ICD-10 Code I27.83 – Eisenmenger’s syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code I27.83 – Eisenmenger’s syndrome
What it is
Eisenmenger’s syndrome is a complication of certain congenital heart defects where long-standing left-to-right shunting leads to pulmonary hypertension and reversed shunting. It reflects advanced cardiopulmonary disease.
Clinical signs
Clinical features may include cyanosis, exertional shortness of breath, clubbing, and signs of pulmonary hypertension. Clinical features vary; refer to documentation.
When to use this code
Use I27.83 when the provider documents Eisenmenger’s syndrome or Eisenmenger physiology. It is appropriate when the record links the syndrome to an underlying congenital shunt and confirms pulmonary vascular disease. If the note only mentions congenital heart disease without Eisenmenger’s syndrome, check documentation.
Do not use for
Do not use this code for isolated congenital heart defects without Eisenmenger’s syndrome. Do not assign it for pulmonary hypertension of another cause unless the provider specifically documents Eisenmenger’s syndrome.
Coding tip
Capture the exact provider wording and code any associated congenital heart defect separately when documented.