Pediatric Cardiology Practice Research Articles

Improvement Science Increases Routine Lipid Screening in General Pediatric Cardiology

ObjectiveTo evaluate the effectiveness of patient education, physician counseling, and point-of-care (POC) testing on improving adherence to lipid screening national guidelines in a general pediatric cardiology practice (2017-2023). Study designRegional primary care providers were surveyed regarding lipid screening practices. Key drivers were categorized (physician, patient, system) with corresponding interventions. Pediatric cardiologists started offering lipid screening during regular visits by providing families with preventive cardiovascular education materials and lab phlebotomy testing. System re-design included educational posters, clinical intake protocol, physician counseling, electronic health record integration, and POC testing. Run charts and statistical process control charts measured screening rates and key processes. ResultsThe primary care survey response rate was 32% (95/294); 97% supported pediatric cardiologists conducting routine lipid screening. Pediatric cardiology mean baseline lipid screening rate was 0%, increased to 7% with patient education, and to 61% after system redesign including POC testing. Screening rates among 1,467 patients were similar across age groups (P = 0.98). More patients received lipid screening by POC (91.7%) compared with phlebotomy (8.3%). Lipid abnormalities detected did not differ by screening methodology (P = 0.49). ConclusionPatient education, counseling, and POC testing improved adherence to national lipid screening guidelines, providing a possible model for primary care implementation.

MYOCARDITIS IN PEDIATRICS: A GROWING CONCERN FOR HEALTHCARE SYSTEMS IN PAKISTAN

Myocarditis, defined as an inflammation of the heart muscle, can pose significant challenges in pediatric cardiology due to its varied causes and potentially serious consequences. While there are several potential causes of myocarditis, for instance, autoimmune diseases and toxins, viral infections account for a large majority of cases in pediatric populations (Tschöpe et al., 2021). The frequency of myocarditis is still rather high due to the increased prevalence of viral diseases in children, especially during specific seasons; this puts a significant strain on hospital resources. Myocarditis in paediatric patients has consequences that go beyond the initial inflammatory insult to the heart. Patients affected with myocarditis can often exhibit acute heart failure, arrhythmias, and, in severe cases, cardiogenic shock (Ammirati et al., 2021). Moreover, long-term consequences can manifest as chronic heart failure, which necessitates continuous, ongoing monitoring and management to optimize cardiac function and quality of life (Upadhya and Kitzman, 2020). We have noticed a worrying trend in our pediatric cardiology practice at Lady Reading Hospital Peshawar, whereby approximately half of the patients who consult our outpatient and inpatient departments have left ventricular failure. A significant portion of these cases are diagnosed as viral myocarditis. We have noticed a worrying trend in our pediatric cardiology practice: about 50% of the cases that come into our outpatient and inpatient departments have left ventricular failure, and a sizable percentage of those cases are diagnosed with myocarditis. This finding not only highlights the frequency of myocarditis but also highlights the pressing need for increased awareness, prompt diagnosis, and effective management of viral myocarditis in order to reduce negative consequences in vulnerable pediatric populations. In resource-poor environments like Pakistan managing myocarditis can unique challenges. Treatment methods such as IV immunoglobulin treatment which can have proven success in some patients, are generally expensive and not readily available (Ammirati et al., 2021). This gap in access to modern treatments can further exacerbate the load on healthcare systems that are already strained by the high prevalence of myocarditis. In low and middle-income countries (LMICs) like Pakistan, proactive preventative interventions are especially important to reduce the strain on already overburdened healthcare systems. The increased frequency of myocarditis in pediatric populations can be decreased by highlighting the significance of vaccination against common viral infections linked to the illness such as adenovirus and enterovirus (Makimaa et al., 2020; Upadhya and Kitzman, 2020). In addition, raising awareness among caregivers and medical professionals about the early detection of myocarditis symptoms and the importance of promptly referring patients to specialized clinics will help to expedite intervention and enhance long-term results (Inoue et al., 2019). In summary, the growing incidence of myocarditis in pediatric patients demands a multimodal strategy that includes early detection, prevention, and complete therapeutic techniques. By addressing these issues collectively and pushing for fair access to key treatments, we can seek to lessen the impact of myocarditis on pediatric cardiac health and alleviate the load on healthcare systems, particularly in resource-limited settings.

FONTAN CIRCULATION AND PREGNANCY: IS IT POSSIBLE

More than 85% of congenital heart disease in children survive to adulthood according to recent advances in pediatric cardiology and heart surgery practice. Fifty percent of this population is made up of women, most of whom have reached child bearing age. In this group of patients, pregnancy represents a new challenge, whose natural history has been modified by surgery. In Fontan circulation, the ventricle unable to compensate for the major restriction in the bottleneck and non physiological suction have to pull blood through the lungs, although the ventricles provide the driving force for the circuit. Women with an adequate Fontan circulation can become pregnant without clinically significant long term sequele. Pre-marital and pre-pregnancy consultation is important. A close collaboration between cardiologist, obstetrician and anaesthesiologist is mandatory in order to get the best outcome of pregnancy.

Toward a Roadmap for Best Practices in Pediatric Preventive Cardiology: A Science Advisory From the American Heart Association.

Cardiovascular disease risk factors are highly prevalent among youth in the United States and Canada. Pediatric preventive cardiology programs have independently developed and proliferated to address cardiovascular risk factors in youth, but there is a general lack of clarity on best practices to optimize and sustain desired outcomes. We conducted surveys of pediatric cardiology division directors and pediatric preventive cardiology clinicians across the United States and Canada to describe the current landscape and perspectives on future directions for the field. We summarize the data and conclude with a call to action for various audiences who seek to improve cardiovascular health in youth, reduce the burden of premature cardiovascular disease, and increase healthy longevity. We call on heart centers, hospitals, payers, and policymakers to invest resources in the important work of pediatric preventive cardiology programs. We urge professional societies to advocate for pediatric preventive cardiology and provide opportunities for training and cross-pollination across programs. We encourage researchers to close evidence gaps. Last, we invite pediatric preventive cardiology clinicians to collaborate and innovate to advance the practice of pediatric preventive cardiology.

Ethical side of decision-making on palliative care to a child with congenital heart disease associated with frontonasal dysplasia

In the practice of pediatric cardiology, situations are possible when a child with congenital heart disease cannot be subjected to radical or even hemodynamic surgery. As a rule, such severe heart defects are combined with other genetic anomalies, which ultimately lead to the palliative status of the newborn. This situa­tion is a serious ethical and psychological challenge for medical workers in both the antenatal and postnatal periods. In the proposed article, we present a case of a child with congenital heart disease, which was part of frontonasal dysplasia due to ZSWIM6 gene mutation. This observation illustrates a number of ethical and psychological problems, difficult decisions and communication questions that are typical for such a difficult life situation as the birth of a terminally ill child. The example is illustrative in general for the ethical problems of a disabling congenital disease, which can be encountered in the practice of a pediatric cardiologist. From a broader perspective, the example illustrates uncertainty as an inevitable component of medical decision making and informing the patient (his legal representatives).

Patient-Specific 3D-Printed Models in Pediatric Congenital Heart Disease

Three-dimensional (3D) printing technology has become increasingly used in the medical field, with reports demonstrating its superior advantages in both educational and clinical value when compared with standard image visualizations or current diagnostic approaches. Patient-specific or personalized 3D printed models serve as a valuable tool in cardiovascular disease because of the difficulty associated with comprehending cardiovascular anatomy and pathology on 2D flat screens. Additionally, the added value of using 3D-printed models is especially apparent in congenital heart disease (CHD), due to its wide spectrum of anomalies and its complexity. This review provides an overview of 3D-printed models in pediatric CHD, with a focus on educational value for medical students or graduates, clinical applications such as pre-operative planning and simulation of congenital heart surgical procedures, and communication between physicians and patients/parents of patients and between colleagues in the diagnosis and treatment of CHD. Limitations and perspectives on future research directions for the application of 3D printing technology into pediatric cardiology practice are highlighted.

Preventive medicine in pediatric cardiology practice.

The objective of the study was to assess contemporary practice patterns of pediatric cardiologists with respect to cholesterol disorders and smoking-related illness. We sent 2 anonymous surveys to the members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and the Pediheart online community. The surveys addressed training in and management of cholesterol disorders and smoking-related illness. There were 97 responses to the cholesterol disorders survey. A total of 51.6% reported little or no formal training on cholesterol disorders. A total of 56.4% underestimated the prevalence of familial hypercholesterolemia by at least twofold. A total of 54.7% were at least somewhat comfortable prescribing statins. In 5 clinical vignettes, respondents frequently gave clinical recommendations in line with the 2019 American Heart Association guidelines although both undertreatment and overtreatment were recommended. There were 90 responses to the survey on smoking-related illness. Little or no formal training in nicotine addiction (52.3%) or smoking cessation (60.5%) was reported by respondents. Respondents screened for tobacco use in less than a one-third of hospitalizations and less than two-thirds of outpatient clinic visits. Screening for exposure to secondhand smoke was even less common. Twenty-seven percent of respondents never recommend a household smoking ban for their patients. A total of 83.3% were uncomfortable prescribing medications for their patients for smoking cessation, and 65.5% rarely or never refer patients for smoking cessation assistance. Although positioned to address the childhood origins of adult heart disease, those cardiologists surveyed placed a limited emphasis on cholesterol disorders and smoking-related disease in their clinical practice.

Pediatric Cardiology Fellowship Standards for Training in Exercise Medicine and Curriculum Outline.

Over the past 2 decades, fundamentals of exercise medicine, including clinical exercise testing, assessment and promotion of physical activity, exercise prescription, and supervised exercise training/rehabilitation programming have demonstrated considerable clinical value in the management of children and adolescents with congenital and acquired heart disease. Although the principles of exercise medicine have become an integral component in pediatric cardiology, there are no standardized training recommendations for exercise physiology during pediatric cardiology fellowship at this time. Thus, the Pediatric Cardiology Exercise Medicine Curriculum Committee (PCEMCC) was formed to establish core and advanced exercise physiology training recommendations for pediatric cardiology trainees. The PCEMCC includes a diverse group of pediatric cardiologists, exercise physiologists, and fellowship program directors. The expert consensus training recommendations are by no means a mandate and are summarized herein, including suggestions for achieving the minimum knowledge and training needed for general pediatric cardiology practice.

Clinical case of complicated course of infectious endocarditis in a teenager

Infective endocarditis (IE) is a heart disease of an infectious nature with a primary lesion of the endocardium, heart valves, ligaments, followed by dissemination of the process to various organs. Infective endocarditis still remains a disease that is difficult to diagnose and unpredictable in terms of outcomes; it is registered in all countries of the world. Despite all the advances in medicine, infective endocarditis remains an extremely dangerous disease, with a mortality rate of 10–30%. The most common causes of lethal outcome of IE are multiple complications: embolisms of various localization, including cerebral arteries, aneurysms, toxic lesions of the spleen, myocarditis and pericarditis, acute renal failure. Currently, in pediatric cardiology practice, the frequency of primary infective endocarditis, which occurs with a change in the clinical picture, its atypical variants, has increased. The awareness of doctors of all specialties about this problem, the correct and timely diagnosis of the disease allows prescribing adequate therapy earlier and thereby improves the prognosis. The aim of our work was to familiarize physicians with the clinical observation of a teenager with an atypical onset of primary infective endocarditis, occurring under the “mask” of acute cerebrovascular accident. Also, the peculiarity of this case was the primary diagnosis of hereditary thrombophilia in the patient and the possible debut of a demyelinating disease of the central nervous system during the examination, which certainly complicated the diagnostic search and affected the outcome of infective endocarditis. The significance of dental procedures preceding the disease in a child, unfortunately, was not properly appreciated by the parents, but it is the inflammatory process in the oral cavity that should be considered as a key etiological factor in the development of infective endocarditis in this case.

The impact of COVID-19 on the practice of pediatric cardiology: a narrative review

Background and Objective: Coronavirus disease 2019 (COVID-19), caused by the severe acute respiratory syndrome virus 2 (SARS-CoV-2), is a global pandemic evolving in real time and affecting persons of all ages. Contemporary pediatric data demonstrate that children of all ages are susceptible to SARS-CoV-2, and that infants under 1 year of age are at risk for severe disease although this still is a relatively rare outcome. This narrative overview discusses the impact of the COVID-19 pandemic on various aspects of pediatric cardiology, and proposes strategies to mitigate disruptions, so as to better future-proof our practice.

Impact of Technologic Innovation and COVID-19 Pandemic on Pediatric Cardiology Telehealth.

Purpose of ReviewEstablished telehealth practices in pediatrics and pediatric cardiology are evolving rapidly. This review examines several concepts in contemporary telemedicine in our field: recent changes in direct-to-consumer (DTC) pediatric telehealth (TH) and practice based on lessons learned from the pandemic, scientific data from newer technological innovations in pediatric cardiology, and how TH is shaping global pediatric cardiology practice.Recent FindingsIn 2020, the global pandemic of COVID-19 led to significant changes in healthcare delivery. The lockdown and social distancing guidelines accelerated smart adaptations and pivots to ensure continued pediatric care albeit in a virtual manner. Remote cardiac monitoring technology is continuing to advance at a rapid pace secondary to advances in the areas of Internet access, portable hand-held devices, and artificial intelligence.SummaryTH should be approached programmatically by pediatric cardiac healthcare providers with careful selection of patients, technology platforms, infrastructure setup, documentation, and compliance. Payment parity with in-person visits should be advocated and legislated. Newer remote cardiac monitoring technology should be expanded for objective assessment and optimal outcomes. TH continues to be working beyond geographical boundaries in pediatric cardiology and should continue to expand and develop.

Health Care Disparities in Congenital Cardiology: Considerations Through the Lens of an Interventional Cardiologist

<h2>Abstract</h2> When resources in a society are dispersed unevenly, generally through allocation standards, distinct patterns emerge along lines of socially defined categories of people. Power, religion, kinship, prestige, race, ethnicity, gender, age, sexual orientation, and class all play a role in determining who has access to social goods in society. In most cases, social inequality refers to a lack of equality of outcome, but it can also refer to a lack of equality of access to opportunity. Unfortunately, health care is not immune to these social disparities and/or inequalities. These health care disparities in interventional cardiology were recently brought to the forefront by the Society for Cardiovascular Angiography and Interventions (SCAI) as a major focus of 2020-2021. In a recent publication, unique factors leading to disparities were reported to exist among the subsections of interventional cardiology. The congenital heart disease council of SCAI created a task force to further investigate the unique challenges and disparities impacting the practice of congenital heart disease and pediatric cardiology.

Adaptations of paediatric cardiology practice during the COVID-19 pandemic.

During the initial surge of the COVID-19 pandemic in the spring and summer of 2020, paediatric heart centres were forced to rapidly alter the way patient care was provided to minimise interruption to patient care as well as exposure to the virus. In this survey-based descriptive study, we characterise changes that occurred within paediatric cardiology practices across the United States and described provider experience and attitudes towards these changes during the pandemic. Common changes that were implemented included decreased numbers of procedures, limiting visitors and shifting towards telemedicine encounters. The information obtained from this survey may be useful in guiding and standardising responses to future public health crises.

Validating a risk assessment tool in United Kingdom and Irish paediatric cardiac catheterisation practice.

No established risk prediction tool exists in United Kingdom and Irish Paediatric Cardiology practice for patients undergoing cardiac catheterisation. The Catheterisation RISk score for Paediatrics is used primarily in North American practice to assess risk prior to cardiac catheterisation. Validating the utility and transferability of such a tool in practice provides the opportunity to employ an already established risk assessment tool in everyday practice. To ascertain whether the Catheterisation RISk score for Paediatrics assessment tool can accurately predict complications within United Kingdom and Irish congenital catheterisation practice. Clinical and procedural data including National Institute for Cardiovascular Outcomes Research derived outcome data from 1500 patients across five large congenital cardiology centres in the United Kingdom and Ireland were retrospectively collected. Catheterisation RISk score for Paediatrics were then calculated for each case and compared with the observed procedural outcomes. Chi-square analysis was used to determine the relationship between observed and predicted events. Ninety-eight (6.6%) patients in this study experienced a significant complication as qualified by National Institute for Cardiovascular Outcomes Research classification. 4% experienced a moderate complication, 2.3% experienced a major complication and 0.3% experienced a catastrophic complication resulting in death. Calculated Catheterisation RISk score for Paediatrics scores correlated well with all observed adverse events for paediatric patients across all CRISP categories. The association was also transferable to adult congenital heart disease patients in lower Catheterisation RISk score for Paediatrics categories (CRISP 1-3). The Catheterisation RISk score for Paediatrics score accurately predicts significant complications in congenital catheterisation practice in the United Kingdom and Ireland. Our data validated the Catheterisation RISk score for Paediatrics assessment tool in five congenital centres using National Institute for Cardiovascular Outcomes Research-derived outcome data.

Improving Compliance with Dyslipidemia Screening Guidelines in a Single-center U.S. Outpatient Pediatric Cardiology Clinic.

Introduction:The development of atherosclerotic cardiovascular disease begins in childhood. The American Academy of Pediatrics (AAP) endorsed guidelines recommending universal hyperlipidemia screening of children ages 9–11 and again at 17–21 years. An AAP Periodic Survey of Fellows demonstrated less than half of pediatricians report adherence to these guidelines. This quality improvement initiative’s objective was to improve compliance with AAP hyperlipidemia guidelines in an outpatient pediatric cardiology clinic at a single academic center to 80% over a 2-month time frame.Methods:We report the results of an IRB-approved chart review at a single-center outpatient pediatric cardiology practice. We defined pediatric cardiologists’ compliance as documented prior lipid screening, ordering a lipid panel, or documented recommendation for follow-up screening. Two plan-do-study-act (PDSA) cycles were undertaken. The first intervention included an informational session to provide pediatric cardiologists with AAP recommendations. The second intervention involved weekly email reminders and a statement for physicians in the electronic medical record.Results:We collected data from 600 individual charts of patients seen over 35 clinic days. We received charts before the first PDSA intervention. Baseline compliance with outpatient hyperlipidemia screening was 0%. After the first PDSA cycle, the average screening rate improved to 49%. After the second PDSA cycle, the average screening rate improved to 89%, and there was a centerline shift in the data, indicating improvement.Conclusion:We improved the pediatric cardiologists’ compliance with the AAP-recommended hyperlipidemia screening guidelines from 0% to 89% through 2 intervention cycles. Further efforts may be required to sustain this change.

#81: Care Process Measures and Outcomes of Percutaneous Pulmonary Valve Implantation-associated Infective Endocarditis

Abstract Background Percutaneous pulmonary valve implantation (PPVI) is being increasingly used as a minimally invasive corrective procedure for right ventricular outflow tract (RVOT) dysfunction. Ten-year survival following PPVI is estimated at over 90% due to the durability of the various bioprosthetic valves. However, infective endocarditis (IE) remains a potential complication of such valves with significant morbidity and mortality. We evaluated the presenting symptoms, clinical features, pathogens, and outcomes of patients with IE following PPVI to identify opportunities to improve early diagnosis and management. Methods A convenience sample of patients at a large Pediatric Cardiology practice in Northern Virginia was queried for PPVI and IE from January 1, 2016, to June 30, 2019. Manual chart review was done to extract clinical points of interest and descriptive analyses were performed. Patients were classified as having IE per modified Duke’s criteria. Results We identified 14 patients who underwent PPVI. Five of these patients (36%) developed IE. All IE patients had underlying Tetralogy of Fallot and none had previous episodes of IE. 60% of patients with IE were male with a median age of 26 years old (IQR 20–30). Four IE patients had a Melody valve and 1 had a SAPIEN valve. The median elapsed time between PPVI and IE diagnosis was 128 days (IQR 32–391) with a median duration of illness prior to the diagnosis of IE of 6 days (IQR 5–9). All IE patients had to present fever. 40% of IE patients had to present chest pain and 20% had presenting musculoskeletal pain. All IE patients had an elevated initial C-reactive protein (CRP) with a median value of 13.1 mg/dL (IQR 12.5–15.2). The median initial white blood cell count was 9.3 × 103/μL (IQR 8.1–10.3). The median duration of bacteremia was 1 day (IQR 1–2). A pathogen was recovered in all five IE patients with different organisms amongst the patients: coagulase-negative Staphylococcus species were recovered in patients who developed IE within 60 days from PPVI (Staphylococcus lugdunensis and Staphylococcus epidermidis) whereas coagulase-negative Staphylococcus species and oral commensal organisms were found in IE patients beyond 60 days from PPVI (Staphylococcus sanguinis, Gamella haemolysans, and Neisseria elongata). The initial echocardiogram did not show vegetations in any of the patients and 40% went on to have sternotomy with valve replacement. There were no deaths. Conclusions With an increase in PPVI, clinicians should have a high index of suspicion for IE in patients with underlying Tetralogy of Fallot who present with fever and elevated CRP, regardless of elapsed time from PPVI or valve type. Empiric antimicrobial therapy for suspected IE following PPVI should remain broad with other possible pathogens beyond coagulase-negative Staphylococcus species.

Atrioventricular Septal Defect: What Is in a Name?

Robert Anderson has made a huge contribution to almost all aspects of morphology and understanding of congenital cardiac malformations, none more so than the group of anomalies that many of those in the practice of paediatric cardiology and adult congenital heart disease now call ‘Atrioventricular Septal Defect’ (AVSD). In 1982, with Anton Becker working in Amsterdam, their hallmark ‘What’s in a name?’ editorial was published in the Journal of Thoracic and Cardiovascular Surgery. At that time most described the group of lesions as ‘atrioventricular canal malformation’ or ‘endocardial cushion defect’. Perhaps more significantly, the so-called ostium primum defect was thought to represent a partial variant. It was also universally thought, at that time, that the left atrioventricular valve was no more than a mitral valve with a cleft in the aortic leaflet. In addition to this, lesions such as isolated cleft of the mitral valve, large ventricular septal defects opening to the inlet of the right and hearts with straddling or overriding tricuspid valve were variations of the atrioventricular canal malformation. Anderson and Becker emphasised the differences between the atrioventricular junction in the normal heart and those with a common junction for which they recommended the generic name, ‘atrioventricular septal defect’. As I will discuss, over many years, they continued to work with clinical cardiologists and cardiac surgeons to refine diagnostic criteria and transform the classification and understanding of this complex group of anomalies. Their emphasis was always on accurate diagnosis and communication, which is conveyed in this review.

203 Racial and ethnic disparities among diagnosed critical congenital heart disease

Critical congenital heart disease (CCHD) is defined as structural disease requiring surgery or catheter intervention during the first 30 days of life, can be diagnosed on prenatal ultrasound. Prenatal diagnosis can reduce morbidity and mortality. The purpose of this study was to determine if race and socioeconomic status (SES) impacts the rate of prenatal diagnosis of CCHD. We conducted a retrospective chart review of neonates at a single, tertiary pediatric cardiology practice from 2007-2018 with a diagnosis of CCHD. Neonates with prenatal diagnosis were compared to neonates diagnosed postnatally by: race/ethnicity, SES and infant mortality. SES was assessed using primary insurance and income quartile by zip code to provide estimated median household income. Seven diagnoses were targeted and divided into three groups based on prenatal ultrasound views that would be expected to make the diagnosis: four chamber, outflow tract, and other views. Data were analyzed using Fisher’s exact test and pairwise comparison if the Fischer’s test was statistically significant. A p-value < 0.05 was statistically significant. We identified 185 cases of CCHD; 102(55.2%) diagnosed prenatally, 83 (44.8%) diagnosed postnatally. Only three ethnic groups had adequate cases for analysis. Rate of prenatal diagnosis did not differ significantly by race/ethnicity (p=0.411) nor did postnatal diagnosis (p=0.499) (Table 1). Rates of prenatal or postnatal diagnosis also did not differ significantly by parental SES income (p=0.706). Infant mortality was higher in Blacks & Hispanics vs. Caucasians (p < 0.001) (Table 1 and 2). Infant mortality prior to 1 year of age was significantly associated with parental SES income: 71% had an annual salary <$50,000 vs. 27% (p <0.001; Table 2). Rate of prenatal detection of CCHD did not differ by race/ethnicity and SES. However, Black and Hispanic neonates had significantly higher infant mortality rates vs. Caucasian neonates. Parental SES was significantly lower in cases of infants who died in the first year of life.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

First-Degree Relatives Screening of Patients with Bicuspid Aortic Valve: Effectiveness and Feasibility in Pediatric Cardiology Daily Practice.

Bicuspid aortic valve (BAV) is the most common congenital heart disease. Since heritability is suspected, actual guidelines recommend to perform an echocardiographic assessment for first-degree relatives (FDR) of patient with BAV. This study aimed to assess the effectiveness and the feasibility of the current guidelines for the screening of FDR of patient with BAV in a pediatric cardiology daily practice. Consecutive patients with BAV and their FDR were prospectively included from January 2015 to March 2018 at Centre Hospitalier Universitaire de Laval, Quebec City (Canada). Data were retrospectively collected and analyzed. A total of 713 FDR of 213 consecutives index cases [median age: 11 (6-20) years] were studied. Up to 32 (6.6%) FDR had a BAV and 26 (5.4%) had an aortic valve dysfunction. A total of 14 (2.9%) FDR had an ascending aorta dilatation according to Z-score including 6 (1.2%) patients with an ascending aorta ≥ 45mm. No statistically significant differences regarding BAV, aortic valve dysfunction and ascending aorta dilatation prevalence were identified between generations. Screening was done in 482 (67.6%), prescribed but not done in 134 (19%), not prescribed in 92 (13%) and declined in 5 (1%) FDR. The prevalence of BAV in FDR was similar to prospective adult studies and supports actual guidelines in pediatric cardiology practice. Ascending aorta dilatation was rare in our young population. Exhaustiveness and additional burden to implement current guidelines remain a challenge in daily practice.

The "Mother of pediatric cardiac anesthesia": An interview with Dr Dolly D. Hansen, a pioneering woman in medicine.

Dr Dolly D. Hansen (1935-), Associate Professor in the Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital and Harvard Medical School, devoted her life to improving the perioperative care of children with congenital heart disease. She applied her knowledge of cardiovascular and pulmonary physiology and the effects on anesthetic agents in children with and without heart disease into clinical practice and thereby greatly influenced the practice of pediatric anesthesia, cardiology, surgery, and critical care medicine. As an exceptional master clinician, leader, program builder, innovator, teacher, and academic role model, she shaped the careers of hundreds of fellows and young attendings, many of whom became leaders in the field.