Issue: 2017 > September > photo quiz

A curvilinear sword

B.A. Khan, S.H. Imam, S.H. Khan, S. Shah, D. Chaudhuri
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An 18-year-old woman presented with a 3-year history of dyspnoea on exertion. Chest X-ray showed right lung hypoplasia with dextroposition of the heart. A CT scan of the chest revealed anomalous venous drainage of the right pulmonary vein into the inferior vena cava (IVC) (figure 1A). Transthoracic echocardiography showed normal sized chambers and normal pulmonary artery pressure with no evidence of an intra-cardiac defect. Two years later, she experienced worsening dyspnoea with significant exercise limitations and recurrent episodes of pneumonia. She could not undergo cardiac MRI due to severe claustrophobia. Subsequently, she had pulmonary angiography, which revealed a scimitar (curvilinear sword shaped) vein from the right lung draining into the supra-diaphragmatic IVC, consistent with the diagnosis of scimitar syndrome (figure 1B). Right cardiac catheterisation demonstrated normal right ventricular (24/2 mmHg) and pulmonary artery (27/10 mmHg) pressures. No intra-cardiac shunt was noticed. She underwent surgical correction and her symptoms improved significantly.