Alveolar capillary dysplasia with vein misalignment

This code identifies a rare, severe developmental lung disorder characterized by abnormal development of the alveolar capillaries and misaligned pulmonary veins. It results in persistent pulmonary hypertension of the newborn and severe hypoxemia due to impaired gas exchange. This condition is often fatal in the neonatal period.

Use this code for neonates diagnosed with alveolar capillary dysplasia (ACD) where pulmonary vein misalignment is also present. Documentation should clearly state the diagnosis of ACD and specifically mention the co-occurrence of vein misalignment, typically confirmed through imaging and/or biopsy.

• Ensure documentation explicitly states "with vein misalignment" to support the specificity of this code.
• Do not confuse with other forms of pulmonary hypoplasia or persistent pulmonary hypertension without the specific histological findings of ACD with vein misalignment.
• This code is typically assigned as a principal diagnosis for affected neonates due to its severe and primary nature.