Congenitally corrected transposition of the great arteries
Congenitally corrected transposition of the great arteries, also known as levo- or L-loop transposition (L-TGA), is a rare cardiovascular anomaly with inversion of the ventricles and great arteries.
This anomaly comprises less than 1% of all congenital heart diseases 1,2,7.
Patients are usually asymptomatic when L-TGA is not associated with other anomalies or complications. Patients may present with congestive heart failure if there is an associated large ventricular septal defect (VSD) or with cyanosis if with associated VSD and pulmonary stenosis. If the anomaly is complicated by right ventricular or tricuspid valve dysfunction, patients can present with exertional dyspnea and easy fatigability.
In this anomaly, the right atrium communicates with the morphologic left ventricle, which gives rise to the pulmonary artery, while the left atrium communicates with the morphologic right ventricle, which gives rise to the aorta. Thus, atrioventricular and ventriculoarterial discordance (double discordance) exists, and although blood flows in the normal direction, it passes through the wrong ventricular chambers. It is also called L-TGA because the morphologic right ventricle is in the levoposition. The aorta is also usually anterior and to the left of the main pulmonary artery.
The presence or absence of associated cardiac anomalies alters the natural history of the anomaly. Patients may have one or more associated anomalies which include:
ventricular septal defect (VSD)
- occurs in approximately 70-80% of patients 2,7
- usually perimembranous in location
- large VSDs may result in congestive heart failure during infancy or childhood
- occurs in approximately 40-50% of patients 2,7
- commonly subvalvular in location
- patients with both a VSD and pulmonary stenosis may present with cyanosis
- pulmonary atresia
- systemic atrioventricular (tricuspid) valve abnormalities
- occurs in up to to 90% of patients 2
- inferior displacement of the valve closer to the cardiac apex
- abnormal conduction system
- unusual position and course of the AV node and bundle of His
- dual AV nodes
- complete AV block and paroxysmal supraventricular tachycardia
- mirror-image coronary artery distribution
- right coronary artery supplying the anterior descending branch and gives rise to a circumflex branch
- left coronary artery resembles a right coronary artery
- situs inversus
Chest radiographs may present with mesocardia or levocardia. Since there is an abnormal relationship of the great arteries, the vascular pedicle may appear abnormally straight. The left ventricular border may also appear more vertical than usual. In frontal radiographs, there could be an abnormal bulge along the upper left cardiac border produced by the inverted aorta and right ventricular outflow tract or by the right atrial appendage lying above the left atrial appendage (juxtapositioning of the atrial appendages).
The diagnosis of L-TGA can be made using the segmental approach. The associated anomalies can also be detected and quantified.
Allows direct visualisation of abnormal atrioventricular and ventriculoarterial relationships. Cardiac-gated cine CT can additionally assess function.
Cardiac MRI is helpful in defining the anomalous anatomy, ventricular function and volumes.
Treatment and prognosis
Early surgical treatment was aimed only at repairing the associated anomalies (i.e. VSD, pulmonary stenosis or atresia and tricuspid valve abnormalities). Despite these repairs, patients still develop tricuspid valve insufficiency and right ventricular failure which complicate the anomaly. This has led to the development of the double switch procedure as the definitive surgical correction and to remove the systemic pressure load from the morphologic right ventricle 3-5. Prior to correction, patients may require pulmonary artery banding to train the morphologic left ventricle to receive the higher systemic pressure load.
In cases with associated severe pulmonary stenosis or pulmonary atresia, a modified Blalock-Taussig shunt is necessary 7.
Some patients with conduction abnormalities may require pacemaker insertion 6,7.
History and etymology
Rokitansky coined the term "corrected transposition" in 1875 while Schiebler et al introduced the term "congenitally corrected transposition" in 1961 8. Although still commonly used in literature, in essence, both terms are misnomers due to the presence of double discordance in this anomaly.
- 1. Hornung TS, Calder L. Congenitally corrected transposition of the great arteries. Heart (British Cardiac Society). 96 (14): 1154-61. doi:10.1136/hrt.2008.150532 - Pubmed
- 2. Warnes CA. Transposition of the great arteries. Circulation. 114 (24): 2699-709. doi:10.1161/CIRCULATIONAHA.105.592352 - Pubmed
- 3. Gaca AM, Jaggers JJ, Dudley LT, Bisset GS. Repair of congenital heart disease: a primer-part 1. Radiology. 247 (3): 617-31. doi:10.1148/radiol.2473061909 - Pubmed
- 4. Imai Y. Double-switch operation for congenitally corrected transposition. Advances in cardiac surgery. 9: 65-86. Pubmed
- 5. Shin'oka T, Kurosawa H, Imai Y, Aoki M, Ishiyama M, Sakamoto T, Miyamoto S, Hobo K, Ichihara Y. Outcomes of definitive surgical repair for congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections: risk analyses in 189 patients. The Journal of thoracic and cardiovascular surgery. 133 (5): 1318-28, 1328.e1-4. doi:10.1016/j.jtcvs.2006.11.063 - Pubmed
- 6. Suhny Abbara, Thomas Gregory Walker, Steven G. Imbesi. Diagnostic Imaging. ISBN: 9781416033400
- 7. Myung Kun Park. Pediatric Cardiology for Practitioners. ISBN: 9780323046367
- 8. Karl TR. The role of the Fontan operation in the treatment of congenitally corrected transposition of the great arteries. Annals of pediatric cardiology. 4 (2): 103-10. doi:10.4103/0974-2069.84634 - Pubmed
Congenital heart disease
There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorised, it is helpful to have a working understanding of normal and fetal circulation, as well as an understanding of the segmental approach to imaging in congenital heart disease.
congenital heart disease
- normal relationship between chambers and valves
- atrioventricular valves
- outflow tract
- great vessels
- venous inflow
- anomalous valves
- abnormal relationship of chambers and valves
- atrioventricular abnormality
- great vessel connection abnormality
- conotruncal cardiac anomalies
- pentalogy of Cantrell
- Shone syndrome
- congenital heart disease - chest x-ray approach
surgical repairs (mnemonic)
- arterial switch procedure
- Blalock-Taussig shunt
- double switch procedure
- Fontan procedure
- Glenn procedure
- Mustard repair
- Norwood procedure
- Pott shunt
- pulmonary artery banding
- Rastelli procedure
- Sano shunt
- Senning repair
- total repair of tetralogy of Fallot (TOF)
- unifocalisation procedure
- Waterston shunt
- acute aortic syndrome
- thoracic aortic aneurysm
- abdominal aortic aneurysm
- endovascular aneurysm repair (EVAR)
- reporting tips for aortic aneurysms
- aortic coarctation
- aortic pseudocoarctation
- cervical aortic arch
- interrupted aortic arch
- transposition of the great arteries
- variant anatomy of the aortic arch
- traumatic aortic injuries