Surgical Repair of Middle Aortic Syndrome in a Three-Year-Old Patient

Ayik, Fatih; Engin, Çağatay; Ertugay, Serkan; Atay, Yüksel

doi:10.1111/j.1540-8191.2011.01322.x

Cited by 7 publications

(5 citation statements)

References 27 publications

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“…Specifically, the young girl reported severe mid-aortic stenosis with a degree of obstruction equal to 59%, close to the threshold (mid-aortic stenosis ≥ 60%) reported by Porras et al ( 4 ) for patients highly likely to require invasive management of MAS. Furthermore, the persistent severe hypertension reported by the patient was refractory to medical therapy, thus potentially exposing the patient to more serious complications such as stroke and congestive heart failure ( 11 ). Major concerns were also associated with the low weight of the baby (i.e., 27 Kg), which may increase the risk of vascular access damage, and the young age, already recognized as a risk factor for vascular complications ( 4 ).…”

Section: Discussionmentioning

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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Section: Discussionmentioning

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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“…However, Surgery may be attempted in infancy but is technically difficult [ 16 , 17 ]. And also, PTA is not likely to achieve a favorable outcome in patients with long-segmental narrowing or hypoplasia [ 18 ].…”

Section: Discussionmentioning

confidence: 99%

Long-segmental middle aortic coarctation: a rare case first diagnosed by transthoracic echocardiography

Wang

Yang

Lai

2022

BMC Cardiovasc Disord

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Background Middle aortic coarctation (MAC), also known as middle aortic syndrome, is an atypical aortic coarctation characterized by narrowing of the distal thoracic aorta and proximal abdominal aorta. MAC is a rare disease commonly diagnosed by computed tomography angiography (CTA). In this paper, we present a case of long-segmental MAC first diagnosed by transthoracic echocardiography (TTE) and further evaluated by CTA. Case presentation. A 14-year-old girl, with dyspnea and fatigue on exertion for 2 months and refractory hypertension for 6 months, was referred by the local clinic to our hospital. Physical examination showed blood pressure up to 176/100 mmHg measured in the arms despite dual antihypertensives, a marked pressure gradient between her arms and legs, and weak pulses in both dorsal pedes arteries. TTE revealed a segmental narrowing in the descending thoracic aorta below the level of the atrioventricular sulcus, with a calcified plaque in the stenotic region. Abdominal vascular ultrasound revealed the segmental narrowing extending to the descending abdominal aorta (5.7 mm in diameter) above the level of the superior mesenteric artery. Subsequently, CTA verified a long-segment narrowing in the descending aorta from the level of T8 to L2 vertebra, with a calcified plaque in the stenotic aorta, right renal artery involvement, and a rich network of collateral vessels between the pre-and post-stenotic region. The patient was referred for cardiovascular surgery in which a successful ascending aorta-abdominal aorta bypass was performed. Conclusions Although MAC is usually diagnosed by CTA, it may also be first diagnosed by TTE in some patients whose longitudinal axis view of the thoracic descending aorta could be shown. Careful TTE scan can improve the diagnostic rate of MAC, especially for some hypertension patients whose marked pressure gradient between arms and legs was ignored by the physician.

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“…An anatomic classification system divides MAS into hypoplastic and segmental groups 15 . The affected aorta in relation to the renal arteries was used to create subgroups: suprarenal, inter-renal, and infrarenal 15, 17. Another classification system based on the on the relationship between the location of the abdominal coarctation to the renal arteries (suprarenal vs infrarenal) and whether renal artery stenosis was present or absent 17 …”

Section: Discussionmentioning

confidence: 99%

“…Aortic occlusive disease can be treated by bypass, aortoplasty, or percutaneous transluminal angioplasty (PTA). However, PTA is not likely to achieve a favorable outcome in patients with longer segments or hypoplasia 17 . Failures after PTA alone suggest that stent placement can be used to overcome the postdilatation recoil of aortic narrowing that is remote from the visceral arteries 16 .…”

Section: Discussionmentioning

confidence: 99%

Long-segment thoracoabdominal aortic coarctation in a child with Down syndrome

Vasconcelos

Cal

Gomes

et al. 2015

Journal of Vascular Surgery Cases

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Midaortic syndrome is a rare vascular anomaly characterized by coarctation of the descending thoracic and abdominal aorta. Down syndrome is associated with multiple congenital cardiac malformations but is rarely associated with developmental vascular anomalies. Midaortic syndrome may result in severe renovascular hypertension that requires early intervention to prevent life-threatening complications. We report a child with Down syndrome who presented with occlusion of the aorta and was treated with aortic bypass. More than 4 years after the procedure, the patient's renal function remains normal, and there is no evidence of recurrent hypertension. Long-term follow-up is important to assess the benefits of surgical repair.

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Surgical Repair of Middle Aortic Syndrome in a Three-Year-Old Patient

Cited by 7 publications

References 27 publications

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

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

Long-segmental middle aortic coarctation: a rare case first diagnosed by transthoracic echocardiography

Long-segment thoracoabdominal aortic coarctation in a child with Down syndrome

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