Eisenmenger's syndrome

This code represents Eisenmenger's syndrome, a severe form of pulmonary hypertension that develops as a complication of an uncorrected congenital heart defect with a left-to-right shunt. The persistent shunt leads to increased blood flow and pressure in the pulmonary arteries, eventually causing irreversible pulmonary vascular disease and shunt reversal (right-to-left). This results in cyanosis and other systemic complications.

Use this code when documentation clearly indicates a diagnosis of Eisenmenger's syndrome. This typically occurs in patients with long-standing, uncorrected congenital heart defects such as ventricular septal defects, atrial septal defects, or patent ductus arteriosus, who have subsequently developed severe pulmonary hypertension with shunt reversal. The physician's diagnosis of Eisenmenger's syndrome is the primary documentation requirement.

• Do not code congenital heart defects separately if they are the underlying cause of Eisenmenger's syndrome, as Eisenmenger's syndrome encompasses the entire complex.
• Distinguish carefully from other forms of pulmonary hypertension; this code is specific to the syndrome resulting from congenital shunts.
• Review documentation for specific mention of "Eisenmenger's syndrome" or a clear description of its components (congenital heart defect, severe pulmonary hypertension, and shunt reversal).