Fetal Diagnosis of Supravalvular Aortic Stenosis and Pulmonary Stenosis in a Family with Non-Syndromic Elastin Mutation.

Supravalvular aortic stenosis (SVAS) has been well described in Williams-Beuren Syndrome and non-syndromic elastin (ELN) mutations. Non-syndromic ELN mutations are inherited in an autosomal dominant pattern with incomplete penetrance and variable expressivity. ELN haploinsufficiency leads to progressive arteriopathy, typically affecting the aortic sinotubular junction.

In Utero Presentation of Left Ventricular Aneurysm.

Congenital left ventricular aneurysm, pseudoaneurysm, and diverticulum are rare entities. These diagnoses can be made pre- and/or postnatally. Although these entities overlap clinically and morphologically, important distinctions can allow for accurate diagnoses.

Chronic Maternal Hyperoxygenation and Effect on Cerebral and Placental Vasoregulation and Neurodevelopment in Fetuses with Left Heart Hypoplasia.

Introduction: In a pilot study of chronic maternal hyperoxygenation (CMH) in left heart hypoplasia (LHH), we sought to determine effect estimates of CMH on head size, vascular resistance indices, and neurodevelopment compared to controls.

Material And Methods: Nine gravidae meeting the inclusion criteria (fetal LHH, ≥25.9 weeks' gestation, and ≥10% increase in percent aortic flow after acute hyperoxygenation) were prospectively enrolled.

Economic and safety implications of introducing fast tracking in congenital heart surgery.

Background: The feasibility of fast-tracking children undergoing congenital heart disease surgery has not been assessed adequately. Current knowledge is based on limited single-center experiences without contemporaneous control groups.

Methods And Results: We compared administrative data for atrial septal defect (ASD) and ventricular septal defect (VSD) surgeries in children 2 months to 19 years of age at the Mount Sinai Medical Center (MSMC) with data from comparable patients at 40 centers contributing to the Pediatric Health Information System.