The present perspective is a synthesis of 80 published investigations in
the setting of a retroaortic left brachiocephalic vein, described in 250
patients. Clinical presentation, radiographic findings, ultrasonographic
findings, saline contrast echocardiography, computed-tomographic
angiocardiography, magnetic resonance imaging, and angiocardiography
provided the diagnostic information used to define the disease entity
prior to considering the surgical approach to the associated cardiac
anomalies. We have also addressed several issues concerning the
influence of isomerism, the establishment of diagnosis, and its
importance in various surgical and interventional procedures. Although
the retroaortic left brachiocephalic vein is asymptomatic, its
recognition during clinical investigation should raise the possibility
of an association with other malformations, especially right aortic
arch, ventricular septal defect, and anomalies of the outflow tracts. We
submit that an increased appreciation of this venous anomaly may
facilitate surgical planning, endovascular procedures, placement of
central venous lines, and transvenous pacemakers.