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Sinus Venosus Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return
Peter Hunold1, Thomas Bartel2, Jörg Barkhausen1
1Department for Diagnostic and Interventional Radiology, 2Division of Cardiology, Department of Internal Medicine, University Hospital Essen, Hufelandstrasse 55, 45122 Essen, Germany
A 74-year-old woman presented with progressive heart failure and central cyanosis during exercise. A transesophageal echocardiography study showed the superior vena cava (SVC) to override a large sinus venosus atrial septal defect (ASD) (Figure 1B). There were enlarged right heart chambers,tricuspid regurgitation,a pulmonary valve flow gradient,and mild pulmonary hypertension. A colour Doppler study showed a bi-directional flow over the defect.Catheterization oxymetry measured a left-to-right shunt of 74% with an increase in oxygen saturations between high (31%) and low SVC (74%).
Magnetic resonance imaging (MRI) confirmed the right heart enlargement and valve regurgitations. Phase contrast flow measurement in the ascending aorta (Ao) and main pulmonary trunk (PT) measured a left-to-right shunt of 68%. Figure 1A shows the volume rendering reformation,and Figure 1C the curved multiplanar reformation of the contrast-enhanced 3D MR angiography of the thoracic vessels. The 3D data set confirmed the anomalous pulmonary venous return (PAPVR) of a vessel into the SVC.The anomalous pulmonary vein (*)with a diameter of 1.5 cm draining the complete right upper lung love was typically connected to the SVC above the ASD and anterior to the right pulmonary artery (rPA) .Another smaller vessel (+) draining the middle lung lobe entered the LA close to the ASD. Three veins from the right lower lobe drained separately to the left atrium. Surgery confirmed these findings.
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