Are paediatric cardiology textbooks obsolete in the current digital era?

INTRODUCTION: With the rise of online references, podcasts, webinars, self-test tools, and social media, it is worthwhile to understand whether textbooks continue to provide value in medical education, and to assess the capacity they serve during fellowship training. METHODS: A prospective mixed-methods study based on surveys that were disseminated to seven paediatric cardiology fellowship programmes around the world. Participants were asked to read an assigned chapter of Anderson's Pediatric Cardiology 4th Edition textbook, followed by the completion of the survey. Open-ended questions included theming and grouping responses as appropriate. RESULTS: The survey was completed by 36 participants. When asked about the content, organisation, and utility of the chapter, responses were generally positive, at greater than 89%. The chapters, overall, were rated relatively easy to read, scoring at 6.91, with standard deviations plus or minus 1.72, on a scale from 1 to 10, with higher values meaning better results. When asked to rank their preferences in where they obtain educational content, textbooks were ranked the second highest, with in-person teaching ranking first. Several themes were identified including the limitations of the use of textbook use, their value, and ways to enhance learning from their reading. There was also a near-unanimous desire for more time to self-learn and read during fellowship. CONCLUSIONS: Textbooks are still highly valued by trainees. Many opportunities exist, nonetheless, to improve how they can be organised to deliver information optimally. Future efforts should look towards making them more accessible, and to include more resources for asynchronous learning.

Exploring the Historical Context of American Indian/Alaska Native Intensive Care Inequities: A Narrative Review.

INTRODUCTION: This narrative review aims to frame the historical context of American Indian/Alaska Native (AI/AN) pediatric intensive care and offers suggestions for mitigating the impact of unique social drivers. METHODS: Recent literature was surveyed to determine pertinent studies describing intensive care outcomes in AI/AN children and was summarized in a narrative review. RESULTS: American Indian/Alaska Native people experience disproportionate health inequites due to unique social drivers of health, including settler colonialism, historical trauma, and systemic racism. These factors contribute to inequities in the pediatric intensive care experience, including rates of admission for injury and infectious diseases and mortality due to injuries and following cardiac surgery. DISCUSSION: These inequities are understudied and require dedicated evaluation. Institutions and providers are responsible for educating, modeling, and providing culturally competent care and aiming to achieve workforce equity to improve outcomes for AI/AN children receiving intensive care.

Twinning International Pediatric Cardiology Fellowship Programs: A Transformative Educational Experience for Trainees with Potential for Global Adoption.

Over the last decade, having endured the COVID-19 pandemic, education and training in pediatric cardiology have undergone a profound disruptive transformation. Trainees experience considerable stress achieving all the competencies required to become a competent pediatric cardiologist. Often the quality of the training experienced by trainees, the approach to patients, and potential institutional preference in management strategy is heavily influenced by the center in which they train. We developed an online live twin program of education between Texas Children's Hospital, Houston, Texas and Children's Health at Crumlin Dublin Ireland in 2019. We explored using grounded theory whether a regular scheduled shared teaching program improved fellow education and training between both centers. Trainees were surveyed to evaluate the benefits and disadvantages of such a twin program. The majority (93%) found the sessions helpful from an educational standpoint with many trainees reporting it to be a transformative experience. Three important learning themes emerged: practice variation between centers, managing uncertainty in clinical practice and cognitive overload. This pedagogical model could be replicated across multiple international pediatric cardiology units and facilitate "collaborative learning" among centers across the globe. Furthermore, this novel educational model could also be adopted by other medical specialties.

Application of Cardiovascular Physiology to the Critically Ill Patient.

OBJECTIVES: To use the ventricular pressure-volume relationship and time-varying elastance model to provide a foundation for understanding cardiovascular physiology and pathophysiology, interpreting advanced hemodynamic monitoring, and for illustrating the physiologic basis and hemodynamic effects of therapeutic interventions. We will build on this foundation by using a cardiovascular simulator to illustrate the application of these principles in the care of patients with severe sepsis, cardiogenic shock, and acute mechanical circulatory support. DATA SOURCES: Publications relevant to the discussion of the time-varying elastance model, cardiogenic shock, and sepsis were retrieved from MEDLINE. Supporting evidence was also retrieved from MEDLINE when indicated. STUDY SELECTION, DATA EXTRACTION, AND SYNTHESIS: Data from relevant publications were reviewed and applied as indicated. CONCLUSIONS: The ventricular pressure-volume relationship and time-varying elastance model provide a foundation for understanding cardiovascular physiology and pathophysiology. We have built on this foundation by using a cardiovascular simulator to illustrate the application of these important principles and have demonstrated how complex pathophysiologic abnormalities alter clinical parameters used by the clinician at the bedside.

Automated prediction of cardiorespiratory deterioration in patients with single-ventricle parallel circulation: A multicenter validation study.

Objectives: Patients with single-ventricle physiology have a significant risk of cardiorespiratory deterioration between their first- and second-stage palliation surgeries. Detection of deterioration episodes may allow for early intervention and improved outcomes. Methods: A prospective study was executed at Nationwide Children's Hospital, Children's Hospital of Philadelphia, and Children's Hospital Colorado to collect physiologic data of subjects with single ventricle physiology during all hospitalizations between neonatal palliation and II surgeries using the Sickbay software platform (Medical Informatics Corp). Timing of cardiorespiratory deterioration events was captured via chart review. The predictive algorithm previously developed and validated at Texas Children's Hospital was applied to these data without retraining. Standard metrics such as receiver operating curve area, positive and negative likelihood ratio, and alert rates were calculated to establish clinical performance of the predictive algorithm. Results: Our cohort consisted of 58 subjects admitted to the cardiac intensive care unit and stepdown units of participating centers over 14 months. Approximately 28,991 hours of high-resolution physiologic waveform and vital sign data were collected using the Sickbay. A total of 30 cardiorespiratory deterioration events were observed. the risk index metric generated by our algorithm was found to be both sensitive and specific for detecting impending events one to two hours in advance of overt extremis (receiver operating curve = 0.927). Conclusions: Our algorithm can provide a 1- to 2-hour advanced warning for 53.6% of all cardiorespiratory deterioration events in children with single ventricle physiology during their initial postop course as well as interstage hospitalizations after stage I palliation with only 2.5 alarms being generated per patient per day.

Effect of exogenous l-carnitine on aortic stiffness in dyslipidemic adolescents: Design of a quadruple-blind, randomized, controlled interventional trial.

Background: Atherosclerotic cardiovascular disease (ASCVD) risk factors including vascular remodeling leading to hypertension and dyslipidemia are prevalent among children and adolescents. Conflicting observational and Mendelian randomization data suggest endogenous carnitine may affect arterial stiffness and lipid traits. Because of this, we developed a study to evaluate the causal role for carnitine in arterial stiffness at a point when the lifecourse trajectory to hypertension can be modified. Methods: This study is a mechanistic, double-blinded, randomized control trial (RCT) in 166 adolescents with dyslipidemia for the effect of 6 months of maximum dose 3 g daily oral l-carnitine supplementation (CS+) versus placebo (CS-) on aortic stiffness measured as carotid-femoral pulse wave velocity (CFPWV) and pulse pressure (PP); lipid concentrations (total cholesterol, HDL-C, triglycerides, and LDL-C) and serum fatty acid oxidation biomarkers by metabolomic analysis. Conclusions: The simultaneous evaluation of endogenous carnitine genetic effects and exogenous l-carnitine supplementation may facilitate future therapies for youth with cardiometabolic derangement to arrest atherosclerotic changes.

The Fontan Udenafil Exercise Longitudinal Trial: Subgroup Analysis.

The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.5 mg twice daily). In this post hoc analysis, we evaluate whether subgroups within the population experienced a differential effect on exercise performance in response to treatment. The effect of udenafil on exercise was evaluated within subgroups defined by baseline characteristics, including peak oxygen consumption (VO2), serum brain-type natriuretic peptide level, weight, race, gender, and ventricular morphology. Differences among subgroups were evaluated using ANCOVA modeling with fixed factors for treatment arm and subgroup and the interaction between treatment arm and subgroup. Within-subgroup analyses demonstrated trends toward quantitative improvements in peak VO2, work rate at the ventilatory anaerobic threshold (VAT), VO2 at VAT, and ventilatory efficiency (VE/VCO2) for those randomized to udenafil compared to placebo in nearly all subgroups. There was no identified differential response to udenafil based on baseline peak VO2, baseline BNP level, weight, race and ethnicity, gender, or ventricular morphology, although participants in the lowest tertile of baseline peak VO2 trended toward larger improvements. The absence of a differential response across subgroups in response to treatment with udenafil suggests that the treatment benefit may not be restricted to specific sub-populations. Further work is warranted to confirm the potential benefit of udenafil and to evaluate the long-term tolerability and safety of treatment and to determine the impact of udenafil on the development of other morbidities related to the Fontan circulation.Trial Registration NCT0274115.

Pre-Fontan Assessment Utilizing Combined Cardiac Catheterization and Cardiac MRI: Comparison to the Pre-Fontan Catheterization.

Single ventricle patients eligible for Fontan completion undergo pre-Fontan catheterization for hemodynamic and anatomic assessment prior to surgery. Cardiac magnetic resonance imaging may be used to evaluate pre-Fontan anatomy, physiology, and collateral burden. We describe our center's outcomes in patients undergoing pre-Fontan catheterization combined with cardiac magnetic resonance imaging. A retrospective review of patients undergoing pre-Fontan catheterization from 10/2018 to 04/2022 at Texas Children's Hospital was performed. Patients were divided into 2 groups: combined cardiac magnetic resonance imaging and catheterization (combined group) and those who underwent catheterization only (catheterization only group). There were 37 patients in the combined group and 40 in the catheterization only group. Both groups were similar in age and weight. Patients undergoing combined procedures received less contrast, and experienced less in-lab time, fluoroscopy time and catheterization procedure time. Median radiation exposure was lower in the combined procedure group but was not statistically significant. Intubation and total anesthesia times were higher in the combined procedure group. Patients undergoing a combined procedure were less likely to have collateral occlusion performed than in the catheterization only group. Bypass time, intensive care unit length of stay, and chest tube duration were similar in both groups at the time of Fontan completion. Combined pre-Fontan assessment decreases catheterization procedure and fluoroscopy time associated with cardiac catheterization at the expense of longer anesthetic times, and results in similar Fontan outcomes compared to when cardiac catheterization alone is utilized.

Hereditary pulmonary arterial hypertension burden in pediatrics: A single referral center experience.

Introduction: Hereditary pulmonary arterial hypertension (HPAH) is a rare yet serious type of pulmonary arterial hypertension (PAH). The burden in the pediatric population remains high yet underreported. The objective of this study is to describe the distribution of mutations found on targeted PAH panel testing at a large pediatric referral center. Methods: Children with PAH panel administered by the John Welsh Cardiovascular Diagnostic Laboratory at Texas Children's Hospital and Baylor College of Medicine in Houston, Texas between October 2012 to August 2021 were included into this study. Medical records were retrospectively reviewed for clinical correlation. Results: Sixty-six children with PAH underwent PAH genetic testing. Among those, 9 (14%) children were found to have pathogenic mutations, 16 (24%) children with variant of unknown significance and 41 (62%) children with polymorphism (classified as likely benign and benign). BMPR2 mutation was the most common pathogenic mutation, seen in 6 of the 9 children with detected mutations. Hemodynamic studies showed higher pulmonary vascular resistance among those with pathogenic mutations than those without (17.4 vs. 4.6 Wood units). All children with pathogenic mutations had severe PAH requiring triple therapy. There were tendencies for higher lung transplantation rate but lower mortality among those with pathogenic mutations. Conclusions: Abnormalities on genetic testing are not uncommon among children with PAH, although majority are of unclear significance. However, children with pathogenic mutations tended to present with more severe PAH requiring aggressive medical and surgical therapies. Genetic testing should be routinely considered due to consequences for treatment and prognostic implications. Larger scale population studies and registries are warranted to characterize the burden of HPAH in the pediatric population specifically.

Organizational Culture as a Determinant of Outcome in Teams: Implications for the Pediatric Cardiac Specialist.

Although enormous effort has focussed on how to build an effective culture in the business community, relatively little effort has addressed how to achieve this in the hospital environment, specifically related to the field of congenital heart disease teams. The examination of culture in pediatric cardiac care is particularly important for several key reasons: first, it represents high-stakes medicine, second, there are multiple stakeholders requiring collaboration between cardiologists, surgeons, anaesthesiologists, perfusionists, nursing staff, and allied health care professionals, and finally, both the patient and the family are intimately involved in the care pathway. This review article investigates some of the critical components to building an effective culture, drawing upon similarities in other disciplines, thereby fostering high performance multidisciplinary teams in congenital cardiology care. Strategies to change culture such as Kotter's model of change are also discussed. High performance teams share one common vital characteristic: psychological safety for team members to speak their minds, thereby fostering an open culture, in which creativity can flourish to facilitate major breakthroughs. Adoption of the "Flight Plan" review promotes patient centric care and champions a psychologically safe culture.

Computer simulation of surgical interventions for the treatment of refractory pulmonary hypertension.

This paper describes computer models of three interventions used for treating refractory pulmonary hypertension (RPH). These procedures create either an atrial septal defect, a ventricular septal defect or, in the case of a Potts shunt, a patent ductus arteriosus. The aim in all three cases is to generate a right-to-left shunt, allowing for either pressure or volume unloading of the right side of the heart in the setting of right ventricular failure, while maintaining cardiac output. These shunts are created, however, at the expense of introducing de-oxygenated blood into the systemic circulation, thereby lowering the systemic arterial oxygen saturation. The models developed in this paper are based on compartmental descriptions of human hemodynamics and oxygen transport. An important parameter included in our models is the cross-sectional area of the surgically created defect. Numerical simulations are performed to compare different interventions and various shunt sizes and to assess their impact on hemodynamic variables and oxygen saturations. We also create a model for exercise and use it to study exercise tolerance in simulated pre-intervention and post-intervention RPH patients.

Optimal Fenestration of the Fontan Circulation.

In this paper, we develop a pulsatile compartmental model of the Fontan circulation and use it to explore the effects of a fenestration added to this physiology. A fenestration is a shunt between the systemic and pulmonary veins that is added either at the time of Fontan conversion or at a later time for the treatment of complications. This shunt increases cardiac output and decreases systemic venous pressure. However, these hemodynamic benefits are achieved at the expense of a decrease in the arterial oxygen saturation. The model developed in this paper incorporates fenestration size as a parameter and describes both blood flow and oxygen transport. It is calibrated to clinical data from Fontan patients, and we use it to study the impact of a fenestration on several hemodynamic variables, including systemic oxygen availability, effective oxygen availability, and systemic venous pressure. In certain scenarios corresponding to high-risk Fontan physiology, we demonstrate the existence of a range of fenestration sizes in which the systemic oxygen availability remains relatively constant while the systemic venous pressure decreases.

Editorial Board Composition Among Pediatric Cardiology Journals: Time to Cast the Net Wider.

Background: No data currently exist on the diversity of editorial board members (EBMs) of pediatric cardiology journals. Objectives: The objective was to investigate the editorial boards of 5 pediatric cardiology journals to assess the composition of these boards in terms of the geographical, gender, and economic representation of their members. Methods: Information on EBMs was collected directly from 5 journal websites accessed in February 2022. The following data were collected: country of practice (including World Bank geographical and income classification), institution of practice, role on editorial board, and whether an individual held a role on 1 or more of the boards included. Results: A total of 455 EBMs were identified. A total of 369 (81%) were male. All editors-in-chief were male, and 4 were from the United States. EBMs practicing in North America accounted for 278 individuals (61%) of the editorial boards reviewed. The next majority of EBMs are practicing within Europe and Central Asia (23%, n = 103), East Asia and Pacific (7%, n = 31), Middle East and North Africa (4%, n = 18), and Latin America and Caribbean (4%, n = 16). Less than 2% (n = 9) practice in Sub-Saharan Africa and South Asia. Over 90% (n = 415) practice in high-income countries. There was no representation from low-income countries. Conclusions: Women and pediatric cardiologists practicing in countries outside of Europe and North America were underrepresented on the editorial boards of the journals studied. Diversifying composition of editorial boards may provide greater representation of underserved areas and encourage broader avenues of investigation and research.

Automated Prediction of Cardiorespiratory Deterioration in Patients With Single Ventricle.

BACKGROUND: Patients with single-ventricle physiology have a significant risk of cardiorespiratory deterioration between their first and second stage palliation surgeries. OBJECTIVES: The objective of this study is to develop and validate a real-time computer algorithm that can automatically recognize physiological precursors of cardiorespiratory deterioration in children with single-ventricle physiology during their interstage hospitalization. METHODS: A retrospective study was conducted from prospectively collected physiological data of subjects with single-ventricle physiology. Deterioration events were defined as a cardiac arrest requiring cardiopulmonary resuscitation or an unplanned intubation. Physiological metrics were derived from the electrocardiogram (heart rate, heart rate variability, ST-segment elevation, and ST-segment variability) and the photoplethysmogram (peripheral oxygen saturation and pleth variability index). A logistic regression model was trained to separate the physiological dynamics of the pre-deterioration phase from all other data generated by study subjects. Data were split 50/50 into model training and validation sets to enable independent model validation. RESULTS: Our cohort consisted of 238 subjects admitted to the cardiac intensive care unit and stepdown units of Texas Children's Hospital over a period of 6 years. Approximately 300,000 h of high-resolution physiological waveform and vital sign data were collected using the Sickbay software platform (Medical Informatics Corp., Houston, Texas). A total of 112 cardiorespiratory deterioration events were observed. Seventy-two of the subjects experienced at least 1 deterioration event. The risk index metric generated by our optimized algorithm was found to be both sensitive and specific for detecting impending events 1 to 2 h in advance of overt extremis (receiver-operating characteristic curve area: 0.958; 95% confidence interval: 0.950 to 0.965). CONCLUSIONS: Our algorithm can provide 1 to 2 h of advanced warning for 62% of all cardiorespiratory deterioration events in children with single-ventricle physiology during their interstage period, with only 1 alarm being generated at the bedside per patient per day.

Novel Method of Calculating Pulse Pressure Variation to Predict Fluid Responsiveness to Transfusion in Very Low Birth Weight Infants.

A novel technique was used to calculate pulse pressure variation. The algorithm reliably predicted fluid responsiveness to transfusion, with a receiver operating characteristic area under the curve of 0.89. This technique may assist clinicians in the management of fluids and vasoactive medications for premature infants.

Mental Health Disorders in Children With Congenital Heart Disease.

BACKGROUND: Data on anxiety, depression, and attention-deficit/hyperactivity disorder (ADHD) are lacking for youth with congenital heart disease (CHD), particularly those with simple CHD. This study aims to characterize these disorders in youth with CHD compared to those without CHD. METHODS: A comparative cross-sectional study was conducted by using the electronic medical records of a large tertiary care hospital between 2011 and 2016. Inclusion criteria were youth aged 4 to 17 years with >1 hospitalization or emergency department visits. Exclusion criteria were patients with arrhythmias or treatment with clonidine and/or benzodiazepines. The primary predictor variable was CHD type: simple, complex nonsingle ventricle, and complex single ventricle. The primary outcome variable was a diagnosis and/or medication for anxiety and/or depression or ADHD. Data were analyzed by using logistic regression (Stata v15; Stata Corp, College Station, TX). RESULTS: We identified 118 785 patients, 1164 with CHD. Overall, 18.2% (n = 212) of patients with CHD had a diagnosis or medication for anxiety or depression, compared with 5.2% (n = 6088) of those without CHD. All youth with CHD had significantly higher odds of anxiety and/or depression or ADHD. Children aged 4 to 9 years with simple CHD had ∼5 times higher odds (odds ratio: 5.23; 95% confidence interval: 3.87-7.07) and those with complex single ventricle CHD had ∼7 times higher odds (odds ratio: 7.46; 95% confidence interval: 3.70-15.07) of diagnosis or treatment for anxiety and/or depression. Minority and uninsured youth were significantly less likely to be diagnosed or treated for anxiety and/or depression or ADHD, regardless of disease severity. CONCLUSIONS: Youth with CHD of all severities have significantly higher odds of anxiety and/or depression and ADHD compared to those without CHD. Screening for these conditions should be considered in all patients with CHD.