Impact of Modified Anesthesia Management for Pediatric Patients With Williams Syndrome.

Objective: This study compared the percent change in systolic blood pressure and the incidence of adverse cardiac events (ACEs; defined as cardiac arrest, cardiopulmonary resuscitation, arrhythmias, or ST-segment changes) during anesthesia induction in patients with Williams syndrome (WS) before and after implementation of a perioperative management strategy.

Design: Retrospective observational cohort study.

Setting: Single quaternary academic referral center.

Surgical repair of coronary artery ostial stenosis in patients with Williams and elastin arteriopathy syndromes.

Objective: Patients with Williams and elastin arteriopathy syndromes often have similar cardiac phenotypes characterized by supravalvar aortic stenosis (SVAS), peripheral pulmonary artery stenosis (PPAS), and coronary artery ostial stenosis (CAOS). SVAS and/or PPAS result in a marked increase in myocardial oxygen demand, whereas CAOS may limit myocardial oxygen supply. This combination predisposes to myocardial ischemic events and sudden cardiac arrest.

Pulmonary Artery Reconstruction After Failed Pulmonary Artery Stents.

Background: Pulmonary artery stents are widely deployed in patients with stenoses in the branch pulmonary arteries. However, stents do not address more peripheral sites of stenosis and invariably develop in-stent restenosis. The purpose of this study was to review our experience with pulmonary artery reconstruction after failed pulmonary artery stents.

Surgical Repair of Peripheral Pulmonary Artery Stenosis in Patients Without Williams or Alagille Syndromes.

Peripheral pulmonary artery stenosis is a relatively rare form of congenital heart disease typically associated with genetic syndromes, such as Williams or Alagille syndromes. However, some patients present with severe stenosis without associated syndromes. The purpose of the study was to review our surgical experience in such patients.

Outcomes of Pulmonary Artery Reconstruction in Williams Syndrome.

Background: The study sought to evaluate the short-term and midterm outcomes of surgical pulmonary artery reconstruction in patients with Williams syndrome (WS).

Methods: We performed a retrospective cohort study of all patients with WS who underwent surgical pulmonary artery reconstruction at Lucile Packard Children's Hospital between January 2001 and May 2018.

Results: There were 25 WS patients (52% female) who underwent pulmonary artery reconstruction during the study period.