ICD-10 Code J84.843 – Alveolar capillary dysplasia with vein misalignment (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Alveolar capillary dysplasia with vein misalignment is J84.843.
2026 ICD-10-CM Diagnosis Code J84.843 – Alveolar capillary dysplasia with vein misalignment

What it is

J84.843 identifies alveolar capillary dysplasia with vein misalignment, a rare congenital lung disorder that disrupts normal formation of the alveoli and pulmonary vessels. It is a serious developmental condition affecting newborns and infants.

Clinical signs

Clinical features vary; refer to documentation. Typical findings include severe respiratory distress soon after birth, persistent hypoxemia, and pulmonary hypertension that does not improve as expected.

When to use this code

Use this code when the provider documents alveolar capillary dysplasia with vein misalignment, or a confirmed diagnosis using pathology, imaging, or specialist evaluation. It may also apply when the condition is recorded as the underlying cause of neonatal respiratory failure. Check documentation if the record only notes unexplained pulmonary hypertension.

Do not use for

Do not use J84.843 for nonspecific neonatal respiratory distress, pulmonary hypertension without this diagnosis, or other congenital lung anomalies. If the chart does not clearly support this disorder, check documentation.

Coding tip

Confirm that the diagnosis is explicitly stated, because this code is for a specific congenital pathology, not a general respiratory presentation.

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