Electrocardiograms Do Not Detect Myocardial Ischemia in Patients With Williams Syndrome and Nonsyndromic Elastin Arteriopathy With Coronary Artery Stenosis.

Coronary artery stenosis (CAS) may affect up to 27% of patients with Williams syndrome (WS), which may lead to myocardial ischemia. Patients with WS face a 25- to 100-fold greater risk of sudden cardiac death, frequently linked to anesthesia. Assessing CAS requires either imaging while under general anesthesia or intraoperative assessment, with the latter considered the gold standard.

Risk Factors for Adverse Outcomes in Term Infants with CHD and Definitive Necrotising Enterocolitis.

Objectives: To define the incidence of definitive necrotising enterocolitis in term infants with CHD and identify risk factors for morbidity/mortality.

Methods: We performed a 20-year (2000-2020) single-institution retrospective cohort study of term infants with CHD admitted to the Boston Children's Hospital cardiac ICU with necrotising enterocolitis (Bell's stage ≥ II). The primary outcome was a composite of in-hospital mortality and post-necrotising enterocolitis morbidity (need for extracorporeal membrane oxygenation, multisystem organ failure based on the paediatric sequential organ failure assessment score, and/or need for acute gastrointestinal intervention).

Arrhythmias in Williams Syndrome.

Williams syndrome (WS) is a congenital, multisystem disorder in which 80% of patients have cardiovascular abnormalities. Sudden cardiac death occurs 25 to 100 times more often in WS than in the general population, and cardiac repolarization is abnormal in WS. We sought to determine the prevalence of primary arrhythmias in patients with WS and whether QTc prolongation impacts arrhythmia risk.

Importance of Formula-Specific Centile Thresholds for Evaluation of Heart Rate-Corrected QT Prolongation in Williams Syndrome.

Patients with Williams syndrome (WS) have a 25- to 100-fold higher risk of sudden death and prolonged heart rate-corrected QT (QTc). A recent study using the Fridericia formula for QT correction suggested that prolongation is principally an issue of heart rate. We used multiple published heart rate correction formulas to reevaluate the prevalence of QTc prolongation in our original dataset from our 2010 study at the Children's Hospital of Philadelphia.