Aortic Arch Type Research Articles

DiGeorge syndrome and anomalous right aortic arch with arch-on-arch and figure-of-eight configurations: Aortic sac maldevelopment and left brachiocephalic artery abnormal remodeling.

We report a 6-month-old female infant with deletion of chromosome 22q11.2 (DiGeorge/VFS TUPLE 1), normal atrial arrangement with concordant atrioventricular connection, pulmonary atresia, large subaortic ventricular septal defect, diminutive native pulmonary arteries, a characteristic weird-shape right aortic arch with arch-on-arch appearance and figure of 8 configuration. We presented the cardiac computed tomographic angiographic and cardiac angiographic features. Using Autodesk 3ds Max 2018 software, we explained and illustrated the speculative embryologic etiology of this bizarre aortic archanomaly with the extensive abnormal remodeling of the left brachiocephalic artery, based on a “five-embryonic aortic arches” concept. As to the best of the authors' knowledge, this is the first report of a genetically confirmed case of DiGeorge syndrome and an exceedingly rare type of right aortic arch anomaly with embryologic explanation according to the “five-embryonic-aortic-arches” concept. It seems that the constellation of pulmonary atresia, bizarreshaped right aortic arch due to abnormal development of the aortic sac, and abnormal remodeling of the left brachiocephalic artery may be strongly suggestive of DiGeorge syndrome.

Anatomic classification of the right aortic arch.

The term right aortic arch is used for an aorta that arches over the right bronchus. Right aortic arch was classified into two types by Felson et al, based on branching patterns, with a proposed embryological explanation, and into three types by Shuford et al. Other anatomical variants of right aortic arch were described later, including isolated left brachiocephalic artery and aberrant left brachiocephalic artery. We have classified right aortic arch anatomy into 10 variants, supported by radiological evidence, and with reference to possible embryology. This classification will help in understanding the morphological basis for the formation of different types of right aortic arch and the course of the recurrent laryngeal nerve in such cases.

Aortic arch types and postoperative outcomes after carotid artery stenting in asymptomatic and symptomatic patients.

The aim of this study was to investigate the influence of the aortic arch type on technical and clinical success of carotid artery stenting (CAS) procedure. Clinical and anatomical data of consecutive patients who underwent CAS from 2010 to 2018 were prospectively collected and retrospectively analyzed. Primary outcome was technical success, define as successful stent delivery and deployment and <30% residual carotid stenosis. Secondary outcomes were death, stroke, myocardial infarction (MI) and transient ischemic attack (TIA) rates at 30 days after CAS. Subgroups analysis with asymptomatic and symptomatic patients were also performed. During the study period, 523 patients were enrolled and analyzed. Among these, 176 (33.6%) had Type I, 227 (43.4%) had Type II and 120 (23.0%) had Type III or bovine aortic arch (BAA) type. Technical success rate was achieved in 96.0% of cases. At 30 days, if compared with Type I or II, patient with Type III or BAA experienced a higher death rate (0 vs. 0 vs. 1.8%, respectively; P=0.056) and combined postoperative stroke/TIA rate (3% vs. 2.8% vs. 9.9%, respectively; P=0.012). No differences for same outcomes between asymptomatic and symptomatic patients were described, although the latter group experienced more postoperative MI. A multivariate analysis revealed Type III or BAA as an independent risk factor for postoperative stroke/TIA (HR 3.23, IC95% 1.40-7.45; P=0.006). In this cohort of patients, death and postoperative neurological complications rates were associated with Type III or BAA, irrespective of symptomatic patients' status. Extremely attention is required during perioperative period in patients who were candidate to CAS and with challenging aortic arch anatomy.

Application of pigtail catheter tailing combined with long-wire swapping technique in cerebral angiography via the right radial artery.

The safety and feasibility of transradial approach for cerebral angiography has been confirmed previously. However, this approach has been limited used due to the difficulty during the procedure. This study aimed to introduce a pigtail catheter tailing and long-wire swapping technique to improve the success rate without increasing complications.From August 2015 to December 2018, 560 patients who underwent cerebral angiography via transradial approach were recruited. The data including the type of aortic arch, type of Simmon catheter were collected.The loop was successfully constructed in 553 patients using Simmon-2 or Simmon-1 catheter. Of these patients, 72 patients successfully underwent cerebral and renal angiography, while 481 patients underwent cerebral angiography. The time for angiography was 52.87 ± 11.23 minutes and 47.8 ± 11.8 minutes, respectively. There were 369 (66.7%), 135 (24.4%), and 49 (8.9%) patients with type I, type II, and type III aortic arches, respectively, and their success rates of looping using Simmon-2 catheter were 97.8%, 97.0%, and 89.8%, respectively. The success rates of angiography in the right internal carotid artery, right vertebral artery, left internal carotid artery, and left vertebral artery were 100%, 100%, 98.9%, and 98.9%, respectively. No serious complications were observed in all patients.The pigtail catheter tailing and long guidewire swapping is considered as a safe procedure with high success rate for loop construction using a Simmon-2 catheter through the right radial artery, subsequently improving the success rate as well as the efficiency of angiography.

Alternative Access in Congenital Heart Disease

Limited arterial vascular access precluded necessary transcatheter intervention in a 22-year-old woman with repaired interrupted aortic arch type B. Alternative transcaval vascular access enabled percutaneous therapy. This practice evolution is likely to benefit the growing numbers of adults with congenital heart disease. (Level of Difficulty: Advanced.)

A new borne with very complex aortic anatomy: diagnosis and treatment challenge\u2014case report

BackgroundInterrupted aortic arch (IAA) is a congenital malformation of the aortic arch which involves 3 out of 1 million live births. This congenital anomaly rarely occurs as an isolated lesion and is often associated with other intracardiac malformations, most commonly ventricular septal defect and patent ductus arteriosus (PDA). The diagnosis and surgical treatment of aortic interruption is usually challenging and may require multiple operations throughout the patient’s life.Case presentationThis case represents a neonate with interrupted aortic arch (type B) and a very long segment of descending aorta hypoplasia and complex anatomy. The patient escaped early diagnosis at birth and presented few days later by a picture that mimicked severe sepsis and shock.His aortic anatomy was very complex and he was treated with long extra-anatomical aortic interposition graft.ConclusionAortic interruption is a rare congenital anomaly and is considered an extreme form of aortic coarctation.It sometimes escapes early diagnosis due to the presence of patent ductus arteriosus and present later with shock and lactic acidosis. Sometimes the aortic anatomy is very complex and requires unusual surgical techniques for its repair.

Vessel diameter and catheter-to-vessel ratio affect the success rate of clot aspiration

BackgroundA direct aspiration first pass technique (ADAPT) is an efficient, safe, cost-effective, and fast thrombectomy technique.ObjectiveTo evaluate anatomical and clot characteristics associated with success of the aspiration component as part...

The Relation Between Aortic Arch Branching Types and the Laterality of Cardio-Embolic Stroke

Background and purposeThe trajectory of cardiogenic emboli could be affected by anatomical and flow characteristics of the aortic arch. We aimed to study the relation between the different aortic arch patterns and the laterality of cardiogenic emboli. Methods192 cardioembolic strokes were classified into 3 groups according to the type of the aortic arch; type 1 (n = 69), type 2 (n = 49), type 3 (n = 74). The side and site of the cerebral vessels occlusion were divided into anterior and posterior circulation strokes, and anterior strokes were further subdivided into right or left internal carotid, middle or anterior cerebral arteries occlusion. ResultsOverall, the anterior circulation embolic occlusions were more common than the posterior, and middle cerebral artery more commonly affected than internal carotid artery. The left side propensity was higher either in the total patients’ pool or after segregation into atrial fibrillation (AF) and non AF cardio-embolic cases in all types of aortic arch except for type 1 aortic arch in the non AF cases. This propensity tended to get higher with advancement of the aortic arch types but failed to show statistically significant difference between the 3 arch types, however combination of type 2 and 3 arches into a single group showed statistically significant rise in the left side propensity in the total cardioembolic cases (P = 0.039) and in the non AF cardioembolic cases (P = 0.029). The bovine arch also showed increased left side propensity. ConclusionCardioemboli tends to have left anterior cerebrovascular predilection especially with AF. Different geometrical patterns of aortic arch branching seem to affect the laterality of cardioemboli and increase its left side predilection.

Thoracoscopic esophagectomy with left recurrent laryngeal nerve monitoring for thoracic esophageal cancer in a patient with a right aortic arch: a case report

BackgroundSurgery for cases of thoracic esophageal cancer with a right aortic arch is rare, and the anatomic abnormalities in such patients necessitate a different surgical approach. Since the position of the recurrent laryngeal nerve often differs from the usual in these cases, the lymph node dissection around the recurrent laryngeal nerve, which is an important step in surgery for thoracic esophageal cancer, requires careful attention. There are some reports on the usefulness of intraoperative recurrent laryngeal nerve monitoring during esophageal cancer surgery. Herein, we report a case of successful thoracoscopic esophagectomy for esophageal cancer in a patient with a right aortic arch using intraoperative recurrent laryngeal nerve monitoring.Case presentationA 70-year-old man was diagnosed as having esophageal cancer (Ut, type 0-IIc, T1b/MtLt, type 0-IIc, T1b, N2, M0, cStage II) and was treated by neoadjuvant chemoradiotherapy followed by radical surgery. Preoperative CT examination revealed a right aortic arch, and based on the findings of 3D-CT, we classified the right aortic arch as type IIIB1 (Edwards classification), which is the most frequent type of right aortic arch. We performed thoracoscopic esophagectomy via a left thoracic approach with the patient placed in the prone position, cervical esophagogastric conduit reconstruction via the retrosternal route, and three-field lymph node dissection. Although Kommerell’s diverticulum could be easily confirmed, the descending aorta took a meandering course, making it difficult for the esophagus to be mobilized and detached and therefore also to identify the ductus arteriosus and left recurrent laryngeal nerve. Intraoperative recurrent laryngeal nerve monitoring using NIM-RESPONSE® 3.0 (Medtronic Japan, Tokyo, Japan) allowed the position of the left recurrent laryngeal nerve to be accurately determined, and upper mediastinal lymph node dissection and mobilization of the upper thoracic esophagus were performed safely. Postoperatively, the patient showed no evidence of recurrent laryngeal nerve palsy, but needed conservative treatment for anastomotic leakage. The patient was discharged 46 days after the surgery.ConclusionIt was suggested that intraoperative recurrent laryngeal nerve monitoring is useful in esophageal cancer with a right aortic arch undergoing surgery, in whom anatomic abnormalities of the recurrent laryngeal nerve can be expected.

Transradial Approach as the Primary Vascular Access with a 6-Fr Simmons Guiding Sheath for Anterior Circulation Interventions: A Single-Center Case Series of 130 Consecutive Patients

In coronary intervention, the transradial approach (TRA) is increasingly used as the primary vascular access because of its numerous advantages over the transfemoral approach. However, in neurointerventions, conventional TRA with a straight-shaped guiding system is used as an alternative vascular access because transradial carotid cannulation can be technically challenging for right common carotid artery (CCA) lesions with steep angulation to the right subclavian artery or left CCA lesions with a nonbovine origin. The purpose of the present study was to evaluate the feasibility and safety of TRA as the primary vascular access with a pre-shaped Simmons guiding sheath for anterior circulation interventions. Between June 2018 and September 2019, 130 consecutive patients (75 carotid artery stenting and 55 cerebral aneurysm coiling cases) who underwent TRA as the primary vascular access were included in this study. A 6-Fr Simmons guiding sheath was introduced into the target CCA by selecting a cannulation technique based on preprocedural image assessment. We retrospectively analyzed the carotid cannulation success, procedural success, and periprocedural or vascular access site complications. Carotid cannulation (69 right CCA, 6 left CCA with a bovine origin, and 55 left CCA with a nonbovine origin) and the subsequent procedure were successfully performed for all 130 patients without periprocedural or vascular access site complications. TRA with a 6-Fr Simmons guiding sheath for anterior circulation interventions is highly successful and safe for all target CCAs and aortic arch types. This method can be utilized as the primary vascular access for anterior circulation interventions.

22q11.2 deletion syndrome and congenital heart disease.

The 22q11.2 deletion syndrome has an estimated prevalence of 1 in 4-6,000 livebirths. The phenotype varies widely; the most common features include: facial dysmorphia, hypocalcemia, palate and speech disorders, feeding and gastrointestinal disorders, immunodeficiency, recurrent infections, neurodevelopmental and psychiatric disorders, and congenital heart disease. Approximately 60-80% of patients have a cardiac malformation most commonly including a subset of conotruncal defects (tetralogy of Fallot, truncus arteriosus, interrupted aortic arch type B), conoventricular and/or atrial septal defects, and aortic arch anomalies. Cardiac patients with a 22q11.2 deletion do not generally experience higher mortality upon surgical intervention but suffer more peri-operative complications than their non-syndromic counterparts. New guidelines suggest screening for a 22q11.2 deletion in the patient with tetralogy of Fallot, truncus arteriosus, interrupted aortic arch type B, conoventricular septal defects as well as those with an isolated aortic arch anomaly. Early identification of a 22q11.2 deletion in the neonate or infant when other syndromic features may not be apparent allows for timely parental screening for reproductive counseling and anticipatory evaluation of cardiac and noncardiac features. Screening the at-risk child or adult allows for important age-specific clinical, neurodevelopmental, psychiatric, and reproductive issues to be addressed.

Adult interrupted aortic arch with aortic regurgitation: Single-stage repair

Adult patients with interruption of the aorta are an extremely rare occurrence. It is commonly associated with additional cardiovascular anomalies. Surgery is the mainstay in the management of the patients. Interrupted aortic arch and associated cardiac disease is a complex situation that requires correction either simultaneously or in stages. We describe the case of a 32-year-old male patient planned for aortic valve replacement at another center, incidentally diagnosed with interrupted aortic arch Type A. He was managed with single-stage aortic valve replacement and extra-anatomical bypass.

Risk factors of distal segment aortic enlargement after complicated type B aortic dissection

ObjectivesDistal segment aortic enlargement (DSAE) is a common complication that influences the long-term prognosis of type B aortic dissection (TBAD) after thoracic endovascular aortic repair (TEVAR). In this study, a multivariate analysis was performed to find potential factors predictive of DSAE. MethodsA single-center retrospective study was performed from 1999 to 2016. Included in the study were complicated TBAD patients who underwent TEVAR with uncovered residual tears. Based on the diameter of the distal segment of the uncovered aorta, we assigned patients to an enlargement group and a non-enlargement group. Data extracted from the medical records included demographic and clinical characteristics and follow-up computed tomography angiography data. The primary endpoints were the all-cause mortality and the presumably aortic-related events that required reintervention during the follow-up period. ResultsFor the 333 patients, all-cause mortality was 38 (11.41%), and 76 (22.82%) patients underwent reintervention. A total of 70 (21.02%) patients experienced DSAE, among them were 2 patients who died of aortic rupture and 58 patients who accepted reintervention. Multivariate analysis reviewed independent risk factors of postoperative DSAE, including current smoking, the residual length of the patent false lumen, the postoperative number of dissection tears in the thoracic aorta and type III aortic arch; as well as protective factors, including the application of a restrictive bare stent (RBS), the length of covered stent in the descending thoracic aorta, and the distance from the residual first tear to the left subclavian artery (LSA). ConclusionDSAE after TEVAR for patients with a complicated TBAD can be influenced by their current smoking habit, the residual length of patent false lumen, the postoperative number of dissection tears in the thoracic aorta and the aortic arch type. Meanwhile, RBS usage, the length of the covered stent in the descending thoracic aorta and the distance from the residual first tear to the LSA could have positive effect on the prognosis.

Common carotid artery puncture in anterior circulation thrombectomy in patients with unfavorable vascular anatomy: A case-control study.

The objective of this study was to compare clinical outcomes in patients who with unfavorable vascular anatomy underwent mechanical thrombectomy (MT) by common carotid artery access versus transfemoral approach.A retrospective review was performed in our hospital database to identify patients with challenging vascular anatomy who underwent MT for anterior circulation large vessel occlusion (LVO) between August 2015 and November 2018. Transcarotid and transfemoral cohorts were compared. Patient characteristics, procedural techniques, clinical outcomes were recorded.A total of 52 patients were included, 16 (31%) underwent MT via transcarotid access. There were no significant differences in patient characteristics, intravenously recombinant tissue plasminogen activator therapy, clot location, or carotid tortuosity and presence of aortic arch type. There were significant differences in clinical outcomes between the 2 cohorts, including mean access-to-reperfusion time (84 vs 44 minutes; P = .000), poor clinical outcome (modified Rankin scale >2) at 90 days follow-up (37.5% vs 63.9%; P = .034). But there were no significant differences in successful revascularization rates (thrombolysis in cerebral infarction score ≥2b 87.5% vs 80.6%; P = .541), post-thrombectomy symptomatic intracranial hemorrhage (12.5% vs 13.9%; P = .892), and mortality (12.5% vs 22.2%; P = .412) were similar between transcarotid and transfemoral cohorts.Our results demonstrate that transcarotid access for MT of anterior circulation LVO in patients with unfavorable vascular anatomy may be considerable. Transcarotid access may be better than transfemoral access in well-selected unfavorable vascular anatomy patients undergoing MT.

Interrupted Aortic Arch in a Patient with Patient-Prosthesis Mismatch after Aortic Valve Replacement.

This case presents a patient who underwent aortic valve replacement and presented 13 years later with high gradients across the prosthesis, mitral insufficiency, and severe systemic hypertension. Her preoperative workup led to the diagnosis of an interrupted aortic arch Type A. Her surgical management included an initial procedure to repair the interruption, and 11 months later after resolution of her hypertension, a second surgery, which included the Ross procedure and mitral valve repair.

EP09.17: Prenatal diagnosis of interrupted aortic arch type B by two‐dimensional echocardiography and colour Doppler

EP09.17: Prenatal diagnosis of interrupted aortic arch type B by two‐dimensional echocardiography and colour Doppler

Tbx1 and Foxi3 genetically interact in the pharyngeal pouch endoderm in a mouse model for 22q11.2 deletion syndrome.

We investigated whether Tbx1, the gene for 22q11.2 deletion syndrome (22q11.2DS) and Foxi3, both required for segmentation of the pharyngeal apparatus (PA) to individual arches, genetically interact. We found that all Tbx1+/-;Foxi3+/- double heterozygous mouse embryos had thymus and parathyroid gland defects, similar to those in 22q11.2DS patients. We then examined Tbx1 and Foxi3 heterozygous, null as well as conditional Tbx1Cre and Sox172A-iCre/+ null mutant embryos. While Tbx1Cre/+;Foxi3f/f embryos had absent thymus and parathyroid glands, Foxi3-/- and Sox172A-iCre/+;Foxi3f/f endoderm conditional mutant embryos had in addition, interrupted aortic arch type B and retroesophageal origin of the right subclavian artery, which are all features of 22q11.2DS. Tbx1Cre/+;Foxi3f/f embryos had failed invagination of the third pharyngeal pouch with greatly reduced Gcm2 and Foxn1 expression, thereby explaining the absence of thymus and parathyroid glands. Immunofluorescence on tissue sections with E-cadherin and ZO-1 antibodies in wildtype mouse embryos at E8.5-E10.5, revealed that multilayers of epithelial cells form where cells are invaginating as a normal process. We noted that excessive multilayers formed in Foxi3-/-, Sox172A-iCre/+;Foxi3f/f as well as Tbx1 null mutant embryos where invagination should have occurred. Several genes expressed in the PA epithelia were downregulated in both Tbx1 and Foxi3 null mutant embryos including Notch pathway genes Jag1, Hes1, and Hey1, suggesting that they may, along with other genes, act downstream to explain the observed genetic interaction. We found Alcam and Fibronectin extracellular matrix proteins were reduced in expression in Foxi3 null but not Tbx1 null embryos, suggesting that some, but not all of the downstream mechanisms are shared.

Prevalence of type III arch configuration in patients with type B aortic dissection.

Type III aortic arch configuration consistently presents anatomical and biomechanical characteristics which have been associated with an increased risk of type B aortic dissection (TBD). Our aim was to investigate the prevalence of type III arch in patients with TBD and type B intramural haematoma (IMH-B). A multicentre retrospective analysis was performed on patients with TBD and IMH-B observed between 2002 and 2017. The computed tomographic images were reviewed to identify the type of aortic arch. Exclusion criteria included previous arch surgery, presence of aortic dissection or aneurysm proximal to the left subclavian artery and bovine arches. An ad hoc systematic literature review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines to assess the prevalence of type III arch in non-TBD and non-aneurysmal patients. Two hundred and sixty-one patients with TBD/IMH-B were found to be suitable for the study and were stratified according to aortic arch classification. The ad hoc literature search provided 10 relevant articles, from which a total of 7983 control cases were retrieved. TBD/IMH-B patients were significantly younger than controls [64.3, standard error: 0.74 (62.84-65.76) vs mean pooled age 70.5, standard error: 0.40 (69.71-71.28)]. Patients with TBD/IMH-B presented with a significantly higher prevalence of type III arch [41.0% (107/261) (35.2-47.1)] than controls [16% (1241/7983) (10-22)]. Our data indicate an association between type III arch configuration and the occurrence of TBD/IMH-B. These findings warrant further studies to disclose the potential role of type III arch configuration as an anatomical risk factor for TBD/IMH-B.

Patient-specific three-dimensional printed heart models benefit preoperative planning for complex congenital heart disease.

Preoperative planning for children with congenital heart diseases remains crucial and challenging. This study aimed to investigate the roles of three-dimensional printed patient-specific heart models in the presurgical planning for complex congenital heart disease. From May 2017 to January 2018, 15 children diagnosed with complex congenital heart disease were included in this study. Heart models were printed based on computed tomography (CT) imaging reconstruction by a 3D printer with photosensitive resin using the stereolithography apparatus technology. Surgery options were first evaluated by a sophisticated cardiac surgery group using CT images only, and then surgical plans were also set up based on heart models. Fifteen 3D printed heart models were successfully generated. According to the decisions based on CT, 13 cases were consistent with real options, while the other 2 were not. According to 3D printed heart models, all the 15 cases were consistent with real options. Unfortunately, one child diagnosed with complete transposition of great arteries combined with interruption of aortic arch (type A) died 5days after operation due to postoperative low cardiac output syndrome. The cardiologists, especially the younger ones, considered that these 3D printed heart models with tangible, physical and comprehensive illustrations were beneficial for preoperative planning of complex congenital heart diseases. 3D printed heart models are beneficial and promising in preoperative planning for complex congenital heart diseases, and are able to help conform or even improve the surgery options.

Abstract WP35: A Comparison of Transradial versus Transfemoral Access in Anterior Circulation Mechanical Thrombectomy

Background: The transradial approach (TRA) to endovascular procedures decreases access site morbidity and mortality when compared to the traditional transfemoral technique (TFA). Despite its improved safety profile, there remains concerns that performing mechanical thrombectomy (MT) via TRA may lead to longer revascularization times and thus worse outcomes. However, TRA may confer an added benefit in mechanical thrombectomy (MT) where navigation of challenging aortic arch and carotid anatomy is often facilitated by a right radial artery trajectory. In this study, we compare technical and clinical outcomes in patients who underwent MT via TRA versus TFA. Methods: We performed a retrospective review of our institutional database to identify 51 patients with challenging vascular anatomy who underwent MT for anterior circulation large vessel occlusion between February 2015 and February 2017. Patient characteristics, procedural techniques, and outcomes were recorded and TFA and TRA cohorts were compared. Results: Of the 51 patients, 35% of patients (n=18) underwent MT via TRA. There were no significant differences between the cohorts in patient characteristics, clot location, or aortic arch type and presence of ICA tortuosity. There were no significant differences in technical or clinical outcomes between the 2 cohorts, including rates of single-pass recanalization (54.6% vs. 55.6%, p=0.949) and average number of passes (1.9 vs. 1.7, p=0.453). Mean time from vascular access to reperfusion (61.9 vs. 61.1 mins, p=0.920), rates of successful revascularization (&gt;= TICI2b; 87.9% vs. 88.9%, p=1.0) and functional outcomes (mRS &gt;=2, 39.4% vs. 33.3%, p=0.669) were also similar between the TFA and TRA cohorts, respectively. Conclusions: Our results demonstrate equivalence in efficacy and efficiency between transradial and transfemoral access for MT in acute large vessel occlusion in the anterior circulation in patients with challenging vascular anatomy. Given the established advantage in access-site morbidity, our data suggests that the transradial approach may be superior to the transfemoral approach in well-selected patients undergoing MT.