Publications by authors named "WG Williams"

Article Synopsis
  • - The study evaluated the survival rates and need for re-operation in children who had surgery for transposition of great arteries (TGA) from 1990 to 2019, finding a strong 30-year survival rate of 92% and a low in-hospital death rate of 3.8%.
  • - Most patients (94%) underwent arterial switch operations, with 82% being free from cardiac re-operations 30 years post-surgery. Re-operation rates were highest in the first 2 years, then decreased for 18 years, followed by an increase after 20 years.
  • - Late complications were noted, particularly a concerning rise in neo-aortic valve and root re-operations after 17 years,
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Article Synopsis
  • Tricuspid atresia (TA) is a heart condition affecting infants and is the second most common type of functionally univentricular heart, with rare cases of left ventricular outflow tract obstruction (LVOTO) in patients who have normally related great arteries.
  • A study involving 445 patients with Type I TA showed that 3% of infants needed interventions for LVOTO, and most of those who underwent surgical procedures had promising outcomes.
  • The long-term survival rate for these infants was estimated at 79% over 20 years, indicating that timely surgical management can lead to successful interventions and good heart function later in life.
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Transportation accounts for one-quarter of all energy related greenhouse gas emissions. As it pertains to transport electrification, higher education institutions-such as universities-can model solutions that affect broader society. Despite this, higher education's role in fleet electrification adoption has been understudied.

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Purpose: We reviewed all 64 articles ever published by The Congenital Heart Surgeons' Society (CHSS) Data Center to estimate the academic impact of these peer-reviewed articles.

Materials And Methods: The Congenital Heart Surgeons' Society has performed research based on 12 Diagnostic Inception Cohorts. The first cohort (Transposition) began enrolling patients on January 1, 1985.

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The Congenital Heart Surgeons' Society (CHSS) was founded by 16 congenital heart surgeons in 1973, who endeavored to share their clinical advances in an informal setting that would stimulate honest and forthright discussions. As the Society grew, prospective studies were organized from a centralized data center that was established and based first in Birmingham, Alabama, thence to Toronto, and recently in a collaboration between Toronto and the Cleveland Clinic. These studies formed the basis for a myriad of outcomes reports that favorably impacted surgical results.

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The concept of a new organization for congenital heart surgeons in North America to discuss their difficult cases and offer each other potential solutions began in 1973. This article pays tribute to the founders of the Congenital Heart Surgeons' Society and offers insight into the desire of these surgeons to improve clinical outcomes for children with congenital heart disease.

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Background: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden death in youth. However, its significance and optimal management in adults is poorly understood. Our objective is to characterize AAOCA in a large single-center adult cohort based on coronary anatomic variants and surgical management strategies.

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Objectives: It remains unclear when sudden cardiac event risk outweighs surgical risk for patients with anomalous aortic origin of a coronary artery. The Congenital Heart Surgeons' Society sought to characterize the surgical risks by determining the techniques, complications, and outcomes of repair.

Methods: Between January 2000 and September 2018, 682 patients with anomalous aortic origin of a coronary artery aged 30 years or less were enrolled.

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Objectives: Improved survival has led to interest in functional health status (FHS) as patients with dextro-transposition of the great arteries (d-TGA) transition to adulthood. Our primary objectives were (1) evaluation of The Medical Outcomes Study Short Form-36 Health Survey (SF-36) results; (2) comparison with results of patients who completed the Child Health Questionnaire-Child Form 87 (CHQ-CF87) previously, or the PedsQL Generic Core Scales (PedsQL) survey subsequently; and (3) determination of factors associated with SF-36 domains.

Methods: Survivors from the d-TGA Congenital Heart Surgeons' Society cohort (1985-1989) completed the SF-36 (2010) as a measure of FHS (n = 210; age 21-26 years).

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Objectives: To analyze outcomes after pulmonary artery banding (PAB) in complete atrioventricular septal defect (AVSD), with a focus on surgical pathway outcome and timing, survival, and atrioventricular valve function.

Methods: PAB was performed in 50 of 474 infants (11%) from 28 institutions between 2012 and 2018 at a median age of 1.1 months.

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Background: Improved survival for patients with dextro-transposition of the great arteries (d-TGA) has led to an increased focus on functional health status (FHS). We assessed late survival and patient-perceived FHS for repaired TGA patients.

Methods: From 1985-1990, 830 neonates admitted to 24 Congenital Heart Surgeons' Society (CHSS) institutions with d-TGA underwent repair, including 516 arterial switch, 110 Mustard, 175 Senning, and 29 Rastelli operations.

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Objectives: We sought to determine anatomic features associated with evidence of myocardial ischemia and sudden cardiac events (arrest or death) for patients with anomalous aortic origin of a coronary artery.

Methods: We enrolled 560 patients, less than or equal to 30 years, at diagnosis from 40 institutions. Ischemia was defined as the presence of exertional syncope, a sudden cardiac event (arrest/death), or abnormal investigation results.

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Article Synopsis
  • Congenital heart surgery requires extensive training due to its complexity, leading to a strong need for enhanced training methods like simulation.
  • A literature review identified five relevant studies on hands-on surgical simulation for congenital heart surgery, focusing on evaluation of training effectiveness and simulator types.
  • While the studies generally support the effectiveness of simulation in training, they largely serve as proof of concept; future research should involve larger participant groups to verify improvements in surgical outcomes through repeated practice.
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Objective: This study sought to determine the prevalence, predictors, prognostic relevance and evolution of pulmonary hypertension (PH) (mean pulmonary artery pressure ≥25 mm Hg) in adult patients with a subaortic right ventricle (RV) in a biventricular circulation (2V-RV).

Methods: We analysed retrospective data from patients with 2V-RV undergoing cardiac catheterisation in our centre between 2000 and 2018. Echocardiographic assessment of subpulmonary ventricular pressures (left ventricular systolic pressure (LVSP)), age and B-type natriuretic peptide (BNP) were assessed as PH screening tools.

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Objectives: Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch.

Methods: Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141).

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Aims: The aims of this study were to provide a detailed descriptive analysis of pre-intervention morphologic and functional echocardiographic parameters in a large, unselected, multicentre cohort of neonates diagnosed with critical left heart obstruction and to compare echocardiographic features between the different subtypes of left-sided lesions.

Methods And Results: Pre-intervention echocardiograms for 651 patients from 19 Congenital Heart Surgeons' Society (CHSS) institutions were reviewed in a core lab according to a standardized protocol including >150 morphologic and functional variables. The four most common subtypes of lesions were: aortic atresia (AA)/mitral atresia (MA) (29% of patients), AA/mitral stenosis (MS) (20%), aortic stenosis (AS)/MS (26%), and isolated AS (iAS) (18%).

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Background: In infants requiring 3-stage single-ventricle palliation for hypoplastic left heart syndrome, attrition after the Norwood procedure remains significant. The effect of the timing of stage 2 palliation (S2P), a physician-modifiable factor, on long-term survival is not well understood. We hypothesized that an optimal interval between the Norwood and S2P that both minimizes pre-S2P attrition and maximizes post-S2P survival exists and is associated with individual patient characteristics.

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Anomalous aortic origin of the coronary arteries is associated with exercise-induced ischaemia, leading some physicians to restrict exercise in patients with this condition. We sought to determine whether exercise restriction was associated with increasing body mass index over time. From 1998 to 2015, 440 patients ⩽30 years old were enrolled into an inception cohort.

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Background: Mortality through single-ventricle palliation remains high and the effect of the timing of stage 2 palliation (S2P) is not well understood. We investigated current practice patterns in the timing of S2P across two professional societies and compared them to actual practice patterns from two databases of patients who underwent S2P.

Methods: A ten-question survey was distributed to the members of the Congenital Heart Surgeons' Society (CHSS) and the European Congenital Heart Surgeons' Association (ECHSA).

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Background: For neonates with critical left ventricular outflow tract obstruction (LVOTO), hybrid procedures are an alternative to the Norwood stage 1 procedure. Despite perceived advantages, however, outcomes are not well defined. Therefore, we compared outcomes after stage 1 hybrid and Norwood procedures.

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The development of congenital cardiac surgery required innovation by committed and talented individuals who integrated science, engineering and emerging medical knowledge while building a team of professionals dedicated to the care of patients. Life-long follow-up of these patients led to evolving management strategies to improve outcomes for neonates, infants, children and adults with congenital heart disease.

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Article Synopsis
  • This study compared echocardiographic reports with expert reviews and surgical findings in 159 patients with anomalous aortic origin of a coronary artery (AAOCA).
  • The research highlighted a lack of consistent protocols for diagnosing AAOCA, which is critical for identifying risks and planning surgical strategies due to its association with sudden death among young individuals.
  • A new imaging protocol was developed to improve diagnostic accuracy, addressing notable discrepancies in data reporting and enhancing the reliability between institutional reports and expert reviews.
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The Congenital Heart Surgeons' Society is a group of over 100 pediatric heart surgeons representing 72 institutions that specialize in the treatment of patients with congenital heart defects. The Society began in 1972 and incorporated as a not-for-profit charitable organization in 2004. It has become the face and voice of congenital heart surgery in North America.

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