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• The cervical aortic arch is a congenital protrusion of the arch above the clavicle. • Two classifications have been proposed—by Haughton and by Zhong. • Often asymptomatic, it can present symptoms related to vascular compression. • In adults, most patients are female, often have a left-sided cervical aortic arch, and have a case associated with aneurysms. • Zhong's classification is capable of identifying additional anatomical features. 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. [Display omitted] [ABSTRACT FROM AUTHOR]

We present hairpin curve tortuous ascending aorta of the fetus using radiant flow and HDlive flow. In a fetus with cystic hygroma and ductus venosus reverse flow at 12 weeks and 6 days of gestation, hypoplastic right ventricle and enlarged right atrium with significant tricuspid regurgitation were noted, and hairpin curve tortuous ascending aorta was evident using radiant flow and HDlive flow. At 17 weeks and 5 days of gestation, chromosome analysis showed Miller-Dieker syndrome [46XY, del(17)(p13.1)]. In another fetus at 21 weeks of gestation, hairpin curve tortuous ascending aorta and dilated pulmonary artery were identified using radiant flow. HDlive flow clearly revealed hairpin curve tortuous ascending aorta, dilated pulmonary artery, and cervical aortic arch. There were no obvious phenotypic features of connective-tissue disorder or other malformation. Tortuous ascending aorta and cervical aortic arch were confirmed after birth. To the best of our knowledge, this might be the first report of hairpin curve tortuous ascending aorta of the fetus with no obvious phenotypic features of connective-tissue disorder. [ABSTRACT FROM AUTHOR]

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.

BackgroundThis 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.MethodsA 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.ResultsThe 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.ConclusionCervical 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 Registrationhttps://www.crd.york.ac.uk/prospero/display_record.php?RecordID=346826, Identifier: CRD42022346826.

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.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.

Purpose: We report the case of a patient with a cervical aortic arch and retro-aortic innominate vein without congenital heart disease. Procedures & main findings: A 40-year-old female was referred to our hospital because of hoarseness for a few months. Computed tomography revealed a saccular aneurysm complicated by a cervical aortic arch with normal brachiocephalic branching, redundant transverse aorta, and left-sided descending aorta. The left innominate vein crossed the aneurysm beneath the aortic arch. Surgical repair was performed under hypothermic circulatory arrest through a fifth intercostal thoracotomy. The arch distal to the left common carotid artery and the proximal descending aorta were replaced with a prosthesis, and the left subclavian artery was reconstructed. The patient was discharged with a favorable postoperative course. Conclusions: Proper diagnosis and relevant surgical manipulations are required for patients with congenital aortic arch abnormalities.

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. [ABSTRACT FROM AUTHOR]

Abstract 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.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Baudo, Varrica, Reali, Saracino, Carminati, Frigiola, Giamberti and Lo Rito.)

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.

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., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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.

Truncus arteriosus, a double aortic arch, and a cervical aortic arch are all rare cardiovascular anomalies. We experienced a unique female newborn with the rare combination of truncus arteriosus with a cervical double aortic arch, which probably resulted from abnormal persistence of the bilateral 2nd or 3rd rather than the 4thembryonic aortic arches and failure of regression of the right 8th somitic segment of the right dorsal aorta. She presented with respiratory distress soon after birth, which was initially attributed to the vascular ring and hypertensive pulmonary arteries. Our inability to relieve her respiratory compromise by surgical division of the vascular ring and main pulmonary artery banding prompted the diagnosis of left main bronchial compression caused by a posteriorly displaced dilated ascending aorta that compressed the right pulmonary artery and left main bronchus against the descending aorta. The patient then underwent successful left main bronchus stent implantation. We speculate the cervical double aortic arch is redundant in nature and is a loose ring that may not cause tracheal compression. Nevertheless, a posteriorly displaced dilated ascending aorta in patients with truncus arteriosus may compress the right pulmonary artery and the main bronchus on the side of the aortic arch against the descending aorta. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Abstract: Cervical aortic arch (CAA) is a rare congenital abnormality in which the aortic arch is situated cranially to its usual position, often associated with complex developmental alteration of aortic laterality and branching. More recently, some authors reported deletion of chromosome 22q11 (del22q11) in few patients with CAA. The aim of this study was to describe the clinical, anatomopathological and genetic pattern of 10 new cases of CAA. From 1975 to 2003, 10 patients with CAA (4 female, mean age 12.4+11.1 years) underwent complete cardiovascular evaluation and screening for del22q11 using fluorescence in situ hybridization. Six patients underwent CAA surgical repair with histological analysis of aorta samples. Six patients were asymptomatic; one patient had syncope on effort, 2 patients had dyspnea on effort and 1 had cyanosis and dyspnea (tricuspid atresia). A murmur and/or a pulsatile mass of the neck was present in all patients. Femoral pulses were weak or absent in 6 patients. Four patients had right and 6 left CAA; kinking or true obstruction of the aortic arch was present in 9/10. Abnormal aortic arch branching pattern was found in 8/10 (mainly aberrant subclavian artery). Two patients had del22q11 with dysmorphic features, multiorgan anomalies and degenerative mediocystic necrosis of aortic wall. Our data confirm clinical polymorphism and anatomic complexity of CAA as well as the association between del22q11 and CCA in 2 syndromic patients. [Copyright &y& Elsevier]

A 55-year-old woman presented with right cervical aortic arch with pseudocoarctation of the aorta further complicated by the presence of multiple aneurysms and a high-grade stenosis at the origin of the left subclavian trunk from the aorta causing a discrepancy in blood pressure between the right and left arms. The branching pattern and the resulting complex steal syndromes involving the left carotid and the subclavian system are unique. The computed tomography angiography, magnetic resonance angiography, and Doppler ultrasound findings are described. [ABSTRACT FROM AUTHOR]

Cervical aortic arch is an unusual malformation. Cervical aortic arch with aneurysm formation is very rare. We report a case of cervical aortic arch associated with a saccular aneurysm in a 59-year-old Japanese man. The aneurysm protruded caudally and was located between the left common carotid and left subclavian arteries. Cardiopulmonary bypass and deep hypothermic circulatory arrest was applied as adjunct methods. A Dacron graft was sutured just distal to the left common carotid artery, with the patient in the Trendelenburg position. The proximal site was left open while oxygen-saturated venous blood was supplied in a retrograde manner to perfuse the lower body during occlusion of the descending aorta. Distal anastomosis to the descending aorta was performed during rewarming. The left subclavian artery was reconstructed by using a branch of the graft. This procedure is simple and useful for distal arch operations, especially in patients with Haughton D type aneurysms. [ABSTRACT FROM AUTHOR]

Cervical arch is a rare congenital anomaly presumed to result from persistence of the third aortic arch and regression of the normal fourth arch. Rather rare is cervical aortic arch associated with aneurysm and obstruction, with eight known cases reported. Definitive diagnosis with a noninvasive imaging modality is desirable and very important to prevent the need for disaster intervention. We present two cases of a pulsatile mass in the left supraclavicular region. Three-dimensional computed tomographic angiography and magnetic resonance angiography clearly showed a left-sided cervical aortic arch (Haughton type D) with arch aneurysm and coarctation (pseudocoarctation). [ABSTRACT FROM AUTHOR]

A right-sided cervical aortic arch, tetralogy of Fallot, and a peculiar tortuosity of the descending aorta in which the aorta formed triple loops were diagnosed by angiocardiography in a 12-year-old girl. Histologic examination of the biopsied wall of the left subclavian artery, taken at the time of a Blalock-Taussig anastomosis, revealed no elastic-tissue defect. [ABSTRACT FROM AUTHOR]

The authors present a case of cervical aortic arch which was discovered in a child of 5 years. The association with a diffuse and major vascular dysplasia, mainly neuroectodermic, is worth reporting. Only 40 cases of this abnormality have been published up to now. The anatomic classification of the different types of cervical aortic arch is presented. [ABSTRACT FROM AUTHOR]