Hypoplastic right heart syndrome

This code describes a congenital heart defect characterized by underdevelopment of the right side of the heart, including structures such as the right ventricle, tricuspid valve, and pulmonary artery. This hypoplasia impairs the heart's ability to effectively pump deoxygenated blood to the lungs, leading to significant circulatory compromise.

Assign this code for documented cases of hypoplastic right heart syndrome, a severe form of congenital heart disease. This diagnosis is typically made prenatally or in infancy based on echocardiography findings demonstrating the underdeveloped right heart structures. Documentation should clearly specify the hypoplasia of the right heart.

• Differentiate from hypoplastic left heart syndrome (Q23.4), which involves underdevelopment of the left side of the heart.
• Ensure documentation specifies "right heart" to avoid confusion with other hypoplastic cardiac conditions.
• Code any associated congenital anomalies of the great arteries or other cardiac structures separately, if documented.