A 25-year-old male was admitted for surgical repair of complex congenital heart disease. He reported having a cardiac murmur for as long as he could remember. Cardiac catherization, done when the patient was five years old, demonstrated L-transposition of the great vessels with ventricular inversion, ventricular septal defect, and probable subpulmonic stenosis.
A 25-year-old male was admitted for surgical repair of complex
congenital heart disease. He reported having a cardiac murmur for
as long as he could remember. Cardiac catherization, done when the
patient was five years old, demonstrated L-transposition of the
great vessels with ventricular inversion, ventricular septal
defect, and probable subpulmonic stenosis. He was followed
conservatively, as he was acyanotic at that time. In 1987, he began
to develop clubbing and progressive dyspnea on exertion, for which
further workup was scheduled. However, the patient was lost to
follow-up. He presented again with worsening dyspnea on exertion
and underwent repeat cardiac catheterization which showed a very
large ventricular septal defect and critical subpulmonic stenosis.
A helical CT examination was performed prior to surgery. A chest
radiograph obtained preoperatively showed dextrocardia without
situs inversus [figure 1].
Sequential images from the helical CT examination demonstrate
the trabeculated morphologic "right" ventricle on the left side
communicating with the morphologic "left" ventricle via a
ventricular septal defect [figure 2]. The aorta arises from the
"right" ventricle and lies anterior to the pulmonary artery and to
the left of midline [figure 3]. Sagittal reconstruction again shows
the anterior lying aorta and demonstrates the critical subpulmonic
stenosis [figure 4]. The patient subsequently underwent successful
repair of his ventricular septal defect and pulmonic stenosis.
Congenitally corrected transposition of the great vessels
Congenitally corrected transpositon refers to atrioventricular
discordance, ventricular inversion, transposition and inversion of
the great arteries.1 This is a rare condition, with an incidence of
approximately 1 in 13,000 live births. It is estimated that 1% of
these cases are uncomplicated (i.e., with no associated cardiac
These patients rarely live beyond the age of fifty; however,
there is a case on record of a patient living to be 80 years old.3
Most afflicted patients die young due to associated congenital
anomalies or heart block.4 Findings in these cases have been
described on plain chest films, angiography and echocardiography.5
MRI and computed tomography imaging also have been used.6,7 All
findings on cardiac catheterization of this patient were well
visualized with helical CT. One advantage of helical CT is the
rapidity of the study [30 seconds]. The short scan time allows for
breath-hold imaging, thus minimizing motion artifacts. It also
enables optimal timing of the bolus to obtain a continuous data set
without an interval. This data set can then be used for multiplanar
reconstructions. In this patient, data set images and
reconstructions clearly delineated the transposed arteries and the
ventricular septal defect, as well as the subpulmonic stenosis.
Review of images in the cine mode can give valuable information
about the direction of shunting.
1. Perloff JK: Congenitally corrected transposition of the great
arteries. In: The Clinical Recognition of Congenital Heart Disease,
pp 62-83. Philadelphia, WB Saunders Co., 1987.
2. Losekoot TG, Anderson RH, Becker AE, et al (eds):
Congenitally corrected transposition, pp 1-191. London, Churchill
3. Pezard PP, Banus Y, Laporte J, et al: Transposition Corrigee
Des Gros Vaisseaux De L'Adulte Age. Arch Mal Coeur 79:1637-1642,
4. Ikeda U, Furuse M, Suzuki O, et al: Long term survival in
aged patients with corrected transposition of the great arteries.
Chest 5:1382-1385, 1992.
5. Tonkin IL, Kelley MJ, Bream PR, Elliott LP: The frontal chest
film as a method of suspecting transposition complexes. Circulation
6. Park JH, Han MC, Kim CW: MR Imaging of congenitally corrected
transposition of the great vessels in adults. AJR 153:491-494,
7. Schlesinger AE, Hernandez RJ: Congenital heart disease:
Applications of computed tomography and magnetic resonance imaging.
Semin Ultrasound CT MR 12:11-27, 1991.
Prepared by Lloyd E. Heller, MD and Harry G. Zegel, MD,
University Hospitals, MCP Division, Philadelphia, PA; and Vikram
S. Dravid, MD, Mercy Catholic Medical Center, Darby, PA.