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

The presence of a restrictive atrial septal defect in hypoplastic left heart syndrome represents a surgical emergency and may negatively affect survival after operation. A neonate with such a disease association, requiring septectomy upon birth developed intractable respiratory failure due to congenital pulmonary lymphangiectasis. The therapeutic implications of this rare pathologic condition are discussed.