Figure 2: Ventriculography showing the morphological right ventrical ejecting into the aorta.
Repairs of congenitally corrected transposition of the great arteries plus dextrocardia in adulthood[4] have been reported in the literature. This case had atrial situs inversus with a bilateral superior vena cava along with congenitally corrected transposition of the great arteries.
Patients with congenitally corrected transposition of the great arteries in which the operation has been performed from the left side have been reported in the literature.[5] In this case, because of the presence of atrial situs inversus and a small-sized right atrium, it was mandatory that the surgery be done from the left side.
Diagnosis is usually made via echocardiography, which can be confirmed by cardiac catheterization or magnetic resonance imaging (MRI). In addition, MRI[6] may be helpful in confirming other associated anomalies. A chest X-ray and ultrasonography of the abdomen are necessary to diagnose visceral situs.
Left ventricular outflow tract obstruction can be relieved with a transannular patch in patients with atrial situs inversus with congenitally corrected transposition of the great arteries since they usually have a normally positioned atrioventricular node[7] and the bundle of His runs on the posterior inferior margin of the ventricular septal defect. This contrasts with the congenitally corrected transposition of the great arteries with atrial situs solitus in which the atrioventricular node is located anteriorly adjacent to the right atrioventricular valve orifice with the bundle of His coursing on the anterior right ventricular free wall caudal to the pulmonary annulus. It then descends to the anterior part of the infundibular septum.
Patients suffering from atrial situs inversus with congenitally corrected transposition of the great arteries are less prone to conduction anomalies compared with patients with atrial situs solitus with congenitally corrected transposition of the great arteries. However, in this case, the patient had conduction abnormalities postoperatively for which a permanent epicardial pacemaker was implanted.
Anatomical correction (double switch) was not possible in this case as there was severe pulmonary stenosis. The atrial switch using the Rastelli procedure was avoided because of problems associated with the prosthetic conduit[8] and the complex nature of the repair.
In conclusion, for patients with atrial situs inversus congenitally corrected transposition of the great arteries with pulmonary stenosis, pulmonary artery augmentation can be done with a transannular patch, thus obviating the need for a prosthetic conduit. This is not possible for patients with atrial situs solitus congenitally corrected transposition of the great arteries with pulmonary stenosis.
The position of the chambers necessitated performing the operation from the left side.
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