A case report of rapid progressive coarctation and severe middle aortic syndrome in an infant with Williams syndrome.

Williams syndrome is a genetic disorder caused by multiple contiguous gene deletions in chromosome 7. Presentation in early life is most often a result of luminal stenosis of right- and left-sided arterial vasculature. We report the case of a newborn infant who had a rapidly progressing diffuse form of arteriopathy that required two surgeries and one percutaneous balloon dilation within the first 2 months of her life.

A novel case of L-transposition with a right-dominant double aortic arch.

We describe a case of congenitally corrected transposition with a double aortic arch. This unique combination of lesions highlights the importance of a complete anatomic assessment prior to referral for surgery.

Impact of mitral stenosis and aortic atresia on survival in hypoplastic left heart syndrome.

Background: Aortic atresia has been implicated as a risk factor for decreased survival after stage 1 palliation. Prior studies evaluating the association of anatomic subtypes and mortality report conflicting results. Our objective was to determine if mitral valve patency with aortic atresia is associated with increased mortality in hypoplastic left heart syndrome (HLHS).

Hypoplastic left heart syndrome with atrial level restriction in the era of prenatal diagnosis.

Background: Despite recent improvements in survival of patients with hypoplastic left heart syndrome, those with severe atrial level restriction continue to face higher rates of mortality. We sought to assess the impact of prenatal diagnosis and immediate intervention on the outcome of this high-risk population.

Methods: We performed a retrospective review of patients with hypoplastic left heart syndrome and restriction at the atrial level born between 1997 and 2006.