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To the Editor:
I read with interest the article by Kirali and colleagues1 describing biventricular repair of right atrial isomerism with complex congenital anomalies, in a 4-year-old boy. The authors explain that this young patient had abdominal situs inversus and polysplenia, and they describe the echocardiogram as showing right atrial isomerism, the large atrial septal defect, a subaortic ventricular septal defect, and double-outlet right ventricle. There was hemiazygos continuation of the interrupted inferior vena cava to a left-sided superior vena cava. Apparently, repair was achieved by directing left ventricular blood into the aorta through the subaortic ventricular septal defect. The computed tomogram is also described as showing right atrial isomerism.
However, in reviewing all of this information, one must point out that the polysplenia and situs inversus are in fact associated with left atrial isomerism rather than right atrial isomerism. Certainly similar cases of double-outlet right ventricle with 2 well-developed atrioventricular valves and left atrial isomerism have been described in previous reports. Clearly the subaortic ventricular septal defect can easily be directed from the left ventricle into the aorta, thereby achieving satisfactory biventricular repair. Indeed the tomogram provided in Figure 2A1 suggests a finger-like left atrial-type appendage over the anterior aspect of the heart.
The authors also do not well describe the exact position of the ventricles. Although it would appear that the morphologic left ventricle was located on the left side of the patient, it is important to define the position of the ventricles relative to the fetal ventricular looping in this complex anomaly. Perhaps the authors should reevaluate their assessment of the atrial appendages as observed at surgery, because most likely this patient should have undergone repair of a left atrial isomerism rather than what is described as biventricular repair of right atrial isomerism.