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CT of Congenital Heart Disease: Normal Anatomy and Typical Pathologic Conditions¹

¹ From the Departments of Radiology (H.W.G., C.H.Y.), Pediatric Cardiology (I.S.P., J.K.K., Y.H.K.), and Pediatric Cardiac Surgery (D.M.S., T.J.Y., J.J.P.), Asan Medical Center, University of Ulsan College of Medicine, 388–1 Pungnap-2 dong, Songpa-gu, 138–736 Seoul, Korea. Recipient of a Magna Cum Laude award for an education exhibit at the 2002 RSNA scientific assembly. Received January 23, 2003; revision requested March 11 and received March 18; accepted April 3. Address correspondence to H.W.G. (e-mail: hwgoo@amc.seoul.kr).

View larger version (162K): [in a new window]   Figure 1.  Normal aorta. CT scan shows the ascending aorta (aA), aortic arch (Ar), and descending aorta (dA). The aortic root (Ro) represents the proximal portion of the ascending aorta, and the aortic isthmus (Is) represents the distal portion of the aortic arch.

View larger version (133K): [in a new window]   Figure 2a.  Normal pulmonary arteries. Curved planar reformatted images show the right (a) and left (b) pulmonary arteries, along with the outlet portion of the right ventricle and the main pulmonary artery.

View larger version (130K): [in a new window]   Figure 2b.  Normal pulmonary arteries. Curved planar reformatted images show the right (a) and left (b) pulmonary arteries, along with the outlet portion of the right ventricle and the main pulmonary artery.

View larger version (125K): [in a new window]   Figure 3.  Normal pulmonary veins. CT scan shows all the pulmonary veins, including the right superior (RS), right inferior (RI), left superior (LS), and left inferior (LI) pulmonary veins, with a normal venoatrial connection with the left atrium.

View larger version (132K): [in a new window]   Figure 4a.  Normal cardiac chambers. (a) CT scan of the right cardiac chambers demonstrates a muscular structure (arrowheads) between the tricuspid valve (TV) and the pulmonary valve (PV). The right ventricle shows coarse trabeculae. (b) CT scan of the left cardiac chambers demonstrates fibrous continuity (arrowhead) between the mitral valve (MV) and the aortic valve (AV). The left ventricle shows fine trabeculae. (c) CT scan shows the atrial appendages with a characteristic appearance. The right atrial appendage (RAA) is triangular with a wide opening, whereas the left atrial appendage (LAA) is narrow and fingerlike. (d) CT scan shows the crista (sulcus) terminalis (arrowhead), an anatomic landmark between the right atrium proper and the right atrial appendage.

View larger version (138K): [in a new window]   Figure 4b.  Normal cardiac chambers. (a) CT scan of the right cardiac chambers demonstrates a muscular structure (arrowheads) between the tricuspid valve (TV) and the pulmonary valve (PV). The right ventricle shows coarse trabeculae. (b) CT scan of the left cardiac chambers demonstrates fibrous continuity (arrowhead) between the mitral valve (MV) and the aortic valve (AV). The left ventricle shows fine trabeculae. (c) CT scan shows the atrial appendages with a characteristic appearance. The right atrial appendage (RAA) is triangular with a wide opening, whereas the left atrial appendage (LAA) is narrow and fingerlike. (d) CT scan shows the crista (sulcus) terminalis (arrowhead), an anatomic landmark between the right atrium proper and the right atrial appendage.

View larger version (134K): [in a new window]   Figure 4c.  Normal cardiac chambers. (a) CT scan of the right cardiac chambers demonstrates a muscular structure (arrowheads) between the tricuspid valve (TV) and the pulmonary valve (PV). The right ventricle shows coarse trabeculae. (b) CT scan of the left cardiac chambers demonstrates fibrous continuity (arrowhead) between the mitral valve (MV) and the aortic valve (AV). The left ventricle shows fine trabeculae. (c) CT scan shows the atrial appendages with a characteristic appearance. The right atrial appendage (RAA) is triangular with a wide opening, whereas the left atrial appendage (LAA) is narrow and fingerlike. (d) CT scan shows the crista (sulcus) terminalis (arrowhead), an anatomic landmark between the right atrium proper and the right atrial appendage.

View larger version (129K): [in a new window]   Figure 4d.  Normal cardiac chambers. (a) CT scan of the right cardiac chambers demonstrates a muscular structure (arrowheads) between the tricuspid valve (TV) and the pulmonary valve (PV). The right ventricle shows coarse trabeculae. (b) CT scan of the left cardiac chambers demonstrates fibrous continuity (arrowhead) between the mitral valve (MV) and the aortic valve (AV). The left ventricle shows fine trabeculae. (c) CT scan shows the atrial appendages with a characteristic appearance. The right atrial appendage (RAA) is triangular with a wide opening, whereas the left atrial appendage (LAA) is narrow and fingerlike. (d) CT scan shows the crista (sulcus) terminalis (arrowhead), an anatomic landmark between the right atrium proper and the right atrial appendage.

View larger version (113K): [in a new window]   Figure 5.  Normal bronchial branching pattern. Coronal multiplanar reformatted image depicts a normal bronchial branching pattern. Note the mild indentation of the trachea by the left aortic arch (Ar). The relationships between the upper lobe bronchi and the pulmonary arteries are well seen. LPA = left pulmonary artery, LUL = left upper lobe, RPA = right pulmonary artery, RUL = right upper lobe.

View larger version (115K): [in a new window]   Figure 6a.  Normal coronary arteries. (a) On a CT scan, the right coronary artery (RCA) and left coronary artery (LCA) arise from the right (R) and left (L) aortic sinuses, respectively. The origin of the left coronary artery is higher than that of the right coronary artery. (b) CT scan shows how the left coronary artery (LCA) is divided into the left anterior descending artery (LAD) and the left circumflex artery (LCX). The right coronary artery (RCA) is seen in the plane that crosses the atrioventricular groove. CT delineates the proximal portions of the coronary arteries more frequently than the distal portions and the left coronary artery more frequently than the right coronary artery.

View larger version (119K): [in a new window]   Figure 6b.  Normal coronary arteries. (a) On a CT scan, the right coronary artery (RCA) and left coronary artery (LCA) arise from the right (R) and left (L) aortic sinuses, respectively. The origin of the left coronary artery is higher than that of the right coronary artery. (b) CT scan shows how the left coronary artery (LCA) is divided into the left anterior descending artery (LAD) and the left circumflex artery (LCX). The right coronary artery (RCA) is seen in the plane that crosses the atrioventricular groove. CT delineates the proximal portions of the coronary arteries more frequently than the distal portions and the left coronary artery more frequently than the right coronary artery.

View larger version (111K): [in a new window]   Figure 7a.  Normal valves. (a) CT scan shows the aortic valve with right (R), left (L), and noncoronary (N) cusps and the pulmonary valve with right (R), left (L), and anterior (A) cusps. (b) CT scan shows the tricuspid valve with septal (S), anterior (A), and posterior (P) leaflets and the mitral valve with aortic (A) and mural (M) leaflets.

View larger version (128K): [in a new window]   Figure 7b.  Normal valves. (a) CT scan shows the aortic valve with right (R), left (L), and noncoronary (N) cusps and the pulmonary valve with right (R), left (L), and anterior (A) cusps. (b) CT scan shows the tricuspid valve with septal (S), anterior (A), and posterior (P) leaflets and the mitral valve with aortic (A) and mural (M) leaflets.

View larger version (121K): [in a new window]   Figure 8a.  (a) Type A1 IAA. VR image (posterior view) shows the left aortic arch with aortic interruption just distal to the left subclavian artery (arrows). (b) Type B3 IAA. VR image (left posterior oblique view) shows interruption between the left common carotid artery and the left subclavian artery (arrowheads). An isolated right subclavian artery is also seen (arrow).

View larger version (70K): [in a new window]   Figure 8b.  (a) Type A1 IAA. VR image (posterior view) shows the left aortic arch with aortic interruption just distal to the left subclavian artery (arrows). (b) Type B3 IAA. VR image (left posterior oblique view) shows interruption between the left common carotid artery and the left subclavian artery (arrowheads). An isolated right subclavian artery is also seen (arrow).

View larger version (106K): [in a new window]   Figure 9.  Coarctation of the aorta. VR image shows diffuse narrowing of the aortic arch and isthmus (tubular hypoplasia). A posterior indentation is seen at the level of the ductus arteriosus (arrowhead).

View larger version (140K): [in a new window]   Figure 10a.  (a) Truncus arteriosus. Combined CT scan and VR image show the truncus arteriosus (TA), from which the ascending aorta (aA) and pulmonary trunk (PT) arise. (b) Aortopulmonary window. VR image shows a distal aortopulmonary window (*), through which the ascending aorta (aA) and pulmonary trunk (PT) communicate with each other.

View larger version (130K): [in a new window]   Figure 10b.  (a) Truncus arteriosus. Combined CT scan and VR image show the truncus arteriosus (TA), from which the ascending aorta (aA) and pulmonary trunk (PT) arise. (b) Aortopulmonary window. VR image shows a distal aortopulmonary window (*), through which the ascending aorta (aA) and pulmonary trunk (PT) communicate with each other.

View larger version (124K): [in a new window]   Figure 11a.  Anomalous origin of one pulmonary artery from the ascending aorta. CT scans show anomalous origin of the right pulmonary artery (RPA), which arises from the ascending aorta (aA). In b, the left pulmonary artery (LPA) arises from the main pulmonary artery (MPA).

View larger version (93K): [in a new window]   Figure 11b.  Anomalous origin of one pulmonary artery from the ascending aorta. CT scans show anomalous origin of the right pulmonary artery (RPA), which arises from the ascending aorta (aA). In b, the left pulmonary artery (LPA) arises from the main pulmonary artery (MPA).

View larger version (103K): [in a new window]   Figure 12a.  Pulmonary artery sling. (a) On a CT scan, the left pulmonary artery (*) originates aberrantly from the proximal right pulmonary artery and crosses the midline between the trachea (arrow) and the esophagus (arrowhead). An associated tracheal ring is also seen. (b) VR image (posterior view) shows the characteristic course of the aberrant left pulmonary artery (*).

View larger version (139K): [in a new window]   Figure 12b.  Pulmonary artery sling. (a) On a CT scan, the left pulmonary artery (*) originates aberrantly from the proximal right pulmonary artery and crosses the midline between the trachea (arrow) and the esophagus (arrowhead). An associated tracheal ring is also seen. (b) VR image (posterior view) shows the characteristic course of the aberrant left pulmonary artery (*).

View larger version (146K): [in a new window]   Figure 13.  PDA. CT scan shows a PDA (arrowheads) between the proximal descending aorta (dA) and the roof of the main pulmonary artery (MPA).

View larger version (117K): [in a new window]   Figure 14.  Total anomalous pulmonary venous connection. CT scan obtained with subvolume MIP shows a supracardiac type of total anomalous pulmonary venous connection with obstruction (arrowheads). All pulmonary veins are connected to the left brachiocephalic vein via the vertical vein (*).

View larger version (135K): [in a new window]   Figure 15.  Partial anomalous pulmonary venous connection. CT scan shows an anomalous connection between the right superior pulmonary vein and the SVC (arrowheads).

View larger version (135K): [in a new window]   Figure 16.  Left SVC. CT scan shows a left SVC (*) connected to the right atrium (RA) via a dilated coronary sinus (CS).

View larger version (109K): [in a new window]   Figure 17.  Coronary artery anomaly. Oblique axial CT scan shows an anomalous right coronary artery (arrowheads) that arises from the left aortic sinus and courses between the ascending aorta (aA) and the pulmonary trunk (PT). An anomalous coronary artery may be compressed between the great arteries, a condition that may lead to myocardial ischemia or infarction.

View larger version (127K): [in a new window]   Figure 18a.  Tetralogy of Fallot. (a) CT scan shows a right ventricular outlet obstruction due to hypertrophy and anterosuperior displacement of the infundibular septum (arrows) and to hypertrophy of the anterior muscle bundles (*). (b) On an MIP image (right anterior oblique view) segmented for the right ventricle and pulmonary artery, the hypertrophic anterior muscle bundles (arrows) are seen to encroach on the right ventricular outflow tract, a finding that is best seen on this view.

View larger version (77K): [in a new window]   Figure 18b.  Tetralogy of Fallot. (a) CT scan shows a right ventricular outlet obstruction due to hypertrophy and anterosuperior displacement of the infundibular septum (arrows) and to hypertrophy of the anterior muscle bundles (*). (b) On an MIP image (right anterior oblique view) segmented for the right ventricle and pulmonary artery, the hypertrophic anterior muscle bundles (arrows) are seen to encroach on the right ventricular outflow tract, a finding that is best seen on this view.

View larger version (123K): [in a new window]   Figure 19a.  Pulmonary atresia with VSD. LPA = left pulmonary artery, RPA = right pulmonary artery. (a) CT scan shows the confluent portion of the central pulmonary artery (arrowheads). (b) CT scan shows absence of the confluent portion of the central pulmonary artery (*). Each pulmonary artery was supplied by a corresponding PDA (not shown). (c) CT scans depict the major aortopulmonary collateral vessels that supply the branch pulmonary arteries. These collateral vessels arise from the descending thoracic aorta (dA).

View larger version (111K): [in a new window]   Figure 19b.  Pulmonary atresia with VSD. LPA = left pulmonary artery, RPA = right pulmonary artery. (a) CT scan shows the confluent portion of the central pulmonary artery (arrowheads). (b) CT scan shows absence of the confluent portion of the central pulmonary artery (*). Each pulmonary artery was supplied by a corresponding PDA (not shown). (c) CT scans depict the major aortopulmonary collateral vessels that supply the branch pulmonary arteries. These collateral vessels arise from the descending thoracic aorta (dA).

View larger version (135K): [in a new window]   Figure 19c.  Pulmonary atresia with VSD. LPA = left pulmonary artery, RPA = right pulmonary artery. (a) CT scan shows the confluent portion of the central pulmonary artery (arrowheads). (b) CT scan shows absence of the confluent portion of the central pulmonary artery (*). Each pulmonary artery was supplied by a corresponding PDA (not shown). (c) CT scans depict the major aortopulmonary collateral vessels that supply the branch pulmonary arteries. These collateral vessels arise from the descending thoracic aorta (dA).

View larger version (104K): [in a new window]   Figure 20.  Ebstein anomaly. Oblique axial CT scan shows apical displacement of the septal and posterior leaflets of the tricuspid valve (arrowhead) from the atrioventricular junction (arrows). Note the dilated right atrium (RA), atrialized right ventricle (ARV), and functional right ventricle (FRV). Clockwise rotation of the heart and posterior bulging of the ventricles are also seen.

View larger version (89K): [in a new window]   Figure 21.  Tricuspid atresia. CT scan shows fatty tissue (arrowheads) between the right atrium (RA) and the right ventricle (RV), which prevents any direct connection between the two compartments. This case represents the more common type of tricuspid atresia.

View larger version (94K): [in a new window]   Figure 22a.  VSD. CT scans reformatted along the interventricular septum provide en face views. (a) Normal interventricular septum. (b-e) Various types of VSDs (*). (b) Large perimembranous defect (arrowheads), which typically abuts the fibrous continuity between the tricuspid and mitral valves and the aortic valve ("central fibrous body"). (c) Perimembranous defect with inlet extension (arrowheads). (d) Doubly committed juxtaarterial defect (arrowheads) immediately below the aortic and pulmonary valves. (e) Restrictive defect ("bulboventricular foramen") (arrowheads) in the double inlet ventricle.

View larger version (108K): [in a new window]   Figure 22b.  VSD. CT scans reformatted along the interventricular septum provide en face views. (a) Normal interventricular septum. (b-e) Various types of VSDs (*). (b) Large perimembranous defect (arrowheads), which typically abuts the fibrous continuity between the tricuspid and mitral valves and the aortic valve ("central fibrous body"). (c) Perimembranous defect with inlet extension (arrowheads). (d) Doubly committed juxtaarterial defect (arrowheads) immediately below the aortic and pulmonary valves. (e) Restrictive defect ("bulboventricular foramen") (arrowheads) in the double inlet ventricle.

View larger version (102K): [in a new window]   Figure 22c.  VSD. CT scans reformatted along the interventricular septum provide en face views. (a) Normal interventricular septum. (b-e) Various types of VSDs (*). (b) Large perimembranous defect (arrowheads), which typically abuts the fibrous continuity between the tricuspid and mitral valves and the aortic valve ("central fibrous body"). (c) Perimembranous defect with inlet extension (arrowheads). (d) Doubly committed juxtaarterial defect (arrowheads) immediately below the aortic and pulmonary valves. (e) Restrictive defect ("bulboventricular foramen") (arrowheads) in the double inlet ventricle.

View larger version (112K): [in a new window]   Figure 22d.  VSD. CT scans reformatted along the interventricular septum provide en face views. (a) Normal interventricular septum. (b-e) Various types of VSDs (*). (b) Large perimembranous defect (arrowheads), which typically abuts the fibrous continuity between the tricuspid and mitral valves and the aortic valve ("central fibrous body"). (c) Perimembranous defect with inlet extension (arrowheads). (d) Doubly committed juxtaarterial defect (arrowheads) immediately below the aortic and pulmonary valves. (e) Restrictive defect ("bulboventricular foramen") (arrowheads) in the double inlet ventricle.

View larger version (117K): [in a new window]   Figure 22e.  VSD. CT scans reformatted along the interventricular septum provide en face views. (a) Normal interventricular septum. (b-e) Various types of VSDs (*). (b) Large perimembranous defect (arrowheads), which typically abuts the fibrous continuity between the tricuspid and mitral valves and the aortic valve ("central fibrous body"). (c) Perimembranous defect with inlet extension (arrowheads). (d) Doubly committed juxtaarterial defect (arrowheads) immediately below the aortic and pulmonary valves. (e) Restrictive defect ("bulboventricular foramen") (arrowheads) in the double inlet ventricle.

View larger version (113K): [in a new window]   Figure 23.  Atrioventricular septal defect. CT scan shows an atrioventricular septal defect (*) and a free-floating common atrioventricular valve with a bridging leaflet (arrowheads) that involves the adjacent atrial and ventricular septa as well as the atrioventricular septum. Dextrocardia and a right descending aorta are also noted.

View larger version (169K): [in a new window]   Figure 24.  Complete transposition of the great arteries. On a CT scan, the ascending aorta (aA) arises from the right ventricle and courses anterior to the pulmonary trunk (PT), which arises from the left ventricle. Aortic coarctation is also seen. LT = left cardiac chamber, RT = right cardiac chamber.

View larger version (151K): [in a new window]   Figure 25a.  Congenitally corrected transposition of the great arteries. (a) On a CT scan, the pulmonary trunk (PT) arises from the left ventricle (LV), which is connected to the right atrium (RA). RV = right ventricle. (b) CT scan shows that the ascending aorta (aA) arises from the right ventricle (RV), which is connected to the left atrium (LA). The ascending aorta courses anterior to and to the left of the pulmonary trunk (PT).

View larger version (136K): [in a new window]   Figure 25b.  Congenitally corrected transposition of the great arteries. (a) On a CT scan, the pulmonary trunk (PT) arises from the left ventricle (LV), which is connected to the right atrium (RA). RV = right ventricle. (b) CT scan shows that the ascending aorta (aA) arises from the right ventricle (RV), which is connected to the left atrium (LA). The ascending aorta courses anterior to and to the left of the pulmonary trunk (PT).

View larger version (165K): [in a new window]   Figure 26.  Double outlet right ventricle. CT scan shows that both the ascending aorta (aA) and the pulmonary trunk (PT) arise from the right ventricle (RV). LV = left ventricle.

View larger version (125K): [in a new window]   Figure 27a.  Right atrial isomerism. (a) CT scan shows both atria with morphologic characteristics of the right atrial appendage (ie, triangular with a wide opening). mRA = morphologic right atrium. (b) On an oblique coronal CT scan, the upper lobe bronchi (UL) are above the descending lower pulmonary arteries (PA). Both main bronchi are symmetrically short. (c) Sagittal reformatted image shows that the inferior vena cava (IVC) and the abdominal aorta (dA) run along the same side of the spine (juxtaposition of the IVC and the abdominal aorta), with the vein in an anterolateral position.

View larger version (89K): [in a new window]   Figure 27b.  Right atrial isomerism. (a) CT scan shows both atria with morphologic characteristics of the right atrial appendage (ie, triangular with a wide opening). mRA = morphologic right atrium. (b) On an oblique coronal CT scan, the upper lobe bronchi (UL) are above the descending lower pulmonary arteries (PA). Both main bronchi are symmetrically short. (c) Sagittal reformatted image shows that the inferior vena cava (IVC) and the abdominal aorta (dA) run along the same side of the spine (juxtaposition of the IVC and the abdominal aorta), with the vein in an anterolateral position.

View larger version (175K): [in a new window]   Figure 27c.  Right atrial isomerism. (a) CT scan shows both atria with morphologic characteristics of the right atrial appendage (ie, triangular with a wide opening). mRA = morphologic right atrium. (b) On an oblique coronal CT scan, the upper lobe bronchi (UL) are above the descending lower pulmonary arteries (PA). Both main bronchi are symmetrically short. (c) Sagittal reformatted image shows that the inferior vena cava (IVC) and the abdominal aorta (dA) run along the same side of the spine (juxtaposition of the IVC and the abdominal aorta), with the vein in an anterolateral position.

View larger version (106K): [in a new window]   Figure 28a.  Left atrial isomerism. (a) On a CT scan, both atria have a long appendage with a narrow opening and therefore represent morphologic left atria (mLA). (b) On an oblique coronal CT scan, the upper lobe bronchi (UL) are below the descending lower pulmonary arteries (PA). Both main bronchi are symmetrically long. (c) Coronal reformatted image shows that the inferior vena cava (IVC) is continuous with the azygos (AZ)-hemiazygos (HZ) vein on the same side. Note the bilateral superior and inferior venae cavae. The descending aorta (dA) runs between two parallel veins.

View larger version (125K): [in a new window]   Figure 28b.  Left atrial isomerism. (a) On a CT scan, both atria have a long appendage with a narrow opening and therefore represent morphologic left atria (mLA). (b) On an oblique coronal CT scan, the upper lobe bronchi (UL) are below the descending lower pulmonary arteries (PA). Both main bronchi are symmetrically long. (c) Coronal reformatted image shows that the inferior vena cava (IVC) is continuous with the azygos (AZ)-hemiazygos (HZ) vein on the same side. Note the bilateral superior and inferior venae cavae. The descending aorta (dA) runs between two parallel veins.

View larger version (148K): [in a new window]   Figure 28c.  Left atrial isomerism. (a) On a CT scan, both atria have a long appendage with a narrow opening and therefore represent morphologic left atria (mLA). (b) On an oblique coronal CT scan, the upper lobe bronchi (UL) are below the descending lower pulmonary arteries (PA). Both main bronchi are symmetrically long. (c) Coronal reformatted image shows that the inferior vena cava (IVC) is continuous with the azygos (AZ)-hemiazygos (HZ) vein on the same side. Note the bilateral superior and inferior venae cavae. The descending aorta (dA) runs between two parallel veins.