Aspirin Nonresponsiveness in Congenital Heart Disease-Prevalence, Risk Factors, and Management.

Background: Aspirin is frequently utilized for antiplatelet therapy in children with congenital heart disease (CHD). Patients who are unresponsive to aspirin, as measured by aspirin reaction units (ARU), are at higher risk for thrombotic events. It is undetermined if dose modification of aspirin results in adequate responsiveness in these patients.

Navigating life with single-ventricle CHD: psychosocial needs across adulthood.

Objective: The population of adults with single-ventricle congenital heart disease (CHD) is growing. This study explores their lived experiences through an adult developmental psychology framework.

Methods: Individuals aged 18 and older with single-ventricle CHD participated in Experience Group sessions and 1:1 interviews.

Statistical process monitoring creates a hemodynamic trajectory map after pediatric cardiac surgery: A case study of the arterial switch operation.

Postoperative critical care management of congenital heart disease patients requires prompt intervention when the patient deviates significantly from clinician-determined vital sign and hemodynamic goals. Current monitoring systems only allow for static thresholds to be set on individual variables, despite the expectations that these signals change as the patient recovers and that variables interact. To address this incongruency, we have employed statistical process monitoring (SPM) techniques originally developed to monitor batch industrial processes to monitor high-frequency vital sign and hemodynamic data to establish multivariate trajectory maps for patients with d-transposition of the great arteries following the arterial switch operation.

Multidisciplinary Surgical Care for a Cervical Esophageal Duplication Cyst.

We present a case of a three-month-old male who presented with a cervical esophageal duplication cyst requiring early surgical intervention. The patient presented with feeding difficulties, poor weight gain, and respiratory distress. Due to the position of the cervical esophageal duplication cyst and airway compression, this unique case required a multidisciplinary surgical approach involving both otolaryngology and cardiothoracic surgery.

Incidence and Outcomes of Iatrogenic Complete Atrioventricular Block After Congenital Heart Surgery.

Background: Iatrogenic complete atrioventricular block (ICAVB) has long been noted as a major complication after congenital heart surgery (CHS), and it contributes to complex postoperative care and potentially affects patients' outcomes.

Methods: This study is a retrospective review of the Pediatric Health Information System database from January 1, 2004 to September 30, 2023. All patients who underwent The Society of Thoracic Surgeons benchmark procedures were included.

Revisiting the Role of Balloon Atrial Septostomy Prior to the Arterial Switch Operation.

Objectives: Patients with dextro-transposition of the great arteries (d-TGA) frequently undergo balloon atrial septostomy (BAS) prior to the arterial switch operation (ASO) to promote atrial-level mixing. Balloon atrial septostomy has inherent risks as an invasive procedure and may not always be necessary. This study revisits the routine utilization of BAS prior to ASO.

Impact of electrophysiologists at daily multidisciplinary report in a paediatric cardiac care unit.

Background: Paediatric cardiac electrophysiologists are essential in CHD inpatient care, but their involvement is typically limited to consultation with individual patients. In our integrated heart centre, an electrophysiologist reviews all cardiac inpatient telemetry over the preceding 24 hours and participates in daily multidisciplinary morning report. This study investigates the impact of the strategy of consistent, formalised electrophysiologist presence at multidisciplinary morning report.

Successful explantation of children from the Berlin Heart EXCOR® ventricular assist device: A systematic review.

Background: The Berlin Heart EXCOR® (BHE) can bridge children with severe heart failure to transplantation, but some are successfully weaned and spared transplantation. This study seeks to identify characteristics of children amenable to successful explantation with BHE support.

Methods: Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020 guidelines were used.

Anomalous Aortic Origin of a Coronary Artery.

Background: Although anomalous aortic origin of a coronary artery (AAOCA) is associated with risk of sudden cardiac arrest (SCA), there is a spectrum of disease, with the appropriate management for many remaining unclear. Increasing data warrant review for an updated perspective on management.

Methods: A panel of congenital cardiac surgeons, cardiologists, and imaging practitioners reviewed the current literature related to AAOCA and its management.

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used.

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used.

Outcomes of thoracic aortic interventions in Marfan syndrome in the state of Texas over 11 years.

Objectives: Marfan syndrome is a heritable connective tissue disorder with significant aortopathy and conveys substantial cardiovascular morbidity. This study characterizes the mortality and morbidities of thoracic aortic interventions (TAI) in the Marfan syndrome population in the state of Texas from 2009 to 2019.

Methods: A retrospective review of the Texas Inpatient Discharge Dataset from 1 January 2009 to 31 December 2019.

The journey of becoming a congenital heart surgeon: Too long, too costly, too unpredictable.

Objective: The pathway to become a congenital heart surgeon (CHS) is challenging and unpredictable. Previous voluntary manpower surveys have shed partial light on this problem but have not included all trainees. We believe that this arduous journey merits more attention.

Pacemaker Management and In-Hospital Outcomes in Neonatal Congenital Atrioventricular Block.

Background: Neonatal congenital atrioventricular block (nCAVB) is rare, causes bradycardia, confers high mortality, and frequently requires pacing. In-hospital outcomes and pacemaker management in nCAVB are limited.

Objectives: The purpose of this study was to analyze pacing and outcomes of nCAVB with and without congenital heart disease (CHD) using a multicenter database.

Evaluating variation in pre-operative evaluation and planning for children undergoing atrial or ventricular septal defect repair.

Background: CHD care is resource-intensive. Unwarranted variation in care may increase cost and result in poorer health outcomes. We hypothesise that process variation exists within the pre-operative evaluation and planning process for children undergoing repair of atrial septal defect or ventricular septal defect and that substantial variation occurs in a small number of care points.

Clinical Presentation and Medium-Term Outcomes of Children With Anomalous Aortic Origin of the Left Coronary Artery: High-Risk Features Beyond Interarterial Course.

Background: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death in children. Surgery is recommended for interarterial AAOLCA, and other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of 3 AAOLCA subtypes.

Ischemia in Anomalous Aortic Origin of a Right Coronary Artery: Large Pediatric Cohort Medium-Term Outcomes.

Background: Anomalous aortic origin of a right coronary artery may cause myocardial ischemia and sudden death in the young. Data on myocardial ischemia or longitudinal outcomes are sparse in pediatric anomalous aortic origin of a right coronary artery population.

Methods: Patients <21 years with anomalous aortic origin of a right coronary artery were prospectively enrolled.

Pericardial Effusions After the Arterial Switch Operation: A PHIS Database Review.

Pericardial effusion (PCE) is a significant complication after pediatric cardiac surgery. This study investigates PCE development after the arterial switch operation (ASO) and its short-term and longitudinal impacts. A retrospective review of the Pediatric Health Information System database.

Decision making in anomalous aortic origin of a coronary artery.

Introduction: There are many uncertainties surrounding anomalous aortic origin of a coronary artery (AAOCA) including the pathophysiology of sudden cardiac death, how to best risk stratify patients, how to best evaluate patients, who would benefit from exercise restriction, who should undergo surgical intervention, and which operation to perform.

Areas Covered: The goal of this review is to provide a comprehensive but succinct overview of AAOCA to help clinicians with the difficult task of navigating optimal evaluation and treatment of an individual patient with AAOCA.

Expert Opinion: Beginning in year 2012, some of our authors proposed an integrated, multi-disciplinary working group which has become the standard management strategy for patients diagnosed with AAOCA.