Electron-beam CT diagnosis of the visceroatrial heterotaxy syndrome

Objetives To assess the usefulness of electron beam CT (EBCT) in diagnosis of the visceroatrial heterotaxy syndrome. Methods Ten patients with the visceroatrial heterotaxy syndrome were evaluated. The patients ranged in age from 7 months to 17 years (averaged 9.5 years). Five of the patients underwent EBCT contrast single slice mode while another 5 patients did continuous volume scan obtained from the superior aperture of thorax to the middle part of abdomen. All the patients had both angiocardiogram and echocardiogram, and 6 patients had operative outcomes. Results (1)Eight patients with right atrial isomerism, bilaterally morphologic right atrial appendages, right lobe dominant symmetric liver, bilaterally epiarterial bronchi, trilobed lungs, and total anomalous pulmonary venous connectionl were clearly detected. Endocardial cushions defect, pulmonary stenosis, right sided aortic arch and descending aorta were documented in 7 patients. Double outlet of right ventricle was imaged in 6 patients and hiatal hernia in 2 patients. Neither a spleen nor splenules were found. (2) Two patients with left atrial isomerism, bilaterally morphologic left atrial appendages, left lobe dominant symmetric liver, bilaterally hyparterial bronchi, bilobed lungs, double outlet of right ventricle, pulmonary stenosis, interruption of inferior vena cave, right sided aortic arch, and descending aorta were documented. Endocardial cushions defect was detected in 1 patient. Multiple spleens were demonstrated in the right upper abdomen in the 2 patients.(3) The number of abnormal obervasions detected by EBCT was 116,while that done by angiocardiogram and echocardiogram were 65 and 43 respectively. Conclusion EBCT is a usefu1 tool in the evaluation of patients with the syndrome of visceroatrial heterotaxia.