Double inlet ventricle

This code represents a rare congenital heart defect where both atria connect to a single ventricle, which then pumps blood to both the pulmonary and systemic circulations. The other ventricle is typically rudimentary or absent. This malformation leads to mixing of oxygenated and deoxygenated blood.

Use this code for patients diagnosed with a double inlet ventricle, regardless of the specific morphology of the great arteries or the presence of a rudimentary outflow chamber. Documentation should clearly state "double inlet ventricle" or a similar anatomical description confirming the connection of both atria to a single ventricular chamber.

• Do not confuse with single ventricle, unspecified (Q20.8), which is a less specific diagnosis.
• Code any associated anomalies of the great arteries (e.g., transposition, malposition) or other cardiac defects separately.
• Ensure documentation specifies the presence of two atria connecting to one ventricle to support this specific code.