Pseudocoarctation of the aorta presenting as a mediastinal mass with dysphagia

Kessler, Randolph M.; Miller, Kevin B.; Pett, Stuart; Wernly, Jorge A.

doi:10.1016/0003-4975(93)90137-7

Cited by 27 publications

(14 citation statements)

References 6 publications

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“…12 Adults with such findings may present with dysphagia, with associated rightward displacement and left-sided indentation of the oesophagus visible on the barium swallow. 4 - 9 The majority, nonetheless, appear to be asymptomatic.…”

Section: Discussionmentioning

confidence: 99%

Severe tortuosity and stenosis of the systemic, pulmonary and coronary vessels in 12 patients with similar phenotypic features: a new syndrome?

et al. 2000

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We describe what is, to the best of our knowledge, a previously unreported association in patients with similar facial features, skin and joint laxity, of lengthening and tortuosity of systemic, pulmonary and coronary vessels. We evaluated 12 patients with similar phenotypes, from eight different families. Detailed echocardiographic and angiographic evaluations were performed in all, and biopsies of the skin in seven. All patients have elongated facies, prominent ears, micrognathia and laxity of their joints. Angiographic pictures showed a varying degree of lengthening and tortuosity of systemic, pulmonary, and coronary arteries. Pulsatile carotid arteries formed cervical masses in 2 patients, and three had severe renal arterial stenoses. All showed varying degrees of branch and peripheral pulmonary arterial stenosis, necessitating placement of stents in six. Biopsy of the skin proved normal in all seven patients studied, thus excluding cutis laxa, Ehlers-Danlos and Marfan syndromes. The constellation of abnormalities suggests a genetic syndrome of connective tissue etiology. Further genetic studies, and gene mapping, are underway.

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Section: Discussionmentioning

confidence: 99%

Severe tortuosity and stenosis of the systemic, pulmonary and coronary vessels in 12 patients with similar phenotypic features: a new syndrome?

et al. 2000

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“…Pseudocoarctation is a rare anomaly characterized by kinking or buckling of the descending aorta at the level of the ligamentum arteriosum (74) (Fig 26). Unlike with true aortic coarctation, there is no significant hemodynamic obstruction or pressure gradient across the lesion (<25 mm Hg), and therefore no collateral vessel formation (75).…”

Section: Pseudocoarctationmentioning

confidence: 99%

“…Pseudocoarctation is usually asymptomatic and is generally considered to be a benign entity that does not require surgical intervention. However, treatment may be required in symptomatic patients and those with associated aneurysm formation, as aneurysms associated with pseudocoarctation may result in sudden rupture or dissection, which can be fatal (74,75,78). The pathogenesis of aneurysm formation beyond the kinked segment may relate to turbulent flow, resulting in damage to the arterial wall (79).…”

Section: Pseudocoarctationmentioning

confidence: 99%

Congenital Variants and Anomalies of the Aortic Arch

Hanneman¹,

Newman²,

Chan³

2017

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Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression. Certain arch anomalies are strongly associated with congenital heart disease, including right aortic arch with mirror image branching. Other malformations of the aortic arch have important associations, such as type B interrupted aortic arch, which is associated with a locus 22q11.2 microdeletion. Noninvasive imaging at CT angiography and MR angiography allows for comprehensive evaluation of the aortic arch and branch vessels in relation to surrounding structures. Familiarity with the spectrum and imaging appearances of aortic arch variants, anomalies, and malformations is essential for accurate diagnosis and classification and to guide management. Online supplemental material is available for this article. RSNA, 2016.

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“…The identification of aortic pseudocoarctation can be an incidental finding or associated with hypertension and symptoms related to compression of adjacent structures 1. Its management is not well established, but surgical treatment has been proposed in symptomatic patients or when associated with aortic aneurysms 3. The case was discussed in a multidisciplinary team, and it was decided that the pseudocoarctation did not require intervention and should be regularly monitored for changes in the aorta.…”

Section: Descriptionmentioning

confidence: 99%