Hypoplastic left heart syndrome

This code identifies a severe congenital heart defect characterized by an underdeveloped left side of the heart, including the left ventricle, mitral valve, aortic valve, and aorta. This malformation significantly impairs the heart's ability to pump oxygenated blood to the body.

Assign this code for patients diagnosed with hypoplastic left heart syndrome, typically identified prenatally or shortly after birth. Documentation should clearly state the diagnosis of hypoplastic left heart syndrome, often supported by echocardiogram findings.

• Do not confuse with other forms of congenital heart disease affecting the left side, such as coarctation of the aorta without the full syndrome.
• Always sequence this code as the primary diagnosis when it is the reason for the encounter or admission.
• Consider additional codes for associated anomalies or complications, such as patent ductus arteriosus or patent foramen ovale, if still present and clinically significant.