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Query: UMLS:C0018681 (headache)
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Interrupted aortic arch (IAA) is an uncommon and usually lethal congenital malformation. The present report describes an unusual case of IAA, an aneurysmal ascending aorta and a bicuspid aorta in a 15-year-old boy. He presented with general malaise, weakness of his legs, headache and hypertension that began six months earlier. He had suffered from effort intolerance since childhood. A three-dimensional gadolinium contrast-enhanced magnetic resonance angiogram demonstrated IAA and a markedly developed collateral circulation. IAA is an uncommon disease that is rarely encountered in an adolescent patient with nonspecific symptoms or hypertension. The present case also shows the clinical value of three-dimensional gadolinium contrast-enhanced magnetic resonance angiography for noninvasive assessment of the aortic arch, and magnetic resonance angiography can replace angiography in the assessment of aortic arch anomalies and visualization of well-developed collaterals.
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PMID:Interrupted aortic arch in an adolescent male. 1556 2

Hypertension is an increasingly important medical and public health issue. Appropriate diagnosis and treatment of hypertension is very important in both reducing the morbidity, mortality, and cost related to it. Interrupted aortic arch (IAA) is an uncommon and usually lethal congenital malformation. It is very rarely encountered in adult patients who generally present with nonspecific symptoms and hypertension. Substantial collateral circulation must be present to maintain blood flow to tissues below the aortic interruption and thus to enable survival. A 40-year-old man presented with general malaise, frequent headaches, weakness in his legs, and hypertension. He had suffered from effort intolerance since childhood. Physical examination revealed upper limb hypertension. Lower-limb pulses were not palpable. Transthoracic echocardiography, aortography, and gadolinium contrast-enhanced magnetic resonance angiography revealed complete interruption of the aortic arch just distal to the origin of the left subclavian artery. The present case describes an unusual case of IAA, in which the diagnosis was delayed until the age of 40 years. A complete physical examination would have ensured the correct diagnosis was made much earlier. As our case implies, physical examination maintains its pivotal role in the diagnosis of some forms of secondary hypertension, although various high-tech diagnostic tools are needed for confirmation.
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PMID:A case of misdiagnosed interrupted aortic arch as primary hypertension for almost two decades. 1765 53