Purpose or Case Report: We report the case of a neonatal male with alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV). ACD/MPV is a rare, fatal disorder of the development of the alveolar vasculature. In this disorder, alveolar capillaries are reduced in number and are improperly located within the walls of the alveoli impeding oxygen exchange. In addition to alveolar capillary abnormalities, there are other associated abnormalities with the cardiovascular, gastrointestinal tract, and genitourinary systems. In this report, we describe the case of a term male neonate with a prenatal diagnosis of hypoplastic left heart who developed respiratory distress shortly after birth. The patient required prolonged ECMO and underwent evaluation with CT angiography of the chest. CT findings included diffuse bilateral interstitial thickening with enhancing airspace disease scattered in the left lung. The respiratory distress continued to be unresponsive to treatment, with difficulty weaning ECMO and respiratory support. Life support was withdrawn on day 30. Pathologic examination at autopsy demonstrated ACD/MPV. Our case adds to the limited literature available in describing imaging findings of ACD/MPV and support for early diagnosis with lung biopsy as it can prevent aggressive futile interventions.
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