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Case 15: Congenitally Corrected Transposition of the Great Arteries¹

¹ From the Department of Radiology, University of California San Francisco, 505 Parnassus Ave, Box 0628, San Francisco, CA 94143-0628. Received July 1, 1998; revision requested August 13; revision received September 11; accepted January 7, 1999. G.P.R. supported in part by National Institutes of Health research training grant number T32-HL07570. Address reprint requests to G.R.C. (e-mail: Gary.Caputo@radiology.ucsf.edu).

Index terms: Aorta, abnormalities, 56.16 • Aorta, NR, 56.121411, 56.121412, 56.12142 • Children, cardiovascular system, 51.16, 56.16 • Diagnosis please • Heart, abnormalities, 51.14, 51.15, 51.16, 51.17 • Heart, MR, 51.121411, 51.121412, 51.12142 • Transposition of great vessels, 56.16 • Venae cavae, abnormalities, 56.16 • Venae cavae, MR, 56.121411, 56.121412, 56.12142

	HISTORY

TOP HISTORY IMAGING FINDINGS DISCUSSION References

 
An athletic 7-year-old boy who was acyanotic since birth developed progressively worsening shortness of breath during physical exertion. Chest radiographs (not shown) obtained during his infancy demonstrated dextrocardia but were otherwise unremarkable.

	IMAGING FINDINGS

TOP HISTORY IMAGING FINDINGS DISCUSSION References

 
The ascending aorta is anterior to and to the left of the main pulmonary artery (Fig 1a). The aorta arises from the ventricle that is positioned on the left (Fig 1a, 1b). This ventricle has a muscular outflow tract (infundibulum) (Fig 1b) that separates the atrioventricular and aortic valves, trabeculations along the ventricular septum, and a moderator band (Fig 1d), which are characteristic of a morphologic right ventricle. The main pulmonary artery originates from the ventricle located on the right (Fig 1a, 1b). This chamber has continuity of the atrioventricular and pulmonary valves without a muscular outflow tract (Fig 1b) and a smooth septal wall (Fig 1d), which are features of a morphologic left ventricle. A small ventricular septal defect is present (Fig 1c), and the myocardium of the morphologic right ventricle is thicker than that of the left ventricle, which is consistent with right ventricular hypertrophy (Fig 1d).

View larger version (117K): [in this window] [in a new window]   Figure 1a. Electrocardiographically gated axial spin-echo T1-weighted (697/20 [repetition time msec/echo time msec]) magnetic resonance (MR) images of the heart and upper abdomen. (a, b) The main pulmonary artery (PA) arises from the left ventricular outflow tract (arrow). Note that the right-sided atrioventricular valve is in continuity with the pulmonary valve, which is characteristic of the morphologic left ventricle (LV). The ventricular outflow tract with muscular walls is the infundibulum of the right ventricle. The aorta arises from the right ventricular outflow tract (RVO). The ascending aorta (A) is anterior to and to the left of the main pulmonary artery. These findings are diagnostic of levotransposition of the great arteries. The superior vena cava (S) drains into the right atrium (RA). (c) The right atrium (RA) connects to the morphologic left ventricle (LV; atrioventricular discordance). A small ventricular septal defect (arrow) is present. ap = left atrial appendage, RV = right ventricle. (d, e) Trabeculations along the septum (short straight arrow) and a moderator band (curved arrow) are present in one of the ventricles and distinguish it as the morphologic right ventricle (RV). The right ventricle is hypertrophied. The septal wall of the morphologic left ventricle (LV) is smooth. The inferior pulmonary veins (long straight arrows) drain into the left atrium (LA), which in turn connects to the morphologic right ventricle (atrioventricular discordance). Levotransposition in association with atrioventricular discordance represents congenitally corrected transposition. The inferior vena cava (I) joins the right atrium (RA). The liver (L) is on the right side, and the stomach (St) and spleen (Sp) are on the left, which indicates visceral situs solitus. In a-d, in the posterior lungs bilaterally, there is dependent atelectasis, which frequently occurs in children sedated for MR imaging.

View larger version (117K): [in this window] [in a new window]   Figure 1b. Electrocardiographically gated axial spin-echo T1-weighted (697/20 [repetition time msec/echo time msec]) magnetic resonance (MR) images of the heart and upper abdomen. (a, b) The main pulmonary artery (PA) arises from the left ventricular outflow tract (arrow). Note that the right-sided atrioventricular valve is in continuity with the pulmonary valve, which is characteristic of the morphologic left ventricle (LV). The ventricular outflow tract with muscular walls is the infundibulum of the right ventricle. The aorta arises from the right ventricular outflow tract (RVO). The ascending aorta (A) is anterior to and to the left of the main pulmonary artery. These findings are diagnostic of levotransposition of the great arteries. The superior vena cava (S) drains into the right atrium (RA). (c) The right atrium (RA) connects to the morphologic left ventricle (LV; atrioventricular discordance). A small ventricular septal defect (arrow) is present. ap = left atrial appendage, RV = right ventricle. (d, e) Trabeculations along the septum (short straight arrow) and a moderator band (curved arrow) are present in one of the ventricles and distinguish it as the morphologic right ventricle (RV). The right ventricle is hypertrophied. The septal wall of the morphologic left ventricle (LV) is smooth. The inferior pulmonary veins (long straight arrows) drain into the left atrium (LA), which in turn connects to the morphologic right ventricle (atrioventricular discordance). Levotransposition in association with atrioventricular discordance represents congenitally corrected transposition. The inferior vena cava (I) joins the right atrium (RA). The liver (L) is on the right side, and the stomach (St) and spleen (Sp) are on the left, which indicates visceral situs solitus. In a-d, in the posterior lungs bilaterally, there is dependent atelectasis, which frequently occurs in children sedated for MR imaging.

View larger version (114K): [in this window] [in a new window]   Figure 1c. Electrocardiographically gated axial spin-echo T1-weighted (697/20 [repetition time msec/echo time msec]) magnetic resonance (MR) images of the heart and upper abdomen. (a, b) The main pulmonary artery (PA) arises from the left ventricular outflow tract (arrow). Note that the right-sided atrioventricular valve is in continuity with the pulmonary valve, which is characteristic of the morphologic left ventricle (LV). The ventricular outflow tract with muscular walls is the infundibulum of the right ventricle. The aorta arises from the right ventricular outflow tract (RVO). The ascending aorta (A) is anterior to and to the left of the main pulmonary artery. These findings are diagnostic of levotransposition of the great arteries. The superior vena cava (S) drains into the right atrium (RA). (c) The right atrium (RA) connects to the morphologic left ventricle (LV; atrioventricular discordance). A small ventricular septal defect (arrow) is present. ap = left atrial appendage, RV = right ventricle. (d, e) Trabeculations along the septum (short straight arrow) and a moderator band (curved arrow) are present in one of the ventricles and distinguish it as the morphologic right ventricle (RV). The right ventricle is hypertrophied. The septal wall of the morphologic left ventricle (LV) is smooth. The inferior pulmonary veins (long straight arrows) drain into the left atrium (LA), which in turn connects to the morphologic right ventricle (atrioventricular discordance). Levotransposition in association with atrioventricular discordance represents congenitally corrected transposition. The inferior vena cava (I) joins the right atrium (RA). The liver (L) is on the right side, and the stomach (St) and spleen (Sp) are on the left, which indicates visceral situs solitus. In a-d, in the posterior lungs bilaterally, there is dependent atelectasis, which frequently occurs in children sedated for MR imaging.

View larger version (112K): [in this window] [in a new window]   Figure 1d. Electrocardiographically gated axial spin-echo T1-weighted (697/20 [repetition time msec/echo time msec]) magnetic resonance (MR) images of the heart and upper abdomen. (a, b) The main pulmonary artery (PA) arises from the left ventricular outflow tract (arrow). Note that the right-sided atrioventricular valve is in continuity with the pulmonary valve, which is characteristic of the morphologic left ventricle (LV). The ventricular outflow tract with muscular walls is the infundibulum of the right ventricle. The aorta arises from the right ventricular outflow tract (RVO). The ascending aorta (A) is anterior to and to the left of the main pulmonary artery. These findings are diagnostic of levotransposition of the great arteries. The superior vena cava (S) drains into the right atrium (RA). (c) The right atrium (RA) connects to the morphologic left ventricle (LV; atrioventricular discordance). A small ventricular septal defect (arrow) is present. ap = left atrial appendage, RV = right ventricle. (d, e) Trabeculations along the septum (short straight arrow) and a moderator band (curved arrow) are present in one of the ventricles and distinguish it as the morphologic right ventricle (RV). The right ventricle is hypertrophied. The septal wall of the morphologic left ventricle (LV) is smooth. The inferior pulmonary veins (long straight arrows) drain into the left atrium (LA), which in turn connects to the morphologic right ventricle (atrioventricular discordance). Levotransposition in association with atrioventricular discordance represents congenitally corrected transposition. The inferior vena cava (I) joins the right atrium (RA). The liver (L) is on the right side, and the stomach (St) and spleen (Sp) are on the left, which indicates visceral situs solitus. In a-d, in the posterior lungs bilaterally, there is dependent atelectasis, which frequently occurs in children sedated for MR imaging.

View larger version (113K): [in this window] [in a new window]   Figure 1e. Electrocardiographically gated axial spin-echo T1-weighted (697/20 [repetition time msec/echo time msec]) magnetic resonance (MR) images of the heart and upper abdomen. (a, b) The main pulmonary artery (PA) arises from the left ventricular outflow tract (arrow). Note that the right-sided atrioventricular valve is in continuity with the pulmonary valve, which is characteristic of the morphologic left ventricle (LV). The ventricular outflow tract with muscular walls is the infundibulum of the right ventricle. The aorta arises from the right ventricular outflow tract (RVO). The ascending aorta (A) is anterior to and to the left of the main pulmonary artery. These findings are diagnostic of levotransposition of the great arteries. The superior vena cava (S) drains into the right atrium (RA). (c) The right atrium (RA) connects to the morphologic left ventricle (LV; atrioventricular discordance). A small ventricular septal defect (arrow) is present. ap = left atrial appendage, RV = right ventricle. (d, e) Trabeculations along the septum (short straight arrow) and a moderator band (curved arrow) are present in one of the ventricles and distinguish it as the morphologic right ventricle (RV). The right ventricle is hypertrophied. The septal wall of the morphologic left ventricle (LV) is smooth. The inferior pulmonary veins (long straight arrows) drain into the left atrium (LA), which in turn connects to the morphologic right ventricle (atrioventricular discordance). Levotransposition in association with atrioventricular discordance represents congenitally corrected transposition. The inferior vena cava (I) joins the right atrium (RA). The liver (L) is on the right side, and the stomach (St) and spleen (Sp) are on the left, which indicates visceral situs solitus. In a-d, in the posterior lungs bilaterally, there is dependent atelectasis, which frequently occurs in children sedated for MR imaging.

This patient's cardiac apex is located on the right side, which indicates dextrocardia (Fig 1d). The liver is on the right side, and the stomach and spleen are on the left, which represents visceral situs solitus (Fig 1e).

The origin of the aorta from the right ventricle and of the pulmonary artery from the left ventricle indicates transposition of the great arteries, or ventriculoarterial discordance. The position of the aortic root to the left of and anterior to the main pulmonary artery signifies levotransposition. Connection of the right atrium to the morphologic left ventricle and of the left atrium to the morphologic right ventricle represents atrioventricular discordance. The combination of ventriculoarterial and atrioventricular discordance is diagnostic of congenitally corrected transposition of the great arteries (1) (Fig 2).

View larger version (43K): [in this window] [in a new window]   Figure 2. Diagram of the patient's cardiac anatomy. The patient has dextrocardia, with the cardiac apex on the right side. The left atrium (LA) connects to the morphologic right ventricle (RV), and the right atrium (RA) connects to the morphologic left ventricle (LV; atrioventricular discordance). The right ventricle gives rise to the aorta, and the left ventricle gives rise to the main pulmonary artery (transposition or ventriculoarterial discordance). The position of the ascending aorta (A) to the left of the main pulmonary artery (PA) indicates that this patient has levotransposition. The combination of ventriculoarterial discordance and atrioventricular discordance is diagnostic of congenitally corrected transposition of the great arteries. Note also the ventricular septal defect (arrow). (Adapted and reprinted, with permission, from reference 2. Reprinted by permission of Wiley-Liss, a division of John Wiley & Sons)

View larger version (144K): [in this window] [in a new window]   Figure 3a. Electrocardiographically gated MR images of the heart. (a) Oblique sagittal spin-echo T1-weighted (731/20) image shows subvalvular pulmonary stenosis (long arrow). Note also the supravalvular stenosis with a shelf (short arrow) in the main pulmonary artery (PA) at the sinotubular junction. LV = left ventricle. (b) Oblique sagittal cine gradient-echo (46/6; flip angle, 30°) image obtained during systole demonstrates a poststenotic jet (arrow) in the main pulmonary artery.

View larger version (157K): [in this window] [in a new window]   Figure 3b. Electrocardiographically gated MR images of the heart. (a) Oblique sagittal spin-echo T1-weighted (731/20) image shows subvalvular pulmonary stenosis (long arrow). Note also the supravalvular stenosis with a shelf (short arrow) in the main pulmonary artery (PA) at the sinotubular junction. LV = left ventricle. (b) Oblique sagittal cine gradient-echo (46/6; flip angle, 30°) image obtained during systole demonstrates a poststenotic jet (arrow) in the main pulmonary artery.

View larger version (124K): [in this window] [in a new window]   Figure 4a. Frames from cineangiography performed 6 years before MR imaging. (a) Frontal and (b) lateral projections of a left ventricular injection. Injection into the morphologic left ventricle (LV; located on the right) shows a ventricular septal defect (white arrow) with shunting from the left ventricle into the right ventricle. The walls of the left ventricle are smooth, which is characteristic of a morphologic left ventricle. The main pulmonary artery (PA) arises from the left ventricle. There is supravalvular pulmonary stenosis owing to a web (black arrow) and poststenotic dilatation of the main pulmonary artery. Mild subvalvular stenosis was also present but is not demonstrated on these frames. (c) Lateral projection of a right ventricular injection. The aorta arises from the morphologic right ventricle. Note the trabeculations of the right ventricular wall, which is a feature of a morphologic right ventricle (RV). A = ascending aorta.

View larger version (116K): [in this window] [in a new window]   Figure 4b. Frames from cineangiography performed 6 years before MR imaging. (a) Frontal and (b) lateral projections of a left ventricular injection. Injection into the morphologic left ventricle (LV; located on the right) shows a ventricular septal defect (white arrow) with shunting from the left ventricle into the right ventricle. The walls of the left ventricle are smooth, which is characteristic of a morphologic left ventricle. The main pulmonary artery (PA) arises from the left ventricle. There is supravalvular pulmonary stenosis owing to a web (black arrow) and poststenotic dilatation of the main pulmonary artery. Mild subvalvular stenosis was also present but is not demonstrated on these frames. (c) Lateral projection of a right ventricular injection. The aorta arises from the morphologic right ventricle. Note the trabeculations of the right ventricular wall, which is a feature of a morphologic right ventricle (RV). A = ascending aorta.

View larger version (108K): [in this window] [in a new window]   Figure 4c. Frames from cineangiography performed 6 years before MR imaging. (a) Frontal and (b) lateral projections of a left ventricular injection. Injection into the morphologic left ventricle (LV; located on the right) shows a ventricular septal defect (white arrow) with shunting from the left ventricle into the right ventricle. The walls of the left ventricle are smooth, which is characteristic of a morphologic left ventricle. The main pulmonary artery (PA) arises from the left ventricle. There is supravalvular pulmonary stenosis owing to a web (black arrow) and poststenotic dilatation of the main pulmonary artery. Mild subvalvular stenosis was also present but is not demonstrated on these frames. (c) Lateral projection of a right ventricular injection. The aorta arises from the morphologic right ventricle. Note the trabeculations of the right ventricular wall, which is a feature of a morphologic right ventricle (RV). A = ascending aorta.

	DISCUSSION

TOP HISTORY IMAGING FINDINGS DISCUSSION References

When atrioventricular discordance occurs in conjunction with ventriculoarterial discordance, the transposition is functionally corrected by ventricular inversion. Thus, this anomaly is known as congenitally corrected transposition of the great arteries. In this rare malformation, which has a reported prevalence of 0.4%–0.6% of all cases of congenital heart disease (1), the atrioventricular valves remain with their respective ventricles. The coronary arteries are inverted along with the ventricles: the morphologic right coronary artery is on the left side, and the morphologic left coronary artery is on the right side (1).

Approximately 99% of patients with corrected transposition have associated anomalies such as ventricular septal defect, pulmonary stenosis (subvalvular, valvular, or supravalvular), or systemic atrioventricular (tricuspid) valve abnormality such as dysplastic valve or Ebstein anomaly (4). Dextrocardia occurs in approximately 25% of patients (4). Because the right ventricle pumps the systemic circulation, most patients with corrected transposition of the great arteries have right ventricular hypertrophy, and many develop right-sided heart failure. A conduction abnormality frequently is present, and complete heart block develops in up to 30% of adolescents and adults with corrected transposition, which often necessitates implantation of an artificial cardiac pacemaker (4).

Echocardiography is the primary imaging method for the evaluation of congenital heart disease, in part because cardiologists are familiar with this technique and because it is widely available. Congenitally corrected transposition can be diagnosed at echocardiography by identifying atrioventricular discordance and ventriculoarterial discordance. In relation to the main pulmonary artery, the aortic root usually is anterior, to the left, and superior. In young children and infants, whose small size affords optimal acoustic windows, echocardiography has a sensitivity and a specificity approaching 100% for diagnosis of congenital heart disease, including corrected transposition (5,6). However, this technique is less accurate for the assessment of some abnormalities that are associated with congenitally corrected transposition, such as pulmonary stenosis and right ventricular hypertrophy and dysfunction (5,7). In addition, echocardiography may be limited in the evaluation of older children, adults, and patients who have undergone surgery (7,8).

In recent years, MR imaging has been used as a supplementary or alternative modality to delineate the cardiac morphology in patients with congenital heart disease (9–12). Cardiac MR imaging can depict the complex anatomy of the patient with congenitally corrected transposition and can be used to identify associated lesions in almost all cases (9,10). MR imaging can be used to distinguish between the morphologic right ventricle (muscular infundibulum separating the atrioventricular and semilunar valves, trabeculations, and moderator band) and the morphologic left ventricle (no muscular infundibulum, fibrous continuity of the atrioventricular and semilunar valves, and smooth walls) and between the right atrium (receives the inferior and superior venae cavae, triangular atrial appendage with wide ostium) and the left atrium (tubular atrial appendage with narrow ostium) (7). Thus, discordant atrioventricular and ventriculoarterial connections can be identified clearly on MR images.

MR imaging can be especially helpful for the evaluation of supracardiac structures, pulmonary stenosis, and complex abnormalities of the ventricles (7,8,13). As in other types of congenital heart disease, MR imaging also can be used to assess the volume, mass, and function of both ventricles, to evaluate valvular regurgitation, and to quantitate intracardiac shunts in patients with corrected transposition. (7,14).

Echocardiography and MR imaging in combination can be used to completely define the anatomy of most patients with congenitally corrected transposition. In the past, cineangiography was the primary technique for diagnosis of congenital heart disease. However, because it is invasive and requires the use of iodinated contrast media and ionizing radiation, cineangiography today should be reserved for situations in which echocardiography and MR imaging are nondiagnostic, MR imaging is contraindicated such as in patients with permanent cardiac pacemakers, pressure measurements are required, or an interventional procedure such as angioplasty is planned.

Patients who have either minor or no associated anomalies usually do not require surgical repair and may have near-normal life expectancy (4,15). Whenever possible, patients are treated conservatively because surgery poses a relatively high risk of complete heart block, morbidity, and mortality (4). If a patient is cyanotic or develops heart failure or severe pulmonary stenosis, cardiac surgery usually is performed. Intracardiac repair of congenitally corrected transposition currently entails a "double switch" procedure, in which the atrioventricular connections and the ventriculoarterial connections are interchanged. Associated abnormalities such as ventricular septal defect and pulmonary stenosis usually are corrected at the time of the double switch procedure (15).

Our congratulations to the 54 individuals who submitted the most likely diagnosis (congenitally corrected transposition of the great arteries) for Diagnosis Please, Case 15. The names and locations of the individuals, as submitted, are as follows:

1. Gholamali Afshang, MD, Tinley Park, Ill
   
2. Lionel Arrivé, MD, Paris, France
   
3. Charles Arteaga
   
4. William W. Atherton, DC, Chesterfield, Mo
   
5. Edward L. Baker, MD, San Francisco, Calif
   
6. Giuseppe Brancatelli, MD, Palermo, Italy
   
7. Daniel Chernoff, MD, PhD, Saratoga Springs, NY
   
8. Seyed A. Emamian, MD, PhD, Washington, DC
   
9. Keith D. Epperson, MD, Milwaukee, Wis
   
10. Akira Fujikawa, Tokyo, Japan
    
11. Douglas Gardner, Windsor, Ontario, Canada
    
12. Sidney Glanz, MD, Mineola, NY
    
13. Gaurav K. Goswami, MD, Forest Hills, NY
    
14. Catarina Holmqvist, MD, Lund, Sweden
    
15. Lowrey H. Holthaus, MD, Richmond, Va
    
16. Douglas S. Katz, MD, Mineola, NY
    
17. Nikolaos L. Kelekis, MD, Larissa, Greece
    
18. Mitchell A. Klein, MD, Milwaukee, Wis
    
19. Richard Krauthamer, MD, Rolling Hills, Calif
    
20. Louis S. Magagna, MD, Ann Arbor, Mich
    
21. Gildo Matta, MD, Cagliari, Italy
    
22. Edward Menges, MD, Aptos, Calif
    
23. Manabu Minami, MD, Tokyo, Japan
    
24. Hidetoshi Miyake, MD, Oita, Japan
    
25. Sergio J. Moguillansky, MD, Rio Negro, Argentina
    
26. Eduardo Mondello, MD, Buenos Aires, Argentina
    
27. Toshio Moritani, Rochester, NY
    
28. John G. Murray, FRCR, Dublin, Ireland
    
29. Anom Muthiah, MD, Mineola, NY
    
30. Al Nizzero, MD, Sudbury, Ontario, Canada
    
31. Vung Duy Nguyen, San Antonio, Tex
    
32. Victor Pérez-Candela, MD, Las Palmas, Spain
    
33. Anita P. Price, MD, Mineola, NY
    
34. Shawn P. Quillin, MD, Charlotte, NC
    
35. M. R. Ramakrishnan, MD, Big Stone Gap, Va
    
36. Enrique Remartinez Escobar, MD, Melilla, Spain
    
37. Dr. Duncan D. Royston, Hillcrest, South Africa
    
38. Michael Sadler, MD, New York, NY
    
39. Dr. Matthias Schmidt, Cologne, Germany
    
40. Dr. Bernhard Schulte, Cologne, Germany
    
41. Steven M. Schultz, MD, Fort Worth, Tex
    
42. Anthony J. Scuderi, MD, Johnstown, Pa
    
43. P. Siotto, MD, Cagliari, Italy
    
44. Paul Stark, MD, Stanford, Calif
    
45. Michael S. Stecker, MD, Iowa City, Iowa
    
46. J. Takasugi, Mercer Island, Wash
    
47. Shendee Teng, MD, Alhambra, Calif
    
48. John To, MD, Iron Mountain, Mich
    
49. Carlos E. Triana Rodriguez, Santafe de Bogota, Colombia
    
50. David A. Turner, MD, Chicago, Ill
    
51. Jonathan L. Williams, MD, Gainesville, Fla
    
52. H. Wouter van Es, MD, Nieuwegein, the Netherlands
    
53. Rolf Wyttenbach, MD, Bellinzona, Switzerland
    
54. Joe Yut, Olathe, Kan
    

	References

TOP HISTORY IMAGING FINDINGS DISCUSSION References

 

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