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Multimodal imaging of anomalous pulmonary venous return in an adult patient with Scimitar syndrome

Lucas Joerg, Olaf Chan-Hi Kim, Reinhard Geyer, Micha T. Maeder
DOI: http://dx.doi.org/10.1093/eurheartj/ehs144 2679 First published online: 4 June 2012

A 40-year-old asymptomatic female was referred for investigation of a systolic murmur. Transthoracic echocardiography revealed dilated right-sided cardiac chambers (Panel A). Transoesopageal echocardiography ruled out an atrial septal defect but demonstrated pulmonary venous flow into the right atrium (Panel B, arrow). Cardiac magnetic resonance imaging (MRI) confirmed the diagnosis (Panels D and E, arrows). Cardiac MRI (Panels D and E) and invasive angiography (Panel C, arrow; contrast injection into the abnormally draining pulmonary vein) showed a large single right-sided pulmonary vein draining into the inferior vena cava. Given a large left-to-right shunt with a pulmonary-to-systemic blood flow ratio of 2.4:1.0, the patient underwent surgical correction with redirection of the pulmonary venous return into the left atrium as illustrated by computed tomography (Panel F, arrow). Three months after surgery, the dimensions of the right-sided cardiac chamber normalized (Panel G).

In this case, multimodal imaging established the diagnosis and enabled an exact description of the anatomy, function, and haemodynamics, which was essential for preoperative planning.