Infantile midaortic syndrome with aortic occlusion

Minson, Susan; McLaren, Clare A.; Roebuck, Derek J.; Tullus, Kjell

doi:10.1007/s00467-011-2039-y

Cited by 13 publications

(5 citation statements)

References 11 publications

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“…The most common lesion site is in the suprarenal region 1 . Surgery or endovascular treatment is the mainstay therapy for MAS 2,4–7 . The patient's age, the location of the stenosis, the length of the lesion, the extent of visceral vessel involvement, clinical presentation, and response to medical therapy are all factors that should be considered before invasive treatment.…”

Section: Discussionmentioning

confidence: 99%

“… 1 Surgery or endovascular treatment is the mainstay therapy for MAS. 2 , 4 , 5 , 6 , 7 The patient's age, the location of the stenosis, the length of the lesion, the extent of visceral vessel involvement, clinical presentation, and response to medical therapy are all factors that should be considered before invasive treatment. In infants, the outcomes from surgical approaches including bypass grafting or aortic patch grafting are poor due to the high rate of reintervention and complications.…”

Section: Discussionmentioning

confidence: 99%

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Mid‐aortic syndrome: a rare cause of heart failure in infants

et al. 2022

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This case reports describe a rare disease, mid‐aortic syndrome (MAS), that can cause severe heart failure and hypertension in infancy. The typical images, key points of diagnosis, and therapy methods of the disease have also been presented. We report two critical thoracoabdominal aortic coarctation cases in infants aged 2 and 11 months with severe heart failure. The patients were initially misdiagnosed as dilated myocardiopathy, with the correct diagnosis confirmed through imaging. Both patients underwent balloon angioplasty; one patient also had bare‐metal stents implanted. The patient treated with balloon angioplasty alone died after the procedure, whereas the other patient recovered well. In conclusion, careful physical examinations, especially upper and lower extremity blood pressure differences and palpation of upper and lower limb pulses, are critical in unexplained infant heart failure cases. Stent implantation may be a safer and more effective treatment than simple balloon angioplasty in infants with MAS.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Mid‐aortic syndrome: a rare cause of heart failure in infants

et al. 2022

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“…Surgical treatments are required for patients with severe stenosis leading to refractory hypertension and targeted organ injury, which include bypass, angioplasty, or percutaneous transluminal angioplasty(PTA). 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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“…MAS has multiple congenital and acquired etiologies. MAS is speculated to be a result of incomplete fusion or overfusion of the paired embryonic dorsal aortae during the fourth week of gestation 12, 13, 14. Congenital causes include neurofibromatosis, William syndrome, Marfan syndrome, and mucopolysaccharidosis 8 .…”

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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Infantile midaortic syndrome with aortic occlusion

Cited by 13 publications

References 11 publications

Mid‐aortic syndrome: a rare cause of heart failure in infants

Mid‐aortic syndrome: a rare cause of heart failure in infants

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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