Pulmonary artery rupture after bilateral pulmonary artery banding in a neonate with Loeys-Dietz syndrome and an interrupted aortic arch complex: report of a case.

ABSTRACT

Loeys-Dietz syndrome (LDS) is a recognized connective tissue disorder characterized by progressive aortic aneurysm and dissection. Patients are at high risk of aortic dissection or rupture at an early age, but to our knowledge, surgery on the great arteries has never been attempted in the neonatal period. We report a case of LDS with dilated pulmonary arteries and an interrupted aortic arch complex in a neonate. We performed bilateral pulmonary artery banding, but 12 days after the procedure, the infant died of rupture of the distal portion of the banding sites following massive dilatation.