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Image in Cardiology
Mid-aortic syndrome in a patient with neurofibromatosis type 1
Síndrome da aorta média em paciente com neurofibromatose tipo 1
Sara P. Magalhãesa,
Corresponding author
sarapintomagalhaes@sapo.pt

Corresponding author.
, Nuno Morenob, Nuno Alvesa, Fernanda Reisa
a Serviço de Radiologia do Centro Hospitalar do Porto, Porto, Portugal
b Serviço de Cardiologia do Centro Hospitalar Tâmega e Sousa, Penafiel, Portugal
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Mid-aortic syndrome is an uncommon condition characterized by constriction of the distal thoracic and&#47;or abdominal aorta and its branches&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 43-year-old male patient with a history of neurofibromatosis type 1 &#40;NF-1&#41; was referred to our hospital for resistant arterial hypertension&#46; At physical examination he presented asymmetric elevated blood pressure in the extremities&#44; higher in the upper limbs&#46; An echocardiogram was performed and showed moderate left ventricular hypertrophy&#44; without signs of coarctation of the aorta&#46; As a part of workup for hypertension&#44; computed tomography &#40;CT&#41; was performed&#44; which showed an abnormal aorta with severe narrowing and a saccular aneurysm at the level of the renal arteries &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figures 1 and 2</a>&#41;&#46; There was extensive collateral blood flow through hypertrophic lumbar&#44; epigastric and mesenteric arteries and the left renal artery showed subtotal stenosis at its origin&#44; causing atrophy of the left kidney &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46; Additional angiographic study enabled a final diagnosis&#46; There were also subcutaneous and retroperitoneal neurofibromas&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Mid-aortic syndrome usually presents with arterial hypertension and is commonly diagnosed in children or young adults&#46; It can be associated with Williams syndrome&#44; Takayasu arteritis&#44; NF-1&#44; Alagille syndrome and Moyamoya disease&#46; Surgery is the primary treatment when it is associated with renovascular hypertension and visceral artery stenosis&#46; Endovascular therapy can be performed in patients with discrete aortic stenosis&#59; however&#44; due to inherent arterial wall anomalies&#44; vascular complications increase after percutaneous procedures&#44; thus making them poor options&#46; Our patient refused surgical procedures and has been treated with medical therapy&#46; Prognosis is generally good&#44; but the syndrome may have high morbidity and mortality if left untreated&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Ethical disclosures</span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Protection of human and animal subjects</span><p id="par0020" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Confidentiality of data</span><p id="par0025" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Right to privacy and informed consent</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conflicts of interest</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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Article information
ISSN: 21742049
Original language: English
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Revista Portuguesa de Cardiologia (English edition)
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