J84.843

HCC Category Mapping

What This Code Means

A severe congenital lung disease where the small blood vessels in the lungs are abnormally positioned, preventing proper oxygen exchange and often fatal in infancy.

Coding Tips

• •This is a life-threatening congenital condition; typically diagnosed in neonates or early infancy
• •Requires confirmation of vein misalignment through imaging or pathology; document whether condition is suspected or confirmed

Clinical Significance

Alveolar capillary dysplasia with vein misalignment is a severe, typically fatal congenital lung disorder characterized by maldevelopment of the pulmonary vasculature. Affected neonates present with severe persistent pulmonary hypertension and refractory hypoxemia within the first days of life. This diagnosis carries extremely high mortality and resource utilization, often requiring ECMO and carrying a uniformly poor prognosis without lung transplant.

Documentation Requirements

• ✓Lung biopsy or autopsy confirming alveolar capillary dysplasia with misaligned pulmonary veins
• ✓Documentation of neonatal presentation with severe refractory hypoxemia
• ✓Clinical evidence of persistent pulmonary hypertension of the newborn not responding to standard therapy
• ✓Genetic testing for FOXF1 mutations if performed
• ✓Documentation of intensive care management including ECMO, inhaled nitric oxide, and transplant evaluation

Commonly Confused Codes