Absence and aplasia of aorta

This code signifies a congenital anomaly where the aorta, the body's main artery, is either completely missing (aplasia) or severely underdeveloped and non-functional (absence). This condition is typically incompatible with life without significant surgical intervention or the presence of other compensatory cardiovascular defects.

This code is used for newborns or infants diagnosed with a complete lack of aortic development or a rudimentary, non-patent aorta. Documentation supporting its use would include echocardiogram reports, cardiac catheterization findings, or autopsy reports detailing the absence or aplasia of the aorta. It is often identified in conjunction with other complex congenital heart defects.

• Always confirm the specific type of aortic anomaly; differentiate from coarctation or hypoplasia, which have distinct codes.
• Sequence this code as the primary diagnosis when it is the main reason for the encounter or admission.
• Look for associated congenital heart defects, as they are frequently present and should be coded additionally.