Alveolar capillary dysplasia with misalignment of the pulmonary veins: a rare but fatal cause of neonatal respiratory failure

Respiratory failure in the neonate that is refractory to maximal medical ma nagement is a frequent indication for extracorporeal life support (ECLS). A lveolar capillary dysplasia with misalignment of the pulmonary veins is an irreversible cause of respiratory failure that cannot be diagnosed on clini cal grounds alone and would not be expected to respond to ECLS therapy. A r ecent experience with a patient prompted us to review the literature regard ing this condition for the purpose of identifying factors suggestive of thi s diagnosis. This condition should be considered in neonates with presumed pulmonary hypertension who cannot be weaned from ECLS. If the diagnosis is made by antemortem open-lung biopsy, a costly, protracted, and unnecessary continued course of ECLS may be avoided.