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Native Aortic Coarctation Presenting As Prolonged Pyrexia In A Teenager With 22q11.2 Deletion

A. Karatza, D. Gkentzi, E. Kostopoulou, S. Rammos
Published 2019 · Medicine

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A 14-year-old boy presented with a 1-month history of fever up to 40 C. He also had malaise, vomiting, decreased appetite and weight loss. He was prescribed antibiotics twice for presumed respiratory tract infection. He had white cell count of 14 080/ mm (polymorphonuclear: 84%), erythrocyte sedimentation rate of 50 mm/h and C-reactive protein of 6 mg/L. Staphylococcus aureus was isolated from two blood cultures and antibiotics were prescribed based on the antibiogram. He looked dysmorphic, had extended tooth decay and unilateral ptosis of the mouth angle. Parents reported learning difficulties. A 3/6 systolic murmur was audible along the left sternal border radiating to the subclavicular area. Femoral pulses were weak with radiofemoral delay and blood pressure (BP) was 110/70 mmHg at the arm and 75/50 mmHg at the leg. Chest radiography showed cardiomegaly, increased pulmonary vascular markings and a round opacity at the left upper cardiac border (Fig. 1a). Echocardiography revealed severe aortic coarctation (CoA), a bicuspid aortic valve and an aneurysm proximally to the CoA site (Fig. 1b,c). Computed tomography angiography diagnosed a large saccular aneurysm at the origin of the left subclavian artery (measuring 5.2 × 4.4 cm), which also had enlarged diameter (13 mm) (Figs 2,3). Right brachiocephalic artery was aneurysmatic (14.5 mm), as well as right subclavian artery (16.4 mm). There was an aberrant left anonymous vein with left paracardiac course draining to the coronary sinus. With the diagnosis of a mycotic aneurysm of the left subclavian artery in the setting of native CoA, the patient was transferred to a cardiothoracic centre, where he was initially managed conservatively with intravenous antibiotics. On day 11 of hospitalisation he developed a septic embolism of the popliteal artery requiring surgical embolectomy. He underwent surgical repair of the CoA and closure of the aneurysm due to severe adhesions, which made its removal technically difficult and dangerous, on day 22, and completed a 6-week antibiotic course. Fluorescent in situ hybridisation identified 22.q11.2 deletion, which was found to be present in his mother.
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