Right corkscrew cervical aortic arch

Fernández-Doblas, Joaquín; Taurón, Manel; Blasco, A.; Abella, Raúl

doi:10.1093/ejcts/ezs340

Cited by 2 publications

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“…A “corkscrew aortic arch” refers to extreme tortuosity occasionally encountered in arterial tortuosity syndrome, but not previously reported in PHACES syndrome. [56] The aortic arch anomalies, namely long-segment transverse arch involvement, tortuosity of vessels, aberrancy of subclavian origin in 60% of patients, and stenosis of arch branches, offer unique challenges in diagnosis and management and increase threshold for surgical interventions. The disease severity is underestimated both clinically and on echocardiography.…”

Section: Discussionmentioning

confidence: 99%

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Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome

et al. 2019

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PHACES syndrome, a diffuse aortocraniocerebral vasculopathy, is a neural tube migration disorder, characterized by aortic coarctation and aberrant arch branches. Clinical diagnosis, echocardiography, and surgical management of coarctation in this syndrome are challenging due to peculiar morphological differences. Corkscrew aortic arch, an extreme tortuosity of the aortic arch described in arterial tortuosity syndrome, is not reported in PHACES syndrome so far. Multimodal imaging of this unusual corkscrew aortic arch in two patients with PHACES syndrome is presented.

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Section: Discussionmentioning

confidence: 99%

“…[4] Corkscrew aorta is a term used to describe extreme tortuosity and often seen in arterial tortuosity syndrome, an entirely different single-gene disease involving SLC2A10 gene in chromosome 20q13 that encodes GLUT10 transporter. [56] Multimodality imaging of corkscrew aorta in two different patients with PHACES syndrome is presented.…”

Section: Introductionmentioning

confidence: 99%

Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome

et al. 2019

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

Baudo,

Varrica,

Reali

et al. 2023

Front. Cardiovasc. Med.

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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.

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Right corkscrew cervical aortic arch

Cited by 2 publications

References 0 publications

Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome

Corkscrew aortic arch in PHACES syndrome: Multimodal imaging of an unusual morphology of tortuous aortic arch in a rare but well-defined syndrome

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

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