Utility congenital heart disease

Congenital heart disease (CHD) is the most common birth defect and a leading cause of infant mortality. CHD often has a genetic etiology and recent studies demonstrate utility in genetic testing. In clinical practice, decisions around genetic testing choices continue to evolve, and the incorporation of rapid genome sequencing (rGS) in CHD has not been well studied. Though smaller studies demonstrate the value of rGS, they also highlight the burden of results interpretation. We analyze genetic testing in CHD at two time-points, in 2018 and 2022-2023, across a change in clinical testing guidelines from chromosome microarray (CMA) to rGS...

The cardiovascular development in vertebrates evolves in response to genetic and mechanical cues. The dynamic interplay among mechanics, cell biology, and anatomy continually shapes the hydraulic networks, characterized by complex, non-linear changes in anatomical structure and blood flow dynamics. To better understand this interplay, a diverse set of molecular and computational tools has been used to comprehensively study cardiovascular mechanobiology. With the continual advancement of computational capacity and numerical techniques, cardiovascular simulation is increasingly vital in both basic science research for understanding developmental mechanisms and disease etiologies, as well as in clinical studies aimed at enhancing treatment outcomes...

Computed tomography (CT) has emerged as a leading imaging modality in the evaluation of congenital heart disease (CHD). With ever-faster acquisition speed, decreasing radiation exposure, impeccable anatomic detail, optional functional data, and numerous post-processing tools, CT offers broad utility in CHD diagnosis, preoperative planning, and postoperative assessment. In this article, the far-reaching role of CT in CHD is reviewed, focusing on technical imaging considerations and key clinical applications.

PURPOSE: The current study aimed to determine the prevalence of specific psychiatric disorders, identify predictors associated with these disorders, and assess the quality of life (QoL) among children with congenital heart disease. METHODS: This comparative cross-sectional study was conducted in the National Heart Institute outpatient clinics. It included 204 children with structural congenital heart defects (CHD). In addition to assessing QoL with the Pediatric Quality of Life Inventory scale, the Mini-International Neuropsychiatric Interview for Children and Adolescents was utilized to identify psychiatric disorders in the children studied...

In September 2023, the European Society of Cardiology (ESC) published new guidelines for the management of endocarditis. Infective endocarditis (IE) remains a significant life-threatening disease, concerning an increasingly younger age group, especially children with congenital heart disease (CHD) and young adults after multiple cardiac surgeries. This study's aim was to alert the medical community to the problem of increasing IE case numbers and IE course complexity. Of the eight patients who suffered from IE treated in 2023 in our department, we describe the four whose course was the most extraordinary...

AIMS: Slow conduction (SC) anatomical isthmuses (AIs) are the dominant substrate for monomorphic ventricular tachycardia (VT) in patients with repaired tetralogy of Fallot (rTF). This study aimed to evaluate the utility of automated propagational analysis for the identification of SC-AI in patients with rTF. METHODS AND RESULTS: Consecutive rTF patients undergoing VT substrate characterization were included. Automated isochronal late activation maps (ILAM) were obtained with multielectrode HD Grid Catheter...

BACKGROUND: Respiratory Syncytial Virus (RSV) infections may lead to severe consequences in infants born preterm with breathing problems (such as bronchopulmonary dysplasia (BPD) and respiratory distress syndrome (RDS)) or congenital heart diseases (CHD). Since studies investigating the influence of different gestational age (WGA) and concomitant specific comorbidities on the burden of RSV infections are scarce, the present study aimed to better characterize these high-risk populations in the Italian context...

• Large uncorrected PDA may first be diagnosed during pregnancy. • Multimodality imaging may be safely utilized in these patients. • Multimodality imaging is safely utilized in these patients. • Multidisciplinary team approach is key for percutaneous closure of PDA in pregnancy.

The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (CHSD) continues to be the most comprehensive database of congenital and pediatric cardiothoracic surgical procedures in the world and contains information on 664,210 operations as of June 30, 2023. The thirty-fifth harvest of the STS CHSD data was undertaken in Spring 2023 spanning the 4-year period January 1, 2019, through December 31, 2022, and included 144,919 operations performed at 114 participating sites in North America. The harvest analysis was successfully executed by the STS research and analysis center...

BACKGROUND: Global longitudinal strain (GLS) imaging is a multifaceted modality that has been utilized in various fields of clinical cardiology in the recent past; however, its implementation for the assessment of ischemia has been limited. OBJECTIVES: This study aimed to document the functional changes in GLS secondary to acute myocardial ischemia in patients with chronic chest pain. METHODS: In this unblinded, single-center, investigator-initiated, prospective pilot study, the functional changes in GLS at baseline, during, and immediately following coronary percutaneous intervention were monitored in 10 ambulatory patients who underwent elective catheterization...

INTRODUCTION/OBJECTIVE: Extubation failure in pediatric patients with congenital or acquired heart diseases increases morbidity and mortality. This study aimed to develop a clinical risk score for predicting extubation failure to guide proper clinical decision-making and management. METHODS: We conducted a retrospective study. This clinical prediction score was developed using data from the Pediatric Cardiac Intensive Care Unit (PCICU) of the Faculty of Medicine, Chiang Mai University, Thailand, from July 2016 to May 2022...

Tracheal stenosis is a rare but life-threatening disease in preterm infants. Misdiagnosis as congenital tracheal stenosis is common, making surgical management challenging. This report presents a case of a preterm infant with tracheal stenosis and congenital heart malformation treated with ECMO-assisted tracheal resection and end-to-end anastomosis. A male infant was born at 30 weeks of gestation with severe asphyxia, cardiac insufficiency, and pneumonia. Following failed medical treatment, fiberoptic bronchoscopy confirmed mid-tracheal to carinal stenosis...

BACKGROUND: Free-running cardiac and respiratory motion-resolved whole-heart 5D MRI can reduce scan planning and provide a means of evaluating respiratory-driven changes in clinical parameters of interest. However, respiratory-resolved imaging can be limited by user-defined parameters which create trade-offs between residual artifact and motion blur. In this work, we develop and validate strategies for both correction of intra-bin and compensation of inter-bin respiratory motion to improve the quality of 5D MRI...

OBJECTIVE: To determine the live born prenatal detection rate of significant congenital heart disease (CHD) in a large, integrated, multi-center community-based health system using a strategy of referral only of patients with significant cardiac abnormalities on obstetrical screening ultrasound for fetal echocardiography. Detection rates were assessed for screening in both radiology and maternal fetal medicine (MFM). The impact on fetal echocardiography utilization was also assessed. METHODS: This was a retrospective cohort study using an electronic health record, outside claims databases and chart review to determine all live births between 2016 and 2020 with postnatally confirmed sCHD that were prenatally detectable and resulted in cardiac surgery, intervention, or death within 1 year...

Early cardiac surgery in neonates and infants with congenital heart disease has been performed since the middle to late years of the twentieth century. To date, there are very few reports of successful congenital heart surgery using cardiopulmonary bypass (CPB) in premature babies less than 1000 g with serious congenital heart disease. Limited information is available in the literature describing perfusion techniques for this extremely fragile patient population. Miniaturization of the CPB circuit contributes to multiple factors that affect this population significantly...

Adults with congenital heart disease (CHD) benefit from cardiology follow-up at recommended intervals of ≤ 2 years. However, benefit for children is less clear given limited studies and unclear current guidelines. We hypothesize there are identifiable risks for gaps in cardiology follow-up in children with CHD and that gaps in follow-up are associated with differences in healthcare utilization. Our cohort included children < 10 years old with CHD and a healthcare encounter from 2008 to 2013 at one of four North Carolina (NC) hospitals...

BACKGROUND: Junctional ectopic tachycardia (JET) complicates congenital heart surgery in 2-8.3% of cases. JET is associated with postoperative morbidity in single-center studies. We utilized the Pediatric Cardiac Critical Care Consortium data registry for the first multicenter epidemiologic description of treated JET. METHODS: This is a retrospective study (2/2019 - 8/2022) of patients with treated JET. INCLUSION: 1) <12 months old at index operation; 2) treated for JET <72 hours after surgery...

AIMS: To examine determinants of access to treatment, outcomes and hospital utilization in patients undergoing secundum atrial septal defect (ASD) closure in adulthood in England and Wales. METHODS AND RESULTS: Large retrospective cohort study of all adult patients undergoing secundum ASD closures in England and Wales between 2000/01 and 2016/17. Data were from population-based official data sets covering congenital heart disease procedures, hospital episodes and death registries...

Background: Coarctation of the aorta can be associated with significant hypoplasia of the aortic arch. In contrast to patch aortoplasty, ascending sliding arch aortoplasty uses viable autologous tissue for potential growth in children. We reviewed the mid- to long-term outcomes of this technique. Methods: Between 2002 and 2023, 28 patients underwent ascending sliding arch aortoplasty for the patients with coarctation of the aorta (n = 22) and interrupted aortic arch (n = 2). Four patients underwent previous surgical coarctation repair at other institutions...

Already a challenging condition to define, adult congenital heart disease (ACHD) -associated heart failure (HF) often incorporates specific anatomies, including intracardiac and extracardiac shunts, which require rigorous diagnostic characterization and heighten the importance of clinicians proactively considering overall hemodynamic impacts of using specific therapies. The presence of elevated pulmonary vascular resistance dramatically increases the complexity of managing patients with ACHD-HF. Total circulatory management in patients with ACHD-HF requires input from multidisciplinary care teams and thoughtful and careful utilization of medical, interventional, and surgical approaches...