From the Departments of Cardiology (E.D.B., T.G.) and Radiology (T.C.,
F.A.H.), Children's Hospital, and the Departments of Pediatrics (E.D.B.,
T.G.) and Radiology (T.C., F.A.H.), Harvard Medical School, Boston, Mass.
Correspondence to Tal Geva, MD, Department of Cardiology, Children's Hospital, 300 Longwood Ave, Boston, MA 02115. E-mail geva_t{at}a1.tch.harvard.edu
B.M. is a previously healthy 18-year-old man
referred for a second opinion on an abnormal echocardiogram. He had
been followed in the first year of life for a nonspecific murmur. He
presented again to medical attention secondary to Navy
prequalification requirements and was referred to a local cardiologist.
His physical examination, chest radiograph, and ECG were normal. On
transthoracic and transesophageal
echocardiograms performed in the referring institution, "the aorta
did not connect to the left ventricle" and the superior aspect of the
ventricular septum was "prominent." Cardiac MRI
revealed levocardia with visceroatrial situs solitus and
D-ventricular loop. The great arteries originated above the
appropriate ventricles (ventriculoarterial concordance)
(Fig 1
Anatomically corrected malposition of the great arteries is a rare form
of congenital heart disease in which the great arteries are abnormally
related to the ventricles and to each other but nonetheless arise above
the anatomically correct ventricles. This abnormal relationship was
first reported in 1895 by Theremin and was characterized by Van Praagh
et al1 in 1975. In the absence of associated
malformations, anatomically corrected malposition is associated with
normal physiology and may be detected incidentally. MRI proved useful
in establishing the diagnosis noninvasively in this patient with
limited acoustic windows.
Footnotes
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
References
1.
Van Praagh R, Durnin RE, Jockin H, Wagner HR,
Korns M, Garabedian H, Ando M, Calder L. Anatomically corrected
malposition of the great arteries. Circulation.. 1975;51:20-31.
© 1998 American Heart Association, Inc.
Images in Cardiovascular Medicine
Anatomically Corrected Malposition of the Great Arteries {S,D,L}
), but their spatial position and
orientation were abnormal: the aortic valve was anterior, superior, and
leftward relative to the pulmonary valve and the great vessels
were side-by-side (Figs 1 and 2). There
was bilateral conus with aortic-mitral and pulmonary-tricuspid
discontinuity and a well-developed subaortic chamber without
obstruction. The patient had normal coronary anatomy
and normal ventricular function.
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Figure 1. Coronal T1-weighted MRI of chest
demonstrates levocardia, atrial situs solitus,
D-ventricular loop, and L-malposition of aorta with
ventriculoarterial concordance. Aorta (AO) and main
pulmonary artery (PA) arise above appropriate ventricles, but
aortic valve is anterior, superior, and leftward relative to
pulmonary valve and is supported by a conus. This image
demonstrates unobstructed left ventricular outflow tract.
LV indicates left ventricle; RA, right atrium; R, right; and S,
superior.
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[in a new window]
Figure 2. Transverse T1-weighted MRI of chest at level
of aortic (AO) and pulmonary (PA) roots demonstrates
side-by-side relationship of great vessels, with aorta being to left of
main pulmonary artery. A indicates anterior; LA, left atrium;
R, right; and SVC, superior vena cava.
This article has been cited by other articles:
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  T. Geva, A. J. Powell, E. C. Crawford, T. Chung, and S. D. Colan Evaluation of Regional Differences in Right Ventricular Systolic Function by Acoustic Quantification Echocardiography and Cine Magnetic Resonance Imaging Circulation, July 28, 1998; 98(4): 339 - 345. [Abstract] [Full Text] [PDF]
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