The lateral X ray with barium swallow shown here clearly demonstrates the reduced anteroposterior dimension with a loss of dorsal kyphosis that is, a straight back; and there is obliteration of the retrosternal space.

Because of this skeletal variation, the cardiac silhouette and pulmonary artery may appear to be enlarged in the PA chest X ray. Patients with this variation may present with an exaggeration of normal bedside findings and mitral valve prolapse may be associated with this syndrome.