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Persistent pulmonary hypertension of newborn due to congenital capillary alveolar dysplasia
Author(s) -
Singh Soraisham Amuchou,
Ibrahim Thowfique,
Clark Deborah Jane,
Taylor Richard Scott,
George David Hilles
Publication year - 2005
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20244
Subject(s) - medicine , bronchopulmonary dysplasia , persistent pulmonary hypertension , pulmonary hypertension , autopsy , pathophysiology , dysplasia , pathology , pediatrics , cardiology , pregnancy , genetics , biology , gestational age
Congenital alveolar capillary dysplasia is a rare and fatal cause of pulmonary hypertension in neonates. We report on a term baby with severe pulmonary hypertension unresponsive to high‐frequency ventilation and nitric oxide. A diagnosis of alveolar capillary dysplasia was established on autopsy. We review the literature regarding pathophysiology, clinical presentations, associated malformations, and treatment trials. Pediatr Pulmonol. © 2005 Wiley‐Liss, Inc.