Hypoplastic left heart syndrome

This code represents a severe congenital heart defect characterized by an underdeveloped left side of the heart, including the left ventricle, aorta, and mitral valve. This malformation significantly impairs the heart's ability to pump oxygenated blood to the body. It is a life-threatening condition requiring complex surgical intervention.

Use this code for patients diagnosed with hypoplastic left heart syndrome, typically identified prenatally or shortly after birth. Documentation should clearly indicate the specific structural abnormalities consistent with this diagnosis, such as hypoplasia of the left ventricle, aortic atresia or severe stenosis, and mitral atresia or severe stenosis.

• Ensure documentation specifies "hypoplastic left heart syndrome" and not just general congenital heart disease.
• This code is typically a primary diagnosis for neonates undergoing staged surgical repair (e.g., Norwood procedure).
• Consider sequencing with codes for associated conditions or complications, such as patent ductus arteriosus or patent foramen ovale, if present and clinically significant.