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Misalignment of pulmonary veins with alveolar capillary dysplasia: affected siblings and variable phenotypic
S Boggs1, M C Harris, D J Hoffman
1Division of Neonatology, University of Pennsylvania School of Medicine, Philadelphia.
The Journal of Pediatrics
|January 1, 1994
Abstract:
Misalignment of pulmonary veins with alveolar capillary dysplasia is recognized as a rare cause of persistent pulmonary hypertension of the neonate. Until now, misalignment of pulmonary veins was thought to be a random occurrence, but its appearance in siblings at our institution suggests that there may be a familial predisposition. There have been reports of variable expression and variable severity in this disease; our report describes this variability in family members.