Clinical Characteristics and Long-Term Outcomes of Midaortic Syndrome

Patel, R. J.; Nguyen, Stéphanie; Lee, Michelle; Price, Matt D.; Krause, Heidi; Truong, Van Thi Thanh; Sandhu, Harleen K.; Charlton-Ouw, Kristofer M.; LeMaire, Scott A.; Coselli, Joseph S.; Prakash, Siddharth K.

doi:10.1016/j.avsg.2019.12.039

Cited by 18 publications

(11 citation statements)

References 20 publications

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“…The total incidence rate of the variation type is less than 1%, which is a rare type and is mostly seen in case reports [1] . Developmental abnormalities of the distal aortic arch accompanied by descending aortic stenosis are common in reports [2 , 3] .…”

Section: Discussionmentioning

confidence: 99%

Distal aortic arch dysplasia accompanied by multiple aneurysms in adult: A case report and review of the literature

Chen,

Yin,

Yang

et al. 2023

Radiology Case Reports

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Section: Discussionmentioning

confidence: 99%

Distal aortic arch dysplasia accompanied by multiple aneurysms in adult: A case report and review of the literature

Chen,

Yin,

Yang

et al. 2023

Radiology Case Reports

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“…NF1 is the most common genetic disorder associated with MAS and encountered for 5–22% of the cases. 1 , 3 , 6 , 7 , 16 , 17 , 19 Another study found NF1 as the second cause of MAS after Williams syndrome. 3 …”

Section: Discussionmentioning

confidence: 99%

Diagnosis and Management of Genetic Causes of Middle Aortic Syndrome in Children: A Comprehensive Literature Review

Lazea¹,

Alkhzouz²,

Sufana³

et al. 2022

TCRM

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Middle aortic syndrome (MAS) is a rare vascular disease representing an important cause of severe hypertension in children. MAS is characterized by segmental or diffuse narrowing of the abdominal and/or distal descending aorta with involvement of the renal and visceral branches. Most cases of MAS are idiopathic, but MAS may occur in genetic and acquired disorders. The most common genetic causes of MAS are neurofibromatosis type I, Williams syndrome, Alagille syndrome, tuberous sclerosis and mucopolysaccharidosis. This review article discusses the pathophysiological aspects, distinctive associated features, and management of genetic forms of MAS in children.

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“…Mid-aortic syndrome (MAS) is a rare disease presenting in pediatric patients with segmental narrowing of the abdominal or distal thoracic aorta and involvement of renal and splanchnic arterial branches, with about 60% of cases with renal involvement exhibiting bilateral renal artery stenosis ( 1 – 3 ). Notably, more than 90% pediatric MAS patients require medical therapy for severe and uncontrolled hypertension ( 4 ), which is recognized as major symptom of MAS in children and can lead to renal failure, congestive heart failure, and cerebrovascular accidents ( 1 , 5 , 6 ).…”

Section: Introductionmentioning

confidence: 99%

Case report: Personalized transcatheter approach to mid-aortic syndrome by in vitro simulation on a 3-dimensional printed model

Giugno

Formato

Chessa

et al. 2023

Front. Cardiovasc. Med.

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An 8-year-old girl, diagnosed with mid-aortic syndrome (MAS) at the age of 2 months and under antihypertensive therapy, presented with severe systemic hypertension (>200/120 mmHg). Computed tomography (CT) examination revealed aortic aneurysm between severe stenoses at pre- and infra-renal segments, and occlusion of principal splanchnic arteries with peripheral collateral revascularization. Based on CT imaging, preoperative three-dimensional (3D) anatomy was reconstructed to assess aortic dimensions and a dedicated in vitro planning platform was designed to investigate the feasibility of a stenting procedure under fluoroscopic guidance. The in vitro system was designed to incorporate a translucent flexible 3D-printed patient-specific model filled with saline. A covered 8-zig 45-mm-long Cheatham-Platinum (CP) stent and a bare 8-zig, 34-mm-long CP stent were implanted with partial overlap to treat the stenoses (global peak-to-peak pressure gradient > 60 mmHg), excluding the aneurysm and avoiding risk of renal arteries occlusion. Percutaneous procedure was successfully performed with no residual pressure gradient and exactly replicating the strategy tested in vitro. Also, as investigated on the 3D-printed model, additional angioplasty was feasible across the frames of the stent to improve bilateral renal flow. Postoperative systemic pressure significantly reduced (130/70 mmHg) as well as dosage of antihypertensive therapy. This is the first report demonstrating the use of a 3D-printed model to effectively plan percutaneous intervention in a complex pediatric MAS case: taking full advantage of the combined use of a patient-specific 3D model and a dedicated in vitro platform, feasibility of the stenting procedure was successfully tested during pre-procedural assessment. Hence, use of patient-specific 3D-printed models and in vitro dedicated platforms is encouraged to assist pre-procedural planning and personalize treatment, thus enhancing intervention success.

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Clinical Characteristics and Long-Term Outcomes of Midaortic Syndrome

Cited by 18 publications

References 20 publications

Distal aortic arch dysplasia accompanied by multiple aneurysms in adult: A case report and review of the literature

Distal aortic arch dysplasia accompanied by multiple aneurysms in adult: A case report and review of the literature

Diagnosis and Management of Genetic Causes of Middle Aortic Syndrome in Children: A Comprehensive Literature Review

Case report: Personalized transcatheter approach to mid-aortic syndrome by in vitro simulation on a 3-dimensional printed model

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