical treatment for right-side aortic arch concomitant with Kommerell's diverticulum: techniques selection and follow-up results.

BACKGROUND: Right-side aortic arch concomitant with Kommerell's diverticulum (KD) is a rare and complex ailment, and there is no consensus on the optimal strategy to deal with this congenital anomaly. We retrospectively analyzed and summary of the cases treated in our center with individual treatment methods for different situations. METHODS: Between September 2018 and December 2021, 10 patients experienced surgical therapy at our institution who presented with a Kommerell's diverticulum arising from an aberrant subclavian artery from the right-side aortic arch. Four main surgical techniques were applied to those patients: 1. total arch replacement with frozen elephant trunk implantation (n = 2); 2. hybrid procedure combining open arch repair and endovascular intervention (n = 1); 3. total endovascular repair using thoracic endovascular aortic repair (TEVAR) with or without left subclavian artery (LSCA) revascularization (n = 6); 4. direct repair underwent endoaneurysmorrhaphy. Clinical characteristics and outcomes were collected. RESULTS: The mean age of these 10 patients was 56.5 years (range 29-79 years) and only 1 woman. The pathology includes aortic dissection (n = 6) and aneurysm (n = 4). The mean diverticulum size was 41.4 [24.2-56.8] mm. There were no in-hospital deaths, and the median hospital stay was 22 [15-43] days. During the follow-up period (21.4 months, 1-44 months), one died of an unknown cause and one died of esophageal fistula. Two patients underwent second-stage endovascular intervention for distal lesion. And none of the patients had endoleak during the follow-up period. CONCLUSIONS: Each of the procedures we have mentioned here has its advantages and disadvantages; individualized treatment should meet the appropriate indications. A single-branched stent graft is feasible and effective in the treatment of aortic disease combined with Kommerell's diverticulum.

The aortic uncrossing procedure.

OBJECTIVE: Cross-sectional imaging allows identification of rare patients with a vascular ring and circumflex aorta. The key diagnostic feature is crossing of the transverse aortic arch from right to left posterior to the trachea and superior to the carina in a patient with a right aortic arch. We evaluated our patients who received an aortic uncrossing procedure. METHODS: We reviewed all patients who underwent aortic uncrossing from 2002 to 2022. All patients received preoperative computed tomography imaging and bronchoscopy. RESULTS: Eleven patients ranging in age from 1.5 to 10 years (median 4 years) underwent aortic uncrossing. Two patients had prior left ligamentum division, and 3 patients had prior left aortic arch division. All had significant clinical symptoms. Eight patients had deep hypothermic circulatory arrest (mean 34 minutes), and 3 patients had antegrade cerebral perfusion (median, 28 minutes). Patch material was not used for aortic augmentation, and no patient underwent a posterior tracheopexy or rotational esophagoplasty. Postoperative length of stay ranged from 4 to 31 days (median, 5 days). One patient required a temporary tracheostomy for bilateral recurrent laryngeal nerve paresis, which recovered. One patient required an aortic extension graft to alleviate esophageal compression from an unusual ectatic esophageal course. All patients had relief of airway symptoms and dysphagia. CONCLUSIONS: In properly selected patients with a right aortic arch and circumflex aorta, aortic uncrossing is a safe and effective therapy to treat airway and esophageal compression. The procedure can be conducted with deep hypothermic circulatory arrest or antegrade cerebral perfusion. Careful attention to the location of the esophagus and recurrent laryngeal nerves is required.

Management of esophageal cancer in patients with a right aortic arch or double aortic arch: a case series of 34 cases.

BACKGROUND: Few cases describing patients with a right aortic arch (RAA) or double aortic arch (DAA) and esophageal cancer (EC) have been reported. METHODS: We analyzed RAA and DAA cases treated with esophagectomy in our center's database and reported in English-language studies until April 1, 2023. Our study assessed the malformation characteristics and surgical details of EC patients with RAA and DAA. RESULTS: We extracted data of 24 EC patients with RAAs and 10 EC patients with DAAs. In both groups, the patients were more likely to be Japanese and male, to have squamous cell carcinoma and to have tumors located in the upper thoracic esophagus or middle thoracic esophagus. Left thoracotomy was commonly applied for RAA patients. For DAA patients, the proportions of left-sided and right-sided approaches were similar. Esophagectomy under video-assisted thoracoscopic surgery (VATS) in RAA or DAA patients had been performed on a routine basis in recent years. There were two anastomotic leakages in each group. Specifically, Kommerell diverticulum rupture occurred in 1 RAA patient; gastric tube dilation occurred in 1 DAA patient; and recurrent laryngeal nerve (RLN) injury occurred in 2 RAA patients. The postoperative course was uneventful for most patients in both cohorts. CONCLUSIONS: Esophageal carcinoma is rarely seen in patients with an RAA or DAA. To adequately dissect superior mediastinal LNs, an auxiliary incision (such as sternotomy), the left door open method or a preceding cervical procedure should be used appropriately. Esophagectomy, whether via thoracotomy or thoracoscopic surgery, can be performed safely for both RAA and DAA.

Utility of the Angle between the Right Aortic Arch and First Branch for Detecting Double Aortic Arch via Fetal Echocardiography.

INTRODUCTION: The presence of a double aortic arch (DAA) is manifested by compressive symptoms, requiring surgery. DAA cases are classified as either complete or incomplete type. DAA and a right aortic arch with mirror image branching (mRAA) have a similar configuration to the first branch artery. The first branch of the mRAA is the left brachiocephalic artery, which appears to be the same as that of an incomplete DAA due to blood flow interruption. The present retrospective study aimed to evaluate the differences between DAA and mRAA by fetal echocardiography. METHODS: This single retrospective cohort study included all patients diagnosed with complete DAA, incomplete DAA, or mRAA at our facility between 2010 and 2022. The patients were diagnosed with complete DAA, incomplete DAA, or mRAA after birth and remaining fetal echocardiograms. The patients were divided into the DAA (complete DAA: n = 4, incomplete DAA: n = 3) and mRAA (n = 4) groups. The following three outcomes were compared: (1) angle between the right aortic arch and first branch (RF angle), (2) ratio of height to width of the region bounded by the aortic arch, first branch of the aortic arch, and descending aorta, and (3) maximum tracheal diameter on a three-vessel trachea view. RESULTS: The incomplete DAA cases were difficult to diagnose via fetal echocardiography. On fetal echocardiography, the RF angle was significantly steeper in the DAA group than in the mRAA group (median 57° [36°-69°] vs. 75° [62°-94°]; p < 0.05). The DAA and RAA groups showed no significant differences in the ratio of height to width of the region bounded by the aortic arch, first branch of the aortic arch, and descending aorta (median 0.57 [0.17-0.68] vs. 0.73 [0.56-1.0]) and maximum tracheal diameter (median 2.5 [1.4-3.3] vs. 3.2 [2.8-3.5] mm). The cut-off value for the presence of DAA was an RF angle <71°. CONCLUSION: The DAA group (complete and incomplete DAA) had a significantly steeper RF angle than the mRAA group. Therefore, RF angle measurement could improve the fetal diagnosis and postnatal prognosis of DAA.

Persistence of aerodigestive symptoms after vascular ring repair.

OBJECTIVE: Vascular rings are often associated with respiratory and swallowing difficulties due to tracheal or esophageal compression. While the results of a vascular ring repair are considered excellent, the long-term effect of tracheal and esophageal remodeling and the persistence of symptoms have scarcely been reported. Our study aims to evaluate the respiratory and swallowing outcomes of vascular ring repair. STUDY DESIGN AND METHODS: A retrospective cohort study of children who underwent vascular ring repair between 2010 and 2022 in a tertiary-care children's hospital. RESULTS: There were 108 patients enrolled: sixty-three patients (57.41 %) with a right aortic arch, 42 patients (38.89 %) with a double aortic arch, and 3 patients (2.78 %) with other vascular rings. Forty-three (39.81 %) patients were diagnosed prenatally. Of the 65 patients (60.19 %) diagnosed postnatally, 35/65 (53.85 %) had either respiratory or swallowing symptoms as the indication for diagnostic workup. Persistent respiratory and swallowing symptoms were noted in 34/108 (31.48 %) and 30/108 (27.78 %) patients, respectively, within a year of surgical repair. Fourteen patients underwent repeated laryngoscopy and bronchoscopy that demonstrated residual tracheomalacia; however, only 2/14 (1.9 %) patients required tracheostomy tube placement, and 6-out-of-7 patients were weaned off positive pressure airway support. Persistent respiratory symptoms were significantly more common in patients with a double aortic arch compared to a right aortic arch. No differences were noted in demographics, comorbidities, and preoperative aerodigestive symptoms between patients with residual symptoms and patients with no residual symptoms. CONCLUSIONS: Persistent respiratory and swallowing symptoms after vascular ring repair are not uncommon. Postoperative evaluation should be pursued by a dedicated team, and treatment considered as appropriate.

Case Report: Kommerell's diverticulum and left aberrant subclavian artery stenosis hybrid treatment with branched aortic stent-graft.

Kommerell's diverticulum in association with left or right aberrant subclavian arteries is a rare finding and is challenging to treat. Contemporary surgical and endovascular techniques provide a broad arsenal of possible treatments. Imaging techniques and modeling technology allow a more personalized strategy for each patient. In this case, we present a symptomatic patient with a Kommerell's diverticulum and a left aberrant subclavian artery complicated by proximal stenosis and poststenotic aneurysm. A hybrid technique using a single-branched thoracic stent-graft (Castor, MicroPort Medical, Shanghai, China) in combination with a surgical left subclavian-carotid bypass and endovascular occlusion of the poststenotic aneurysm using a vascular plug device (Amplatzer Vascular Plug, Abbott, Chicago, United States) was performed. This approach was planned and facilitated by the use of a 3D model. Alternative treatment options and the strengths of this approach are briefly reviewed and discussed.