Case 3485

Magnetic Resonance Imaging of

Congenitally Corrected
Transposition of the Great Arteries
Published on 20.06.2005

DOI: 10.1594/EURORAD/CASE.3485
ISSN: 1563-4086
Section: Cardiovascular
Imaging Technique: MR
Imaging Technique: MR
Imaging Technique: MR
Case Type: Clinical Cases
Authors: Skoulikaris N, Kalioras V, Patouras P, Plotas A,
Lachanis S
Patient: 26 years, male

Clinical History:

Asymptomatic 26-year old male with a PA chest X-ray showing straightening of the left upper heart border and no
evidence of the pulmonary artery shadow.
Imaging Findings:

An asymptomatic 26 year old male was admitted to our hospital because of a pathologic routine chest radiograph
obtained for enrollment in army service. PA chest X-ray (fig.1)showed straightened left upper heart border with no
evidence of the pulmonary artery shadow. Echocardiography showed dextrocardia and set indications of probable
corrected transposition of the great vessels.Patient referred for Cardiac MRI for further evaluation. MRI showed that
the morphologic RV was on the left,the morphologic LV was on the right,the aorta originated from RV and was
anteriorly and to the left of the pulmonary artery (L-transposition),LA was connected to morphological RV and the
RA was connected to morphological LV (fig. 2a,2b,3,4). Thus,the combination of ventriculoarterial discordance and
atrioventricular discordance was diagnostic of corrected transposition of the great vessels. Further MRI evaluation
revealed a moderate valvular pulmonary stenosis with an estimated peak velocity 250 cm/sec,RV function analysis
showed ejection fraction 60%, Qp:Qs=1.There was mild hypertrophy of RV wall . There was no evidence of
ventricular septal defect or other anomalies.
Discussion:

Congenitally corrected transposition of the great arteries (ccTGA) or ventricular inversion is a rare form of congenital
heart disease, first described by Von Rokitansky in 1875, comprised of both atrioventricular and ventriculoarterial
discordance. The atrioventricular discordance implies that the right atrium (RA) enters the morphological left
ventricle (LV) and the left atrium (LA) drains into the morphological right ventricle (RV). The ventriculoarterial
discordance suggests that the RV gives rise to the aorta and the LV supplies the pulmonary circulation. It results
during the development of the embryonic heart tube at 4-6 weeks of intrauterine life from failure of the dividing
common truncus arteriosus, which gives rise to the aorta and the main pulmonary artery, to rotate and from bending
of the heart tube to the left, so-called l-looping. At the completion of this process, though the cardiac apex is
normally directed to the left, the morphological LV lies anterior and to the right of the posterior ventricle which is of
right morphology (RV) and they give rise posteriorly to the pulmonary trunk and anteriorly to the aorta respectively.
CCTGA has a reported prevalence of approximately 0.5% of all cases of congenital heart disease, is of unknown
cause and usually associated with a variety of other abnormalities and intra-cardiac defects, i.e. ventricular septal
defect (VSD) in 80%, pulmonic stenosis (PS) (usually subvulvar) in 50%, functional systemic abnormalities (usually
tricuspid regurgitation) in 1/3 and with some type of AV conduction abnormalities in 1/3 of cases. These additional
malformations will finally reflect to the patient's clinical presentation. A plain PA chest radiograph may suggest
corrected transposition of the great arteries in the absence of associated defects. Cardiac MRI is a supplementary or
alternative imaging technique to echocardiography and cardiac catheterization in diagnosis of complex cardiac
congenital anomalies. MRI can identify the morphologic features of ventricles and atria, can identify the
atrioventricular and ventriculoarterial connections, can evaluate associated cardiac anomalies, can diagnose and
quantify valvular stenoses and regurgitations, can assess the volume, mass and function of right ventricle. MRI is
particularly valuable in serial assessment of RV function as it can failure over years, because echo is inaccurate in
evaluation of the RV.
Differential Diagnosis List: Congenitally corrected transposition of the great arteries- levocardia

Final Diagnosis: Congenitally corrected transposition of the great arteries- levocardia

References:

Reddy G, Caputo G.
Congenitally corrected transposition of the great arteries.
Radiology 1999; 213: 102-106. (PMID: 10540648)
Park J H, Han C M, Kim C W.
MR imaging of Congenitally Corrected Transposition of the Great Vessels in Adults.
AJR Am J Roentgenol. 1989 Sep; 153(3): 491-494. (PMID: 2763945)
Figure 1
a

Description: PA chest X-ray shows straightening of the left upper heart border with no evidence of
pulmonary artery convexity (curved arrow) Origin:
Figure 2
a

Description: ECG-gated T1WI shows aorta (AO) in front of pulmonary artery (PA). Origin:
b

Description: The morphological RV (mRV) is at the left and the morphological LV (mLV) is at the right.
Origin:
Figure 3
a

Description: Sagittal sb-FFE shows aorta (AO) emanating from morphological RV (mRV) and mitroid
valve between left atrium (LA) and morphological RV Origin:
Figure 4
a

Description: Coronal sb-FFE shows the vertical position of the interventricular septum (arrow)Origin: