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Number 15-11: CMR is the Technique to Evaluate Anatomically Corrected...
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Number 15-11: Cardiac magnetic resonance is the technique to evaluate anatomically corrected malposition of the great arteries

Case from: Lamia Ait-Ali*§,MD, PhD,  Bruno Murzi†, MD, MD, Pierluigi Festa§ ‡, MD

Institute: *Institute of Clinical Physiology, CNR, Massa-Pisa, Italy, § Pediatric Cardiology and GUCH unit, †Pediatric cardiac surgery, Heart hospital, Fondazione G. Monasterio-CNR, Regione Toscana, Massa, Italy. ‡ MRI Laboratory Fondazione G.  Monasterio-CNR, Regione Toscana Pisa, Italy 

Clinical history:

A symptomatic 50 year old woman previously diagnosed as congenitally corrected transposition of great arteries (L-TGA) was referred to our department. She was overweight and  complained shortness of breath  and palpitations.

Basal echocardiogram showed a situs solitus, levocardia, a small restrictive ventricular septal defect (VSD), associated to a mild sub-pulmonary stenosis and a severe sub-aortic tunnel –like stenosis with the aorta lying anteriorly and to the left of the pulmonary artery and finally two hypertrophied ventricles; however because of a bad acoustic window, the AV valve and ventricles morphology were not well identified. She was scheduled for Cardiac Magnetic Resonance (CMR) evaluation.

CMR Finding:

At CMR the following features were found: -the left side sided AV valve was bileaflet , therefore consistent with a mitral valve (Fig. 1; movie 1,2) and a morphological tricuspid valve was identified on the right side (atrio-ventricular concordance)


Figure 1


Movie 1


Movie 2

Fig 1: Still image and cine SSFP short axis ventricle (movie 1), 4 chamber view (movie 2): typical mitral valve (white arrow) connected to a posterior left ventricle. LA: left atrium; LV: Left ventricle.

-a sub-pulmonary VSD  (Fig. 2) leading to a left-to-right shunt with QP/QS evaluated as 2; 

Fig 2: Black blood MR of right ventricle outflow tract showing a sub-pulmonary ventricular septal defect

VSD: ventricular septal defect; PA: pulmonary artery.

- both great arteries abnormally arising above the appropriate ventricles (ventriculo-arterial concordance)  with conal tissue beneath each great artery, resulting in aortic-mitral and pulmonary-tricuspid discontinuity; the aorta arises form the posterior hypertrophic left ventricle (LV) through a stenotic fibro-muscular tunnel, lying anterior and to the left of the pulmonary artery ; the last arises from the anterior RV with a mild sub-valvular stenosis (Fig 3, 4; movies 3,4,5). 


Fig 3, movie 3: Cine SSFP showing anterior aorta arising form the posterior LV through a stenotic fibromuscular tunnel (white arrow)

AO: aorta; LV: Left ventricle.

Fig 4, movie 4:  Sagittal Cine SSFP showing anterior aorta arising form the posterior LV and a posterior pulmonary artery arising from the anterior RV

AO: aorta; LA: left atrium; LV: left ventricle; PA: pulmonary artery.

Movie 5:  Axial stack showing the relations of the ventricles to the corresponding great vessels.

Conclusion :  CMR diagnosis was Anatomically corrected malposition of the great arteries (ACMGA) -  VSD- sub aortic and sub pulmonary stenosis.  A cardiac catheterization confirmed the severe sub-aortic stenosis with a LV-Ao gradient of 60 mmHg, moderate sub-pulmonary stenosis (RV-PA gradient 30 mmHg), and a restricted VSD with QP/QS = 2.  Surgical report confirmed the CMR diagnosis, therefore bilateral miectomy to relieve LVOT and sub-pulmonary obstruction was performed and the VSD was also closed by means of an autologous treated pericardial patch. Post–op CMR (Fig 5) illustrates the good surgical result. 



Fig 6: Post operative CMR

A) Cine SSFP left outflow tract

B)  Cine SSFP right outflow tract

AO: aorta; LV: Left ventricle; PA: pulmonary artery: RV: right ventricle. 

Perspective: CMR was critical to obtain the correct diagnosis of ACMGA, restrictive VSD, subaortic and subpulmonary stenosis.  ACMGA is a very rare form of congenital heart disease in which the great arteries arise above the anatomically correct ventricles but are abnormally related to the ventricles and to each other [1,2].  Subaortic stenosis occurs in about 15% of ACMGA [3].  ACMGA is frequently misdiagnosed  as congenitally corrected transposition (L-TGA)[4,5] however because of the technical problem of the ventricular septal defect closure to avoid the conduction pathway, they must be differentiated precisely [6].  CMR should be the preferred technique in this setting because it is radiation-free, has a high anatomic definition and overcomes some of the limitations of ultrasound mainly in adult patients.


1. Van Praagh R, Durnin RE, Jockin H, Wagner HR, Korns M, Garabedian H, Ando M, Calder L (1975) Anatomically corrected malposition of the great arteries. Circulation 1975;51:20–31. 

2. Van Praagh R. The story of anatomically corrected malposition of the great arteries. Chest. 1976;69:2–4. 7.  3.

3. Bernasconi A, Cavalle-Garrido T, Perrin DG, Anderson RH: What s anatomically corrected malposition? Cardiol Young 2007;17:26-34.   

4. Oku H, Shirotani H, Yokoyama T, Kawai J, Nishioka T, Noritake S et al. "Anatomically corrected malposition of the great arteries--case reports and a review." Jpn Circ J. 1982;46:583-594. 

5. Rittenhouse EA, Tenckhoff L, Kawabori I, Mansfield PB, Hall DG, Brown JW. Surgical repair of anatomically corrected malposition of the great arteries. Ann Thorac Surg.1986;42:220–8.

6. Anderson RH, Becker AE, Losekoot TG, Gerlis LM. Anatomically corrected malposition of great arteries. Br Heart J. 1975 Oct;37(10):993-1013. 

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