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Aim: Passive immunisation with palivizumab is recommended in many countries for children with haemodynamically significant cardiac disease. We trialled respiratory syncytial virus (RSV) immunoprophylaxis in such infants during 2008–2009.Methods: We identified all RSV admissions between 2005–2009 and examined all patients with significant cardiac disease who received palivizumab in 2008–2009.Results: Infants with symptomatic cardiac disease had a more complicated course of RSV bronchiolitis with longer hospital stay, more frequent intensive care admission, longer intensive care stay and were more likely to receive respiratory support (all P < 0.05). One hundred seventeen infants with symptomatic cardiac disease received palivizumab. Of these, two (1.7%) required admission for RSV bronchiolitis. Overall, there was a reduction in admission of infants with symptomatic cardiac disease with RSV bronchiolitis in 2008–2009 (2% per year) compared with 2005–2007 (5–9% per year; P < 0.03). The number of patients with symptomatic cardiac disease who required intensive care for RSV bronchiolitis in the same period was unchanged, as a number presented to our service with RSV infection prior to commencing immunoprophylaxis or having had their cardiac diagnosis made in other centres.Conclusions: Compared with other infants, those with haemodynamically significant cardiac disease have a more complicated course of illness with RSV bronchiolitis. In these infants, palivizumab reduced the number of hospitalisations because of RSV. Cohorting patients for maximal palivizumab use reduced overall cost. To significantly impact on intensive care admissions overall, immunoprophylaxis should be considered at a regional level. [ABSTRACT FROM AUTHOR]

RATIONALE: Rebound pulmonary hypertension (PHT) can complicate the weaning of nitric oxide (NO), and is in part related to transient depletion of intrinsic cyclic guanosine monophosphate. Rebound is characterized by increased pulmonary arterial (PA) pressure, cardiopulmonary instability, and in some cases, the need to continue NO beyond the intended period of use. There is anecdotal evidence that sildenafil, a phosphodiesterase-5 inhibitor, may prevent recurrence of rebound. OBJECTIVES: We investigated the role of sildenafil in preventing rebound (an increase in PA pressure of 20% or greater, or failure to discontinue NO) in patients in whom previous attempts had not been made to wean from NO. METHODS: Thirty ventilated infants and children, receiving 10 ppm or greater inhaled NO, were randomized to receive 0.4 mg/kg of sildenafil, or placebo, 1 h before discontinuing NO. Twenty-nine patients completed the study. MEASUREMENTS: PA pressures and blood gases were measured before the study drug, and 1 and 4 h after stopping NO. MAIN RESULTS: Rebound occurred in 10 of 14 placebo patients, and 0 of 15 sildenafil patients (p < 0.001). PA pressure increased by 25% (14-67) in placebo patients, and by 1%(-9-5) in sildenafil patients (p < 0.001). Four placebo patients could not be weaned from NO due to severe cardiovascular instability, whereas all sildenafil patients were weaned (p = 0.042). Duration of ventilation after study was 98.0 (47.0-223.5) h for placebo patients and 28.2 (15.7-54.6) h for sildenafil patients (p = 0.024). CONCLUSION: A single dose of sildenafil prevented rebound after withdrawal of NO, and reduced the duration of mechanical ventilation. Prophylaxis with sildenafil should be considered when weaning patients from inhaled NO. [ABSTRACT FROM AUTHOR]

Traditionally, neonates with transposition of the great arteries are immediately transferred to a cardiac centre. Travelling to the bedside to perform a balloon atrial septostomy and allowing the child to remain there for a few days before transfer is safe, effective, and a good use of medical resources. [ABSTRACT FROM AUTHOR]

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., Competing Interests: CONFLICTS OF INTEREST The authors have no conflicts of interest relevant to this article to disclose., (Copyright © 2024 National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved.)

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., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

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.

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., Competing Interests: Dr. Gomez’s institution received funding from the National Institute of Diabetes and Digestive and Kidney Diseases, Baxter, and bioMérieux; he received funding from Trilinear Bioventures, AclRx, Novartis, and the National Kidney Foundation. Dr. Burkhoff received funding from PVLoops. The remaining authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2024 by the Society of Critical Care Medicine and Wolters Kluwer Health, Inc. All Rights Reserved.)

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., (© 2024. The Author(s).)

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 (VO 2 ), 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 VO 2 , work rate at the ventilatory anaerobic threshold (VAT), VO 2 at VAT, and ventilatory efficiency (VE/VCO 2 ) for those randomized to udenafil compared to placebo in nearly all subgroups. There was no identified differential response to udenafil based on baseline peak VO 2 , baseline BNP level, weight, race and ethnicity, gender, or ventricular morphology, although participants in the lowest tertile of baseline peak VO 2 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., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Competing Interests: Dr. Burkoff disclosed they are an owner/consultant to PVLoops LLC. The remaining authors have disclosed that they do not have any potential conflicts of interest.

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., Competing Interests: We declare we have no financial disclosures nor conflicts of interest. No funding sources or other stakeholders had any impact on design, completion or decision to publish this manuscript., (© 2023 The Authors. Published by Elsevier Inc.)

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., (© 2023 The Authors.)

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., Competing Interests: NPV receives research support from the PePH registry. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Ishizuka, Zou, Whalen, Ely, Coleman, Lopez-Terrada, Penny, Fan and Varghese.)

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., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Institute of Mathematics and its Applications. All rights reserved.)

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., (© 2022. The Author(s).)

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., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose.PERSPECTIVESCOMPETENCY IN MEDICAL KNOWLEDGE: This research paper identifies a gender, economic, and geographical gap among editorial board members of dedicated pediatric cardiology journals. The lack of diversity in representation may have implications for the type of research being published, with the potential for research in high-income locations being overrepresented, to the detriment of research based in other parts of the world. TRANSLATIONAL OUTLOOK: Additional research is required to further investigate the reasons behind the reduced representation for women and individuals from low- and middle-income countries. Further work is required to solve this problem., (© 2022 The Authors.)

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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Ahmad, Jin, Penny, Rusin, Peskin and Puelz.)

Competing Interests: Dr. Burkhoff's institution received funding from Abiomed; he received funding from PVLoops LLC, CardioDyme, Aquapss, Axon Therapies, BioMind, Corvia Medical, Orchestra Biomedical, IMPULSE Dynamics, and Zoll; he disclosed that he is the founder of PVLoops LLC. The remaining authors have disclosed that they do not have any potential conflicts of interest.

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., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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., Competing Interests: Funding Support and Author Disclosures This research was supported in part by the National Institutes of Health (1R56HL131574 and 1R01HL142994) and the American Heart Association (16BGIA27490024). Dr. Rusin is a cofounder of Medical Informatics Corp.; no funding was provided by the company to support this work. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Competing Interests: The authors have disclosed that they do not have any potential conflicts of interest.

Epic Supra valves have been used off-label in the pulmonary position. We aim to evaluate the durability of Epic valves in the pulmonary position. We performed a retrospective review of all Epic valves placed in the pulmonary position from October 2008 to May 2019. Time-to-event analysis was performed using Kaplan-Meier estimates to evaluate freedom from valve intervention, moderate pulmonary regurgitation, and peak velocity greater than 3.5 m/s. Valve dysfunction was a composite of all 3 end points. A total of 79 patients had Epic valves implanted in the pulmonary position. Median age was 18.5 years (15th-85th percentile 11.2-41.0). In total, 1 (1%) 19 mm valve, 4 (5%) 21 mm valves, 8 (10%) 23 mm valves, 23 (29%) 25 mm valves, and 43 (54%) 27 mm valves were implanted. There were no deaths or transplants. Median follow-up was 3.1 years (interquartile range 1.0-5.5). At 5 years, freedom from valve intervention was 95%, freedom from valve dysfunction was 68%, freedom from moderate pulmonary regurgitation was 73%, and freedom from peak velocity greater than 3.5 m/s was 82%. Epic Supra valves provide an acceptable valve replacement in the pulmonary position for children and adults. Longer follow-up is needed to determine valve durability through the entirety of the valve life expectancy., (Copyright © 2020 Elsevier Inc. All rights reserved.)

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., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

Objective: To propose a new method to estimate pulse pressure variability (PPV) in the arterial blood pressure waveform., Methods: Traditional techniques of calculating PPV using peak finding have a fundamental flaw that prevents them from accurately resolving PPV for small tidal volumes, limiting the use of PPV to only mechanical ventilated patients. The improved method described here addresses this limitation using Fourier analysis of an oscillatory signal that exhibits a time-varying modulation of its amplitude. The analysis reveals a constraint on the spectral representation that must be satisfied for any oscillatory signal that exhibits a time-varying modulation of its amplitude. This intrinsic mathematical structure is taken advantage of in order to improve the robustness of the algorithm., Results: The applicability of the method is tested using synthetic data and 100 h of physiologic data collected from patients admitted to Texas Children's Hospital., Significance and Conclusion: The proposed method accurately recovers values of PPV at signal-to-noise ratios six times smaller than the traditional method. This is a significant advance for the potential use of PPV to recognize fluid responsiveness during low tidal volume ventilation or spontaneous breathing for which the signal-to-noise ratio is expected to be small., Competing Interests: Declaration of Competing Interest Dr. Rusin declares that he has a conflict with Medical Informatics Corp. Medical Informatics Corp did not financially support this work. All other authors have no conflicts of interest.

Iatrogenic aortopulmonary communications (IAPCs) are an uncommon but important complication after percutaneous intervention in postoperative patients. Iatrogenic aortopulmonary communications typically occur after balloon angioplasty or other interventions of the pulmonary outflow tract in certain anatomic configurations in which there is a denuded tissue plane between the pulmonary artery and aorta. They can present with signs and symptoms ranging from subtle clues which are difficult to recognize to near immediate hemodynamic instability. Once recognized, these lesions can become management dilemmas, and intraprocedural interventions can be complicated by complex anatomy and inadequate visualization by standard imaging techniques. We report cases where intracardiac echocardiography (ICE) was integral in the evaluation and management of IAPC as complications of prior transcatheter interventions. We found using ICE safely and effectively identified IAPCs and reduced the technical difficulty of intervention after IAPC discovery.

Background: The Fontan operation creates a total cavopulmonary connection, a circulation in which the importance of pulmonary vascular resistance is magnified. Over time, this circulation leads to deterioration of cardiovascular efficiency associated with a decline in exercise performance. Rigorous clinical trials aimed at improving physiology and guiding pharmacotherapy are lacking., Methods: The FUEL trial (Fontan Udenafil Exercise Longitudinal) was a phase III clinical trial conducted at 30 centers. Participants were randomly assigned udenafil, 87.5 mg twice daily, or placebo in a 1:1 ratio. The primary outcome was the between-group difference in change in oxygen consumption at peak exercise. Secondary outcomes included between-group differences in changes in submaximal exercise at the ventilatory anaerobic threshold, the myocardial performance index, the natural log of the reactive hyperemia index, and serum brain-type natriuretic peptide., Results: Between 2017 and 2019, 30 clinical sites in North America and the Republic of Korea randomly assigned 400 participants with Fontan physiology. The mean age at randomization was 15.5±2 years; 60% of participants were male, and 81% were white. All 400 participants were included in the primary analysis with imputation of the 26-week end point for 21 participants with missing data (11 randomly assigned to udenafil and 10 to placebo). Among randomly assigned participants, peak oxygen consumption increased by 44±245 mL/min (2.8%) in the udenafil group and declined by 3.7±228 mL/min (-0.2%) in the placebo group ( P =0.071). Analysis at ventilatory anaerobic threshold demonstrated improvements in the udenafil group versus the placebo group in oxygen consumption (+33±185 [3.2%] versus -9±193 [-0.9%] mL/min, P =0.012), ventilatory equivalents of carbon dioxide (-0.8 versus -0.06, P =0.014), and work rate (+3.8 versus +0.34 W, P =0.021). There was no difference in change of myocardial performance index, the natural log of the reactive hyperemia index, or serum brain-type natriuretic peptide level., Conclusions: In the FUEL trial, treatment with udenafil (87.5 mg twice daily) was not associated with an improvement in oxygen consumption at peak exercise but was associated with improvements in multiple measures of exercise performance at the ventilatory anaerobic threshold., Clinical Trial Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT02741115.

There have been no clinical studies evaluating the use of drug-eluting stents (DES) versus bare metal stents (BMS) for infants who underwent ductus arteriosus (DA) stent placement for ductal-dependent pulmonary blood flow (PBF). We aimed to compare the use of second-generation (fluoropolymer-coated everolimus) DES to BMS in infants who underwent DA stenting for ductal-dependent PBF. A retrospective study of infants who underwent DA stenting for ductal-dependent PBF from January 2004 to March 2018 at a single tertiary care pediatric hospital was performed. Of 94 infants identified, 71 (46 BMS and 25 DES) met inclusion criteria. Baseline characteristics of the DES and BMS cohorts were comparable. The patent lumen to stent diameter on subsequent angiographic evaluation was 81% in DES as compared with 50% in BMS group; p = 0.01. There were 2 deaths early in our experience, both in the BMS group. Unplanned reinterventions were less in the DES group (3, 12% patients) compared with the BMS group (13, 28%), p = 0.03. Pulmonary artery size as assessed using Nakata and pulmonary artery symmetry index was comparable in both the groups. There was no difference in infection rates between the groups. On multivariate analysis, prematurity, BMS, and lower oxygen saturations at discharge were associated with subsequent unplanned reintervention (p = 0.01, 0.03 and 0.03, respectively). In conclusion, our clinical experience suggests that in infants who underwent DA stenting for ductal-dependent PBF, (fluoropolymer-coated everolimus eluting) DES results in less luminal loss and lower unplanned reintervention for cyanosis as compared with BMS implantation., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: In asymptomatic children, screening echocardiography has been used to attempt to diagnose rheumatic heart disease (RHD) at an early stage (latent RHD). World Heart Federation guidelines have standardized categorization of "definite," "borderline," or no RHD by echo findings. The progression of RHD diagnosed through echo screening is not known. In 2014, we screened 1450 schoolchildren in Malawi., Objective: Our objective was to evaluate 2-year RHD evolution among those diagnosed through screening., Methods: Two-year follow-up echocardiograms of those diagnosed with latent RHD were read by a primary, secondary, then third reader if there was disagreement. Progression or regression of both definite and borderline groups were tabulated. Penicillin adherence, age, gender, number in home, and household income were compared between those with definite RHD who regressed to borderline and those that stayed definite. We utilized the local system used to track HIV defaulters in order to bring participants back into care. Comparisons were made using Fisher's exact and Wilcoxon rank-sum tests., Results: Of the 39 with borderline RHD, 1 was lost to follow-up (2.6%), 1 progressed to definite (2.6%), 19 remained borderline (48.7%), 17 (43.6%) regressed to normal, and 1 was reclassified as mitral valve prolapse (2.6%). Of the 11 with definite RHD, 6 (54.5%) remained definite, 4 regressed to borderline (36.4%), and 1 regressed to normal (9.1%). Two of 11 with definite RHD had penicillin adherence above 80% for the 2-year follow-up period. There were no differences in adherence, gender, age, household income, or number in household between those with definite RHD that regressed to borderline and those who did not (P > .19)., Conclusions: Borderlines had a very low progression rate to definite RHD. A strength of our study was a high retention rate (98%). Longer follow-up is needed to determine expected disease evolution., (© 2019 Wiley Periodicals, Inc.)

Background: With expanding use of clinical whole exome sequencing (WES), genetic variants of uncertain significance are increasingly identified. As pathologic mutations in genes associated with arrhythmogenic right ventricular cardiomyopathy (ARVC) carry a risk of sudden death, determining the diagnostic relevance of incidentally identified variants associated with these genes is critical., Methods: WES variants from a large, predominantly pediatric cohort (N = 7,066 probands) were obtained for nine ARVC-associated genes (Baylor Miraca). For comparison, a control cohort was derived from the gnomAD database and an ARVC case cohort (N = 1,379 probands) was established from ARVC cases in the literature. Topologic mapping was performed and signal-to-noise analysis was conducted normalizing WES, or case variants, against control variant frequencies. Retrospective chart review was performed of WES cases evaluated clinically (Texas Children's Hospital)., Results: Incidentally identified variants occurred in 14% of WES referrals and localized to genes which were rare among ARVC cases yet similar to controls. Amino acid-level signal-to-noise analysis of cases demonstrated "pathologic hotspots" localizing to critical domains of PKP2 and DSG2 while WES variants did not. PKP2 ARM7 and ARM8 domains and DSG2 N-terminal cadherin-repeat domains demonstrated high pathogenicity while normalized WES variant frequency was low. Review of clinical data available on WES referrals demonstrated none with evidence of ARVC among variant-positive individuals., Conclusions: Incidentally identified variants are common among pediatric WES testing with gene frequencies similar to "background" variants. Incidentally identified variants are unlikely to be pathologic., (© 2019 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.)

Background: There is geographical overlap between areas endemic for rheumatic heart disease (RHD) and those endemic for HIV. A recent pilot study demonstrated that children living with HIV might be at less risk for RHD development; however, the sample size was too small to make definitive conclusions. Our objective was to determine the prevalence of RHD among HIV-positive children in Uganda., Methods: We conducted a prospective, cross-sectional study of HIV-positive children (5-15 years of age) receiving care at the Baylor Uganda HIV Clinic, Kampala, Uganda. A focused echocardiogram and chart review was performed. A sample size of 988 children was needed to provide 80% power to detect a difference in population prevalence between HIV-positive children and the general population, 2.97% [95% confidence interval (CI): 2.70-3.24%], based on previous reports., Results: Screening echocardiography of 993 HIV-positive children found 15 individuals (1.5%; 95% CI: 0.88%-2.54%) with RHD. Of these 15, 2 were classified as definite RHD and 13 as borderline RHD. The majority of children had isolated mitral valve disease (93%). Children found to have RHD were older than those without RHD, 12 versus 10 years of age (P = 0.004). When separated based on geographic location, the prevalence of RHD among HIV-positive children from Kampala was 1.28% (95% CI: 0.63%-2.51%) compared with 2.1% (95% CI: 0.89%-4.89%) in those from outside Kampala., Conclusions: Children living with HIV have a lower prevalence of RHD than the general pediatric population. Further studies are needed to explore this protective association.

Cirrhotic cardiomyopathy (CCM), a comorbidity of end-stage cirrhotic liver disease, remains uncharacterized in children, largely because of a lack of an established pediatric definition. The aim of this retrospective cohort analysis is to derive objective two-dimensional echocardiographic (2DE) criteria to define CCM associated with biliary atresia (BA), or BA-CCM, and correlate presence of BA-CCM with liver transplant (LT) outcomes in this population. Using receiver operating characteristic (ROC) curve analysis, optimal cut-off values for left ventricular (LV) geometrical parameters that were highly sensitive and specific for the primary outcomes: A composite of serious adverse events (CSAE) and peritransplant death were determined. These results were used to propose a working definition for BA-CCM: (1) LV mass index (LVMI) ≥95 g/m 2.7 or (2) relative wall thickness of LV ≥0.42. Applying these criteria, BA-CCM was found in 34 of 69 (49%) patients with BA listed for LT and was associated with increased multiorgan dysfunction, mechanical and vasopressor support, and longer intensive care unit (ICU) and hospital stays. BA-CCM was present in all 4 waitlist deaths, 7 posttransplant deaths, and 20 patients with a CSAE (P < 0.01). On multivariable regression analysis, BA-CCM remained independently associated with both death and a CSAE (P < 0.01). Utilizing ROC analysis, LVMI was found to be a stronger predictor for adverse outcomes compared with current well-established markers, including Pediatric End-Stage Liver Disease (PELD) score. Conclusion: BA-CCM is highly sensitive and specific for morbidity and mortality in children with BA listed for LT. 2DE screening for BA-CCM may provide pertinent clinical information for prioritization and optimal peritransplant management of these children., (© 2018 by the American Association for the Study of Liver Diseases.)