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Intractable Pulmonary Hypertension in a Late Preterm Neonate: Alveolar Capillary Dysplasia, a Lethal Disease

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Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins (ACDMPV) is a rare disorder, with only approximately 200 cases reported to date. The most common presentation of ACDMPV is hypoxic… Click to show full abstract

Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins (ACDMPV) is a rare disorder, with only approximately 200 cases reported to date. The most common presentation of ACDMPV is hypoxic respiratory failure, a condition commonly encountered in preterm neonates. However, the clinical course of the hypoxic respiratory failure and unremitting pulmonary hypertension contrasts dramatically from the usual course of persistent pulmonary hypertension of the neonate (PPHN) in late preterm infants. A 35 4/7 week male infant was born via spontaneous vaginal delivery after routine resuscitation with APGARS of 8 and …

Keywords: pulmonary hypertension; alveolar capillary; capillary dysplasia; late preterm; hypertension

Journal Title: Pediatrics
Year Published: 2018

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