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Cardiac MRI and Pulmonary MR Angiography of Sinus Venosus Defect and Partial Anomalous Pulmonary Venous Connection in Cause of Right Undiagnosed Ventricular Enlargement

¹ Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, United Kingdom.
² Adult Congenital Heart Centre, Royal Brompton Hospital, London, United Kingdom.
³ National Heart and Lung Institute, Imperial College, London, United Kingdom.
⁴ Department of Radiology, Division of Cardiology, Queen's University Cardiovascular Laboratory, Kingston General Hospital, 76 Stuart St., Kingston, ON K7L 2V7, Canada.

View larger version (164K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A —23-year-old woman with partial anomalous pulmonary venous connections. Phase images of through-plane velocity maps for quantifying flow in pulmonary artery and aorta in this patient with two anomalous right pulmonary venous connections and secundum atrial septum defect (patient 37). PA = pulmonary artery, Ao = aorta.

View larger version (160K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B —23-year-old woman with partial anomalous pulmonary venous connections. Phase images of through-plane velocity maps for quantifying flow in pulmonary artery and aorta in this patient with two anomalous right pulmonary venous connections and secundum atrial septum defect (patient 37). PA = pulmonary artery, Ao = aorta.

View larger version (11K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C —23-year-old woman with partial anomalous pulmonary venous connections. Velocity-versus-time curves for blood flow in aorta and pulmonary artery. Qp/Qs = pulmonary-to-systemic blood flow ratio.

View larger version (94K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A —44-year-old man with sinus venosus defect. Transverse steady-state free precession cine frame (A) shows sinus venosus defect (arrow) between superior vena cava (asterisk) and left atrium (LA). Superior nature of this defect and absence of an upper rim of defect are evident in sagittal image (B). Ao = aorta, PA = pulmonary artery, RPA = right pulmonary artery, RA = right atrium.

View larger version (105K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B —44-year-old man with sinus venosus defect. Transverse steady-state free precession cine frame (A) shows sinus venosus defect (arrow) between superior vena cava (asterisk) and left atrium (LA). Superior nature of this defect and absence of an upper rim of defect are evident in sagittal image (B). Ao = aorta, PA = pulmonary artery, RPA = right pulmonary artery, RA = right atrium.

View larger version (132K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3A —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Transverse images in 32-year-old man with sinus venosus defect (patient 24). Steady-state free precession cine image (A) shows sinus venosus defect (black arrow) between left atrium (LA) and superior vena cava (SVC) (asterisk). Corresponding in-plane velocity flow map (B) shows dark inflow from pulmonary vein (white arrow) into LA crossing sinus venosus defect and entering SVC.

View larger version (145K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3B —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Transverse images in 32-year-old man with sinus venosus defect (patient 24). Steady-state free precession cine image (A) shows sinus venosus defect (black arrow) between left atrium (LA) and superior vena cava (SVC) (asterisk). Corresponding in-plane velocity flow map (B) shows dark inflow from pulmonary vein (white arrow) into LA crossing sinus venosus defect and entering SVC.

View larger version (136K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3C —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Sagittal images in 35-year-old woman with sinus venosus defect (patient 4). Steady-state free precession cine frame (C) shows superior nature of sinus venosus defect (arrow) between LA and SVC (asterisk). Corresponding in-plane velocity flow map (D) shows dark inflow from LA across sinus venosus defect (arrow) into right atrium (RA).

View larger version (149K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3D —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Sagittal images in 35-year-old woman with sinus venosus defect (patient 4). Steady-state free precession cine frame (C) shows superior nature of sinus venosus defect (arrow) between LA and SVC (asterisk). Corresponding in-plane velocity flow map (D) shows dark inflow from LA across sinus venosus defect (arrow) into right atrium (RA).

View larger version (108K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3E —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Coronal images in 18-year-old woman with sinus venosus defect (patient 20). FLASH image (E) shows bright flow disturbance in SVC (asterisk) related to flow through sinus venosus defect. Through-plane velocity flow map (F) in same position as Eshows sinus venosus defect as dark region of flow (arrow) from LA. Ao = aorta.

View larger version (143K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3F —Velocity flow mapping in three patients with sinus venosus defect. (See also Figs. S3A-S3F in supplemental data at www.ajronline.org). Coronal images in 18-year-old woman with sinus venosus defect (patient 20). FLASH image (E) shows bright flow disturbance in SVC (asterisk) related to flow through sinus venosus defect. Through-plane velocity flow map (F) in same position as Eshows sinus venosus defect as dark region of flow (arrow) from LA. Ao = aorta.

View larger version (119K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4A —46-year-old woman with partial anomalous pulmonary venous connection and sinus venosus defect. LA = left atrium, asterisk indicates superior vena cava (SVC). Turbo spin-echo image shows connection of right upper pulmonary vein (arrow) to SVC.

View larger version (124K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4B —46-year-old woman with partial anomalous pulmonary venous connection and sinus venosus defect. LA = left atrium, asterisk indicates superior vena cava (SVC). Slice 20 mm caudad to A clearly shows sinus venosus defect (arrow).

View larger version (124K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4C —46-year-old woman with partial anomalous pulmonary venous connection and sinus venosus defect. LA = left atrium, asterisk indicates superior vena cava (SVC). Steady-state free precession cine still images at same levels as Aand Balso show sinus venosus defect (arrow, D). Arrow in C indicates anomalous connection of upper pulmonary vein to SVC.

View larger version (132K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4D —46-year-old woman with partial anomalous pulmonary venous connection and sinus venosus defect. LA = left atrium, asterisk indicates superior vena cava (SVC). Steady-state free precession cine still images at same levels as Aand Balso show sinus venosus defect (arrow, D). Arrow in C indicates anomalous connection of upper pulmonary vein to SVC.

View larger version (118K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5A —57-year-old man with sinus venosus defect and right partial anomalous pulmonary venous connection. LA = left atrium, RA = right atrium, RPA = right pulmonary artery, RV = right ventricle. Oblique axial steady-state free precession cine image (A) shows anomalous connection of right upper pulmonary vein (arrowhead). Sinus venosus defect (arrow) is also evident at this level. Bright white signal in this velocity flow map (B) confirms flow from pulmonary vein into RA.

View larger version (163K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5B —57-year-old man with sinus venosus defect and right partial anomalous pulmonary venous connection. LA = left atrium, RA = right atrium, RPA = right pulmonary artery, RV = right ventricle. Oblique axial steady-state free precession cine image (A) shows anomalous connection of right upper pulmonary vein (arrowhead). Sinus venosus defect (arrow) is also evident at this level. Bright white signal in this velocity flow map (B) confirms flow from pulmonary vein into RA.

View larger version (144K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5C —57-year-old man with sinus venosus defect and right partial anomalous pulmonary venous connection. LA = left atrium, RA = right atrium, RPA = right pulmonary artery, RV = right ventricle. Coronal steady-state free precession cine image shows connection of right pulmonary vein to RA-superior vena cava junction (arrowhead).

View larger version (160K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5D —57-year-old man with sinus venosus defect and right partial anomalous pulmonary venous connection. LA = left atrium, RA = right atrium, RPA = right pulmonary artery, RV = right ventricle. Dark signal in this velocity flow map shows flow (arrowhead) from anomalous pulmonary vein into RA.

View larger version (113K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6A —Contrast-enhanced MR angiography maximum-intensity-projection coronal images in three patients with partial anomalous pulmonary venous connection. Ao = aorta, RA = right atrium. 18-year-old woman with anomalous connection of right pulmonary vein (arrow) to superior vena cava (SVC) (asterisk). PA = pulmonary artery.

View larger version (129K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6B —Contrast-enhanced MR angiography maximum-intensity-projection coronal images in three patients with partial anomalous pulmonary venous connection. Ao = aorta, RA = right atrium. 63-year-old woman with anomalous connection of left upper pulmonary vein to vertical vein (arrow) that drains into brachiocephalic vein (BV) and from there into SVC (asterisk). RPA = right pulmonary artery.

View larger version (159K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6C —Contrast-enhanced MR angiography maximum-intensity-projection coronal images in three patients with partial anomalous pulmonary venous connection. Ao = aorta, RA = right atrium. 45-year-old woman with anomalous scimitar vein. Image shows that all right pulmonary veins are connected to an anomalous vein (arrow) that, in turn, drains into inferior vena cava (IVC).

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