Clinical Presentation and Management of the Cervical Aortic Arch in the Adult Population: A Review of Case Reports.

The cervical aortic arch (CAA) is an uncommon congenital anomaly in aortic development, characterized by an elongated aortic arch extending at or above the medial ends of the clavicles. Our objective was to examine the clinical and surgical characteristics of this infrequent condition in the adult population. PubMed, ScienceDirect, SciELO, DOAJ, and Cochrane Library databases were searched until December 2023 for case reports describing the presence of a cervical aortic arch in patients aged ≥18 years. Case reports and series were included if the following criteria were met: (1) description of the cervical aortic arch, (2) age ≥18 years, and (3) English language. The literature search identified 2,325 potentially eligible articles, 61 of whom met our inclusion criteria and included a combined number of 71 patients. Mean age was 38.6 ± 15.4 years, with a female prevalence of 67.1% (47/70). Two-thirds of the CAA were left-sided (48/71, 67.6%), and 62.0% (44/71) of patients presented a concomitant arch aneurysm. Asymptomatic patients were 45.7% (32/70), while of those that were symptomatic, 60.5% (23/38) had symptoms related to vascular-induced compression of trachea and esophagus. Surgery was performed in 42 patients (62.7%) among 67 cases that reported the patient's treatment, and 5 patients (11.9%) among those surgically treated underwent the procedure through an endovascular approach. CAA is an uncommon congenital abnormality that presents challenges in diagnosis and treatment due to its high anatomical variability, diverse clinical manifestations, and presence of concomitant diseases. Surgery seems to be a safe and effective option for the resolution of symptoms.

Case report: A novel combination of anomalies in a patient with 22q11.2 deletion syndrome.

A frequently occurring genetic disorder, 22q11.2 deletion syndrome can manifest with various abnormalities. The range of cardiac anomalies associated with this syndrome is extensive, with conotruncal defects being the most prevalent. In this study, we report the case of a patient with a unique combination of anatomical abnormalities such as crisscross pulmonary arteries, a cervical aortic arch with coarctation of the aorta, and a ventricular septal defect. The patient underwent initial surgical intervention, which resulted in significant clinical improvement.

Cervical aortic arch in the pediatric population: a meta-analysis of individual patient's data.

Background: This is the first meta-analysis to analyze all reports of published pediatric cases of cervical aortic arch (CAA) by highlighting the clinical characteristics and treatment outcomes using the reported individual data of the patients. The aim of the study is to investigate the clinical features and surgical outcomes of such a rare disease in the pediatric population. Methods: A comprehensive search was conducted in various academic databases, including PubMed, ScienceDirect, SciELO, DOAJ, and Cochrane Library, until June 2022 for case reports describing the presence of cervical aortic arch in the pediatric age. Case reports and series were included if the following criteria were met: (1) description of the cervical aortic arch; (2) patient of pediatric age; and (3) published in the English language. All other types of publications that lacked patient-specific information were excluded from the analysis. This systematic review was conducted in accordance with the PRISMA guidelines. The primary outcome measure of the analysis was early and late mortality. Results: The literature search identified 2,272 potentially eligible articles, 72 of which met our inclusion criteria with 96 patients including the author's institutional case. At a median of 365 (90-730) days, the overall cohort registered a 7.3% (7/96) mortality rate. In the subset of patients who underwent surgery, the mortality rate was also 7.3% (4/55), and the mortality rate following surgery to treat only CAA was 2.4% (1/42). Dyspnea was identified as an independent determinant of mortality by employing the univariable Firth bias-reduced logistic regression method. Conclusion: Cervical aortic arch is a rare congenital heart disease that poses treatment challenges due to the high anatomical variability, diverse clinical presentations, and the presence of other concomitant diseases. The surgical treatment appears to be a safe and effective approach for resolving the symptoms, although it needs to be tailored individually for each patient. Systematic Review Registration: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=346826, Identifier: CRD42022346826.

Surgical repair of interrupted right-sided cervical aortic arch with hypoplasia of the descending thoracic aorta in a child with PHACE syndrome.

Obstruction of a right cervical aortic arch in association with hypoplasia of the descending aorta is a rare congenital cardiac malformation. We report the case of a 6-month-old boy with posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies and eye anomalies (PHACE) syndrome and interruption of a right-sided cervical aortic arch. The descending thoracic aorta in the child had a long hypoplastic segment and the patient also had small ventricular septal defect and pulmonary valve stenosis. The surgical technique of reconstruction of the aortic arch and the descending thoracic aorta through a median sternotomy is described.

Right cervical aortic arch associated with anomaly of epiaortic vessels and haemodynamically significant obstruction between ascending and descending aorta. Extra-anatomic bypass graft between ascending and descending aorta and long-term follow-up.

Right cervical aortic arch is a rare malformation in which a right-sided aortic arch is unusually long, forms a loop, descends, and crosses behind the oesophagus to continue as a left descending aorta. We present a case of right cervical aortic arch associated with anomaly of epiaortic vessels and aortic obstruction, surgically treated with extra-anatomic bypass graft between ascending and descending aorta.

Congenital Anomalies of Aortic Arch: A Pictorial Essay.

Aortic abnormalities account for 15 to 20% of all congenital cardiovascular diseases. The purpose of this pictorial essay is to illustrate various congenital anomalies of the aortic arch, which influence patient management and outcome.

Cervical aortic arch with aneurysm formation and an anomalous right subclavian artery and left vertebral artery: A case report.

BACKGROUND: A cervical aortic arch (CAA) refers to a high-riding aortic arch (AA) that often extends above the level of the clavicle. This condition is very rare, with an incidence of less than 1/10000. CASE SUMMARY: A 29-year-old woman was admitted to the otolaryngology department of our hospital for repeated bilateral purulent nasal discharge for the prior 3 mo. The patient was diagnosed with chronic sinusitis and chronic rhinitis at admission. A preoperative noncontrast chest computed tomography scan showed a high-riding, tortuous AA extending to the mid-upper level of the first thoracic vertebra with local cystic dilatation. A further computed tomography angiography examination showed that the brachiocephalic trunk, left common carotid artery, left vertebral artery (LVA) (slender), and left subclavian artery sequentially branched off of the aorta from the proximal end to the distal end of the AA. The proximal end of the right subclavian artery (RSCA) was tortuous and dilated. The AA showed tumor-like local expansion, with a maximum diameter of approximately 4 cm. After consultation with the department of cardiac macrovascular surgery, the patient was diagnosed with left CAA with aneurysm formation and an anomalous RSCA and LVA and was transferred to that department. The patient underwent AA aneurysm resection and artificial blood vessel replacement under general anesthesia and cardiopulmonary bypass. No abnormality was found during the 2-mo follow-up after discharge. CONCLUSION: A CAA is a rare congenital anomaly of vascular development. The present unique case of CAA with aneurysm formation and an anomalous RSCA and LVA enriches existing CAA data.

Left cervical and circumflex aortic arch with aberrant right subclavian artery arising from a Kommerell diverticulum: A rare vascular ring.

We report a rare case of vascular ring due to a left cervical circumflex aortic arch with aberrant right subclavian artery arising from a Kommerell diverticulum. This is a very rare case of vascular ring in a newborn with stridor and stenosis of the left pulmonary artery. A cardiac catheterization and an angio-computed tomography scan were helpful to clarify the diagnosis. The surgical correction was performed with division of the arterial duct, reimplantation of the right subclavian artery, aortic and tracheal suspension, and pulmonary arterial plasty. The patient was asymptomatic at discharge and 1-year follow-up.

Upper Airway Obstruction in an Adult: An Unusual Presentation-Dissection of the Cervical Aortic Arch.

Cervical aortic arch is a rare congenital anomaly. It is usually asymptomatic and an incidental finding but can have symptoms of cough, hoarseness of voice, and dysphagia. Although an association with aneurysmal dilatation is described, dissection of a cervical aortic arch is hitherto an unreported complication. We report a 46-year-old woman recently diagnosed with a cervical aortic arch, who presented with an acute upper airway obstruction leading to cardiorespiratory arrest secondary to spontaneous dissection of the cervical aortic arch. How to cite this article: Satyavolu RSN, Fischer R, Ramadoss R, Upper Airway Obstruction in an Adult: An Unusual Presentation-Dissection of the Cervical Aortic Arch. Indian J Crit Care Med 2021;25(6):732-734.

Virtual modeling and interactive virtual reality display of unusual high-riding cervical aortic arch.

A cervical aortic arch is a rare vascular malformation that is characterized as a high positioned aortic arch, above the clavicle. The knowledge of its branching pattern is essential to characterize the entity further accurately. Noninvasive cross-sectional imaging, including computed tomography angiography or magnetic resonance angiography, is the imaging methods of choice. Due to highly complex anatomy, three-dimensional (3D) images help in providing improved anatomical visualization. Virtual reality is a relatively new computer-generated simulation technique that allows the interactive display of high-resolution models using a wearable headset and interactive controllers. We describe a rare form of a cervical arch and briefly discuss the latest methods of improved visualization using 3D virtual reality displays and smartphones.

Surgical repair of cervical aortic arch: An alternative classification scheme based on experience in 35 patients.

OBJECTIVE: Cervical aortic arch (CAA) is rare and difficult to repair. Clinical experience is limited. We report the surgical techniques and midterm outcomes in 35 patients with CAA based on an alternative classification scheme. METHODS: Of 35 patients with CAA, 30 (85.7%) had left-sided aortic arch and 5 had (14.3%) right-sided aortic arch (all 5 had a vascular ring). Mean age was 34.2 ± 13.1 years, 23 were female (65.7%), and 18 were asymptomatic (51.4%). Surgical access and procedure were chosen according to an alternative classification scheme that is based on the presence or absence of vascular ring and relationship of descending aorta to the side of the aortic arch. In the left-sided aortic arch group, aortic arch reconstruction though median sternotomy was performed in 15 patients, and distal arch and descending thoracic aortic replacement via left thoracotomy in 15 patients. In the right-sided aortic arch group, ascending-to-descending aortic bypass was done via median sternotomy in 2 patients and right thoracotomy in 1, and distal arch and descending thoracic aortic replacement via right thoracotomy in 2 patients. RESULTS: Neither death nor spinal cord injury occurred. Left recurrent laryngeal nerve injury, prolonged ventilation, and reexploration for bleeding occurred in 1 each. In 11 patients with coarctation, the upper-lower limb gradient decreased significantly postoperatively (from 34.0 ± 12.7 to 10.2 ± 2.7 mm Hg; P < .01). The diseased aortic segment was excluded in 34 patients, except 1 with residual aneurysm in the proximal descending thoracic aorta. Follow-up was complete in 100% at mean 4.4 ± 2.0 years. No late death, limb ischemia, or stroke occurred. Endovascular repair was performed in 1 patient, and ascending aortic dilation occurred in 1 patient. The residual aorta remained nondilated in 33 patients. Aortic grafts were patent in 100%, with no anastomotic leak or pseudoaneurysm. At 6 years, the incidences of death, aortic events, and event-free survival were 0%, 6.5%, and 93.5%, respectively. CONCLUSIONS: Open repair of CAA can achieve favorable early and midterm outcomes. Surgical accesses and procedures should be chosen based on type of CAA, anatomic variations and associated anomalies. Our alternative categorization scheme of CAA is intuitive and comprehensive, which may facilitate classification and surgical decision making.

Management of cervical aortic arch complicated by multiple aneurysms.

OBJECTIVES: Cervical aortic arch with aneurysm formation is considered an extremely rare condition. Here, we summarize our experience in treating 8 patients. We validated extra-anatomic ascending-to-infrarenal abdominal aorta bypass through the retroperitoneal cavity without circulatory arrest as an alternative treatment for patients with a tortuous arch that was unsuitable for endovascular repair. METHODS: From March 2015 to April 2018, 8 patients (7 women; median age 46 years) diagnosed with cervical aortic arch complicated with aneurysm formation were treated at Peking Union Medical College Hospital and the Affiliated Hospital of Qingdao University. After assessment of the anatomical characteristics, 4 patients underwent endovascular repair. Three patients with a tortuous aortic arch and saccular aneurysm formation between the left common carotid artery and the left subclavian artery were treated with an extra-anatomic ascending-to-infrarenal abdominal aorta bypass and aneurysm indwelling. One patient refused surgical intervention and is being followed up on a yearly basis at our outpatient clinic. No circulatory arrest was required during surgery. RESULTS: No severe postoperative complications were observed during follow-up (6-36 months). Postoperative computed tomography angiography revealed patent blood flow in the prosthetic aortic graft bypass. No endoleak, migration or stenosis of the stent grafts was observed in patients following endovascular repair. The left subclavian artery was preserved in 3 patients. Follow-up computed tomography angiography revealed satisfactory postoperative results in all patients, with no signs of aortic dilation or coarctation. CONCLUSIONS: Ascending-to-infrarenal abdominal aorta bypass through the retroperitoneal cavity is a safe and effective treatment for cervical aortic arch with a tortuous aorta complicated by aneurysm formation and coarctation.

Anomaly of Haughton type D left cervical aortic arch in combination with type B dissection: case report and literature review.

BACKGROUND: The anomaly of cervical aortic arch is a rare phenomenon first described by Reid in 1914 and categorized by Haughton in 1975. The left cervical aortic arch Type D consisting of an ipsilateral descending aorta and coarctation or aneurysmatic formation of the arch demonstrates a complicated form requiring surgical management. Because of its rarity and unspecific symptoms only few cases are documented with the focus on surgical management. CASE PRESENTATION: A 43-year old, asymptomatic woman presented with a mediastinal mass overlapping the aortic arch region in a routine x-ray. For verification, a computed tomography was performed and revealed incidentally a type B dissection originating from an aneurysm of a left cervical arch with a maximum diameter of 6 cm. Because of the huge diameter and the potential risk of rupture, an urgent surgical repair was planned. Surgical access was performed through median sternotomy and an additional left lateral thoracic incision through the fourth intercostal space. Simultaneously to the preparation, partial cardiopulmonary bypass was installed in the left groin. After preparation of the recurrent and phrenic nerve and the supraaortic branches, the descending aorta was clamped. Before the distal anastomosis to a straight graft, we performed a fenestration of the dissection membrane about a length of 5 cm to preserve the perfusion of both lumina. Then, the straight graft was sutured to the proximal part of descending aorta. The left axillary artery originated directly from the aneurysm and was dissected and reimplanted with a separate 8 mm sidegraft to the straight graft between the distal arch and proximal descending aorta. The patient was extubated on first postoperative day and recovered well. CONCLUSION: The left cervical aortic arch type D is a rare disease, which is prone to aneurysm formation due to abnormal flow patterns and tortuosity of the aorta. The difficulty lays in the identification of the pathology, especially in the physical examination, since a pulsating mass or cervical murmur seem to be the most specific symptoms in the majority of young, female patients. If diagnosed, surgical therapy with resection of the aneurysm and reimplantation of the axillary artery under cardiopulmonary bypass demonstrates the treatment of choice.

An unusual case of interrupted cervical aortic arch associated with long segment coarctation of the descending thoracic aorta.

Interrupted aortic arch is a rare congenital anomaly in newborns and infants and is commonly associated with other cardiovascular anomalies. Here, we report an unusual case of type A interrupted cervical aortic arch associated with long segment coarctation of the descending thoracic aorta. Patent ductus arteriosus reconstituted the descending thoracic aorta. Proximal segments of the left common carotid and left subclavian arteries were atretic. Echocardiography-gated multidetector CT angiography not only identified the type of aortic arch interruption in the neonate but also delineated the exact anatomical details.

Surgical repair of a pseudocoarctation with cervical aortic arch complicated by multiple aneurysms of the aorta: a case report.

Aortic pseudocoarctation is a rare congenital anomaly characterized by elongation and deformity of the aortic arch and is known to be associated with aneurysmal formation. Several studies unite to say it leads to a surgical sanction as soon as symptomatic or associated with aneurysms of the aortic arch. Our patient is a 12 years old boy, followed since birth for a little tight pseudocoarctation with a cervical aortic arch and transverse aortic arch hypoplasia. Close clinical and paraclinical monitoring including angioscans, showed the gradual enlargement of the superior mediastinum, in relation with the appearance of three aneurysms of the aortic arch. The intervention, performed by sternotomy, has consisted of the resection of the aneurysmal area and the interposition of a Dacron tube to repair the aortic arch and the reimplantation of the left subclavian artery into the left carotid artery. The postoperative course was uneventful. Management of pseudocoarctation associated with cervical aortic arch and aneurysms remains surgical. Close monitoring of patients with pseudocorctation, seems to be essential to avoid fatal complications such as aneurysmal rupture.

Surgical repair of left-sided cervical aortic arch aneurysm / 中华胸心血管外科杂志

Objective To summarize our experience of surgical repair for cervical aortic arch(CAA) aneurysm and eval-uate early and midterm results of these patients.Methods From January 2010 to December 2014, 22 patients with left-sided CAA aneurysm admitted in our center.There were 6 male and 16 female patients with a mean age of(34.09 ±13.14) years. Comorbidities included pseudocoarctation in 9 patients, hypertension in 4 patients, and aortic valve insufficiency, Stanford type B aortic dissection and middle cerebral artery aneurysm each had 1 patient.All of the patients underwent surgical aortic arch re-construction using artificial graft replacement.Among them, 4(4/22, 18.18%) were performed under moderate hypothermic circulatory arrest(MHCA) combined with selective antegrade cerebral perfusion(SACP) via a median sternotomy, and concom-itant aortic valve replacement(AVR) was implemented in 1 patient.18(18/22, 81.82%) were performed via posterolateral left thoracotomy through the 4th intercostal space, and adjunct methods applied included partial CPB and “simple clamping” in 10 and 8 of these patients respectively.Results The average mechanical ventilation time and ICU stay time was (13.05 ± 4.73)h and(19.14 ±8.08) h respectively.1 patient required repeat thoracotomy for bleeding, 1 patient with delayed wound healing and 1 patient suffered transient liver dysfunction.There were no in-hospital deaths.Mean follow-up time was 34.73 months, and 3 patients were lost during follow-up.There were no late deaths during follow-up.Conclusion Repair of CAA is indicated for the patients with arch aneurysm formation .According to the locations and types of aneurysms and other concomi-tant proximal cardiovascular diseases, performing one-stage surgical aortic arch reconstruction with individualized incisions , ad-junct methods and operative procedures can obtain satisfactory clinical outcomes in patients with CAA aneurysm .

Cervical aortic arch with multiple aortic aneurysms that required two aortic replacements with a 34-year interval.

A 57-year old female was referred to our hospital after symptoms of hoarseness and dysphagia for a few years. She was diagnosed with a cervical vessel anomaly during childhood and had undergone surgery for a right-sided thoracic descending aortic aneurysm through a right thoracotomy at the age of 23 years. Now, enhanced computed tomography revealed newly developed multiple aneurysms in the aorta between the left carotid artery and the proximal descending aorta in the left hemithorax. A successful aortic surgical repair was accomplished via a left posterolateral thoracotomy. To our knowledge, this is the first case of a cervical aortic arch with formation of multiple aneurysms that required multiple surgeries via bilateral thoracotomies. This case suggests the inherent fragility and possibility of later aneurysmal formation in malformed vessels, thereby emphasizing the importance of periodical imaging tests in this rare congenital anomaly.

Pulsatile neck mass: A rare cause.

A 20-year-old female presented with a pulsatile neck mass. On evaluation, she was found to have right cervical aortic arch, which is a rare anomaly. We highlight the conventional and Computed tomography angiography features of this vascular anomaly.