Hypoplastic left heart syndrome with restrictive atrial septal defect and congenital pulmonary lymphangiectasis

The presence of a restrictive atrial septal defect in hypoplastic left hear t syndrome represents a surgical emergency and may negatively affect surviv al after operation. A neonate with such a disease association, requiring se ptectomy upon birth developed intractable respiratory failure due to congen ital pulmonary lymphangiectasis. The therapeutic implications of this rare pathologic condition an discussed. (C) 1998 by Elsevier Science Inc.