I27.83
BillableEisenmenger's syndrome
HCC Category Mapping
V28HCC 226 — Heart Failure, Specified
0.000V24HCC 85 — Congestive Heart Failure
0.368ESRDHCC 85 — Congestive Heart Failure
0.000RxHCCHCC 183 — Primary Pulmonary Hypertension
0.000What This Code Means
A rare congenital heart condition where blood vessels are reversed, causing oxygen-poor blood to bypass the lungs and mix with oxygen-rich blood.
Coding Tips
- •This is a complex congenital condition; ensure documentation includes the specific cardiac anatomy and any surgical history
- •Often associated with cyanosis and pulmonary hypertension; code related conditions separately
Clinical Significance
Eisenmenger syndrome is a rare, severe congenital heart condition where a left-to-right shunt reverses due to progressive pulmonary hypertension, resulting in cyanosis and significantly reduced life expectancy. This represents one of the most complex congenital cardiac conditions and requires specialized lifelong management.
Documentation Requirements
- ✓Provider diagnosis of Eisenmenger syndrome
- ✓Underlying congenital cardiac defect documented (ventricular septal defect, atrial septal defect, patent ductus arteriosus)
- ✓Evidence of shunt reversal (right-to-left shunting)
- ✓Documentation of cyanosis and pulmonary hypertension
- ✓Surgical history and current management plan
Code Also
Commonly Confused Codes
Q21.0 — Ventricular septal defect; the underlying defect, not the syndromeI27.0 — Primary pulmonary hypertension; different etiology, not congenitalQ21.1 — Atrial septal defect; another potential underlying defectI27.21 — Secondary pulmonary arterial hypertension; Eisenmenger is a specific syndrome beyond just secondary pulmonary hypertension
Code Hierarchy
└I27Other pulmonary heart diseases└I27.8Other specified pulmonary heart diseases└I27.83Eisenmenger's syndrome
└I27.83Eisenmenger's syndrome