Magnetic resonance imaging (MRI) with MR angiography (MRA) was also performed ( Fig. A tubular structure (1.8 cm in diameter) was seen connecting the main pulmonary artery to the descending aorta, consistent with a large PDA. A ventricular septal defect (VSD) measuring 2.2 cm × 1.2 cm with a predominant left-to-right shunt was seen. (C) A ventricular septal defect could also be visualized (arrow)Ī transthoracic echocardiogram was obtained and showed evidence of right atrial enlargement and right ventricular hypertrophy. The pulmonary trunk (* in A) was markedly dilated. These findings were consistent with type B interrupted aortic arch (IAA).Ĭomputed tomography images showing: (A) and (B) a small-calibre ascending aorta with the aortic arch interrupted after the origin of the left common carotid artery (arrow in A) and separated from the left subclavian artery (arrowhead in B) and descending aorta (curved arrow in B). The ascending aorta was of small calibre. 2) revealed discontinuity between the ascending aorta and descending aorta, with the descending aorta and left subclavian artery supplied by the grossly enlarged pulmonary trunk (measuring 6 cm in diameter) via a patent ductus arteriosus (PDA). Therefore, contrast-enhanced computed tomography (CT) was arranged.ĬT ( Fig. In view of the presence of polycythaemia, the initial clinical suspicion was that of lymphoproliferative disease, for example, lymphoma, or an EPO-producing tumour with paraneoplastic syndrome. There was mild thrombocytopenia with a platelet count of 56×10 9/l. Erythropoietin (EPO) levels were increased, suggestive of secondary polycythaemia rather than polycythaemia rubra vera. Routine blood tests showed a haemoglobin (Hb) level of 22.0 and a haematocrit (Hct) of 0.70. The chest radiograph showed a grossly enlarged cardiac shadow, an enlarged pulmonary trunk and a small-calibre ascending aorta.
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