Alveolar capillary dysplasia with misalignment of pulmonary
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Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal, developmental lung disorder, which usually presents as persistent pulmonary hypertension of the newborn (PPHN) unresponsive to treatment.The majority of the reported cases have been associated with other multiple congenital nonlethal anomalies, most frequently involving the cardiovascular, gastrointestinal, urogenital and musculoskeletal systems. Increasing awareness of this clinical entity may prevent the use of more invasive and futile treatments, including extracorporeal membrane oxygenation (ECMO).We present three cases of ACD/MPV associated with gastrointestinal and urological malformations. All the newborns had an overwhelming course, with PPHN and hypoxemia refractory to treatment. The diagnosis of ACD/MPV was established by autopsy...

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