Abstract
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 authors present their own experience with three cases admitted during the last 15 years.
Highlights
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. e authors present their own experience with three cases admitted during the last 15 years
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV; OMIM number 265380) is a rare, fatal, developmental lung disorder, which usually presents as persistent pulmonary hypertension of the newborn (PPHN) unresponsive to treatment [1, 2]. e majority of the reported cases have been associated with other multiple congenital nonlethal anomalies, most frequently involving the cardiovascular, gastrointestinal, urogenital, and musculoskeletal systems [1]
Newborns affected with ACD/MPV develop respiratory distress and severe PPHN within the rst 48 hours of life and die of respiratory failure within the rst month of life, longer survivals and later presentations have been reported [2, 4,5,6]
Summary
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV; OMIM number 265380) is a rare, fatal, developmental lung disorder, which usually presents as persistent pulmonary hypertension of the newborn (PPHN) unresponsive to treatment [1, 2]. e majority of the reported cases have been associated with other multiple congenital nonlethal anomalies, most frequently involving the cardiovascular, gastrointestinal, urogenital, and musculoskeletal systems [1]. E majority of the reported cases have been associated with other multiple congenital nonlethal anomalies, most frequently involving the cardiovascular, gastrointestinal, urogenital, and musculoskeletal systems [1]. Increasing awareness of this clinical entity may prevent the use of more invasive and futile treatments, including extracorporeal membrane oxygenation (ECMO). Full-term female newborn admitted to our NICU (Neonatal Intensive Care Unit) in 1997 was diagnosed with severe bilateral hydronephrosis. She was the second daughter of a young healthy, unrelated couple.
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