CARDIOVASCULAR JOURNAL OF AFRICA • Vol 23, No 5, June 2012
AFRICA
e7
The patient was referred for cardiovascular surgery but she
refused and has been followed up medically.
Discussion
This case presents a unique feature rarely described in the
medical literature, namely, the combination of several congenital
anomalies. CTGA may remain asymptomatic for several years
in individuals without associated cardiopathies and may only
be seen on ECG or X-ray due to the unusual position of the
ventricles. Over time, systemic ventricular failure may develop
due to pressure overload, causing fatigue and dyspnoea.
1
In this anomaly, the right atrium enters the morphological
left ventricle, which leads to the pulmonary artery, and the left
atrium communicates with the morphological right ventricle,
which leads to the aorta. Therefore, atrio-ventricular and
ventriculo-arterial discordance exists, and although blood flows
in the normal direction, it passes through the ‘wrong’ ventricular
chamber. This ‘double discordance’ results in the term CTGA,
which is, in essence, a misnomer.
5
It is also called L-transposition
because the morphological right ventricle is in the levoposition.
The aorta is also usually, but not universally, anterior and to
the left, and the great arteries may be side by side. Because the
tricuspid valve always enters a morphological right ventricle,
it too is on the left side in the systemic circulation and is more
appropriately termed the systemic atrio-ventricular valve.
2
In our
case, the morphological right ventricle was in the dextroposition,
the aorta was anterior and to the right, and the great arteries were
side by side (in the D-transposition) (D-CTGA).
Coronary artery anomalies in congenitally corrected
transposition of the great arteries have seldom been reported in
the medical literature due to the rarity of this disorder and the
relatively small number of patients in each reported series.
6
The
coronary arteries usually course to their respective ventricles.
Significant coronary artery abnormalities may affect the surgical
approach, with unexpected anatomy being associated with higher
morbidity and mortality rates,
7
so clinicians must be careful to
identify these anomalies in a timely manner.
Conclusion
A single case of CTGA combined with ASD, pulmonary stenosis
and situs inversus has been reported before.
8
Our report, however,
demonstrates the combination of ASD, pulmonary stenosis, right
arcus aorta and coronary artery anomalies in a case of CTGA
involving situs inversus. We believe that this combination is
extremely rare. To our knowledge, this is the first case of its kind
reported in the medical literature.
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