Hugh D. White et al. JIMG 2013;6:

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Presentation transcript:

Hugh D. White et al. JIMG 2013;6:1342-1345 Patient #7: Primum ASD Another rare type of ASD encountered in the adult is the ostium primum defect. When the atrial septum develops in the fetus, the first septum that arises, the septum primum, grows from the basal wall of the atria toward the apical wall (toward the ventricles). In the ostium primum ASD, this septum fails to reach its destination, and there is persistent communication between the atria with a variable amount of intact septum attached to the basal wall. This defect commonly presents with a proximal ventricular septal defect (VSD). The combination of this ASD and VSD is referred to as the “endocardial cushion defect” because the endocardial cushion is the site where the atrial septum meets the ventricular septum and the mitral valve meets the tricuspid valve. It has also been referred to as the “atrioventricular septal defect” and “atrioventricular canal defect.” This type of defect is the most common congenital heart defect associated with Down syndrome. (A) Four-chamber, (B) atrial short axis, (C) atrial sagittal, and (D) ventricular short axis CTA images are shown. Patient #7 has both a primum ASD (yellow arrows) and a proximal VSD (red arrows). (A) The incomplete atrial septum is clearly shown (blue arrow), which is attached to the basal wall but does not extend to meet the ventricular septum. (C) The variability in this type of ASD is highlighted; in this case, the defect extends inferiorly and could be mistaken for the inferior sinus venosus ASD on this image alone. (D) This short axis image through the proximal ventricles clearly demonstrates the VSD component of the endocardial cushion defect. The patient was a 46-year-old man with Down syndrome. The chronic left-to-right shunting through the endocardial cushion defect resulted in marked dilation of the RV, RVOT, and PA. Abbreviations as in Figures 1 and 2. Hugh D. White et al. JIMG 2013;6:1342-1345 American College of Cardiology Foundation