USEFULNESS OF ELECTRON-BEAM COMPUTED-TOMOGRAPHY IN CHILDREN WITH HETEROTAXY SYNDROME

Children with heterotaxy syndrome frequently have complex cardiac and noncardiac malformations requiring detailed diagnostic evaluation for management planning. Direct delineation of these structures by electro n beam computed tomography (EBCT) is validated as a means of diagnosis . From July 1995 to March 1997, 32 patients (16 girls, 16 boys) with c linically impressed heterotaxy syndrome were enrolled in this study. A fter evaluation by echocardiography, EBCT studies were performed. inte rpretation of cardiac anomalies was performed by sequential analysis b ased an these cross-sectional images. The diagnoses were subsequently confirmed by angiocardiography and surgical findings. Twenty-eight pat ients had bilateral trifurcated bronchi, and most of these (24 of 28) did not have a spleen. Four patients had bilateral bifurcated bronchi, 2 patients had polysplenia and the other 2 patients had a lobulated s ingle spleen. We found that laterality could be identified by EBCT in all patients. Comparison of diagnostic yield between echocardiography, catheterization, and EBCT showed that EBCT is superior to echocardiog raphy and catheterization in demonstration of pulmonary venous anatomy and presence of a very small rudimentary ventricle. In addition, asso ciated visceral, bronchopulmonary, mediastinal, and intracardiac anoma lies could all be delineated by EBCT at the same time. Thus, EBCT is a promising complementary mo dality for an overall understanding of het erotaxy syndrome. (C) 1998 by Excerpta Medica, Inc.