Coronary Artery Aneurysm Thrombosis Causing Acute Myocardial Infarction in an Adolescent With Neurofibromatosis Type 1.

Acute myocardial infarction is a rare though possible occurrence in young patients, especially with predisposing conditions, and the treatment is still debated. We present the case of a teenager known to have neurofibromatosis type 1 with acute coronary syndrome caused by coronary aneurysms and thrombotic occlusion of the left circumflex artery treated with a stent-sparing strategy.

Implementation and analysis of clinic administered behavioural health screen in an outpatient paediatric cardiology clinic.

Previous studies investigating behavioural health screening processes have focused on selected diagnoses within paediatric cardiology and focused on a smaller number of potential concerns. We developed and administered a brief survey in our paediatric heart centre to assess the presence of a wider variety of behavioural health concerns and to connect patients with resources. A cohort of 305 patients aged 2-29 years (M = 11.97 years; SD = 6.00 years; 50.49% female), representing a variety of indications for a cardiology clinic visit, or a parent, completed a survey of 14 common behavioural health concerns. Behavioural health concerns were included based on practice patterns within paediatric psychology. Respondents indicated if they were currently receiving behavioural health services and if they were interested in behavioural health follow-up. Surveys were administered during check in and collected by clinic staff. A behavioural health provider attempted to reach all those who indicated interest by phone. Approximately 45% of the sample endorsed one or more behavioural health concerns and 30.16% of the sample endorsed at least one concern but were not already connected to services. Only 27.17% of this group requested follow-up. Most commonly endorsed concerns were anxiety, sleep problems, depressed/irritable mood, and somatic complaints. Survey results converge with existing literature to indicate that behavioural health concerns are common among youth seen in a paediatric cardiology clinic but most patients are not connected to appropriate services. Screening programmes can help meet this need but challenges remain. Clinical implications and future directions are discussed.

Ventricular Tachycardia Substrates in Children and Young Adults With Repaired Tetralogy of Fallot.

BACKGROUND: Patients with repaired tetralogy of Fallot (rTOF) have a time-dependent increased risk of ventricular tachycardia (VT). Slow conducting anatomical isthmuses (SCAIs) are the dominant VT substrates in adults with rTOF. It is unknown if they are present at younger age. OBJECTIVES: This study aimed to characterize VT substrates in patients with rTOF <30 years of age. METHODS: Data of consecutive patients with rTOF aged <30 years who underwent electroanatomical mapping and programmed electrical stimulation between 2005 and 2022 were analyzed. RESULTS: Fifty-five patients were included (median age: 15.8 years, IQR: 13.8-21.8 years; 15 repaired via ventriculotomy; 13 complex TOF variants). Twelve patients had right ventricle-to-pulmonary artery conduits inserted during initial repair or had early pulmonary valve replacement (PVR) (<1 year after repair). Indications for electroanatomical mapping and programmed electrical stimulation were spontaneous VT, before PVR, and risk stratification in 5, 40, and 10 patients, respectively. In 16 patients (29%), SCAI 3 was identified; no other SCAI was present. Monomorphic VT was inducible in 8 and related to SCAI 3 in 7 patients. Identified VT substrates were targeted by ablation. Right ventricle-to-pulmonary artery conduit/early PVR, ventriculotomy, and complex TOF were associated with SCAI 3 in univariable analysis. During a median follow-up of 5.3 years, VT recurred in 2 patients. No patients died. CONCLUSIONS: In young patients with rTOF, SCAI 3 is the dominant substrate for VT. Complex TOF and interrelated type and timing of (re-)operation may contribute to the development of SCAI 3 already at a young age.

Echocardiograms and bed placement in patients with multisystem inflammatory syndrome in children.

Background: Understanding of multisystem inflammatory syndrome in children (MIS-C) continues to evolve with extensive evaluations, including echocardiograms, obtained in emergency departments (EDs) to assist with clinical decision making and bed allocation. We assessed the utility of obtaining echocardiograms in the ED to assist in determining bed placement for this patient population. Methods: This 2-year retrospective single-center study of patients 0-21 years old without underlying cardiac disease hospitalized for MIS-C focused on individuals whose initial evaluation occurred in the institution's ED and whose echocardiogram was obtained either in the ED or within 24 hours of admission. Patients were placed in two cohorts-those remaining in their unit of admission without transfer (cohort WoT) and those transferred (cohort T) from their initial unit to one with a differing level of care within 24 hours. Pearson chi-square test assessed the relationship between echocardiogram status and appropriate bed placement, defined as no transfer within 24 hours. Results: Of the 60 patients who met study criteria, no significant difference was detected in rates of transfer between patients whose echocardiograms were obtained in the ED versus those obtained within 24 hours of admission (odds ratio =2.08; 95% confidence interval: 0.58, 7.95; P=0.28). Conclusions: Cardiac involvement is a known complication of MIS-C; however, our study yields no evidence in favor of obtaining echocardiograms in the ED to ensure appropriate bed placement. While this modality remains integral in evaluation and management, it does not appear to be requisite as part of an emergent workup prior to admission.

Clinical and Hemodynamic Outcomes with Enalapril Orodispersible Minitablets in Young Children with Heart Failure Due to Congenital Heart Disease.

Background: The angiotensin-converting enzyme inhibitor (ACEI) enalapril is often administered to infants and young children with heart failure (HF) in various dosing regimens and formulations not adapted for their age. Methods: This prospective, two-center, open-label 8-week study evaluated an age-appropriate formulation of orodispersible minitablets (ODMTs) of enalapril (0.25 mg and 1 mg) in children aged 0 to 6 years with HF due to congenital heart disease. An age/weight-based dosing schedule was followed. Measures of echocardiographic parameters, blood pressure, heart rate, modified Ross score, and biochemistry were obtained over the 8-week period. The following two groups were assessed: ACEI-naïve and ACEI-pretreated patients. Results: In total, 53 children (age range of 0.05 to 4.8 years) were enrolled and 29 were ACEI-naïve. The average enalapril dose was 0.098 mg/kg (0.06-0.17 mg/kg) in the naïve group and 0.15 mg/kg (0.07-0.3 mg/kg) in pretreated patients. After 8 weeks, the modified Ross score and left ventricular diastolic dimension (LVD) z-score showed a significant decrease in both groups (p < 0.005). During 8 weeks follow-up, there were no difference in the z-scores for the systolic blood pressure (p = 0.071) or heart rate (p = 0.146). Conclusions: Pediatric patients treated with ODMTs of enalapril for 8 weeks had favorable improvements in LVD and HF symptoms.

Off-label uses of the Amplatzer Piccolo Occluder in children with congenital and acquired heart diseases.

Aim: The Amplatzer Piccolo Occluder (APO) is approved for patent ductus arteriosus (PDA) occlusion in infants weighing >700 g but could offer versatility to treat other lesions.Methods: Retrospective review of children in whom APO was utilized for defects other than PDA between January 2022 and June 2023.Results: The APO was used in nine patients; three for ventricular septal defects, four with coronary fistulas, one for a ventricular pseudoaneurysm and one where APO deployed within a fenestration of a previously placed Amplatzer Septal Occluder. All nine patients had successful occlusions without complications.Conclusion: The APO is a versatile device that can be used to treat various small diameter lesions in children besides the PDA for which it is currently approved for.

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Cardiac Catheterization Post Congenital Cardiac Surgery: Analysis of Risk Factors for Mortality and Literature Review.

Background Diagnostic and interventional cardiac catheterization plays a significant role in the management of congenital heart defects with acceptable risks. Its role has also evolved in sick children but is associated with higher risks due to technical difficulties and co-morbidity factors. Some of the post-cardiac surgery children who show resistance to conventional management during the early postoperative period usually have residual defects or obstructions. Trans-catheter intervention (TCI) in such high-risk circumstances and relatively sick children is challenging, demands much expertise, and should be backed up by a competent multidisciplinary team. Some cases improve clinically, while others may require surgical or transcatheter re-intervention for a positive outcome. There is minimal data so far regarding the major complications after interventional cardiac catheterization during the immediate postoperative period after cardiac surgery. We analyzed multiple factors, including age, sex, weight, the initial diagnosis, and the time interval between surgery and TCI, to stratify the possible risks for mortality after TCI during the immediate postoperative period after cardiac surgery. Results Thirty-five patients fulfilled the inclusion criteria and underwent 43 interventional procedures. Five patients could not survive. Four had stent angioplasties on natural vasculature and one patient had in synthetic conduit. None of the mortality was related to the procedure. Multivariable risk factor analysis confirmed a moderate positive correlation coefficient (r) of 0.8017 between the variables. Still, it was not statistically significant if compared among subgroups or among the mortality and survival groups. Conclusion Interventional cardiac catheterization in sick children during the immediate postoperative period can be carried out without much-added risks in expert hands and under the supervision of a multi-disciplinary team. Though no conclusions could be drawn, our study adds to the limited existing data that could inspire others to perform such procedures on sick children. Moreover, the trend in our results indicated a large sample size could have identified a possible risk factor for mortality.

Validity of the Pneumonitor for Analysis of Short-Term Heart Rate Asymmetry Extended with Respiratory Data in Pediatric Cardiac Patients.

Background: Wearable technologies have been developed to measure physiological parameters conveniently. To consider the new measurement device valid, the crucial point is to assess its reliability with the gold standard. The study aimed to assess the validity of the Pneumonitor (PM, fs = 250 Hz) for acquisition of 5 min RR intervals (RRi) for analysis of heart rate asymmetry (HRA) in relation to the electrocardiography (ECG, fs = 1000 Hz) in a group of 19 pediatric cardiac patients. Association between HRA and respiratory rate (RespRate) was verified. Methods: The validation comprised Bland-Altman analysis, intraclass correlation coefficient, and Student's t-test. Results: Sufficient agreement between 10 from 16 HRA parameters was observed. Different HRA parameters values calculated based on RRi from both devices were related to different results of correlation analysis between two parameters and RespRate. Conclusions: The PM might be considered valid for recording RRi, which are then processed to calculate selected HRA parameters in a group of pediatric cardiac patients in rest condition. However, RRi recorded using devices with fs < 250 Hz may be not adequate for reliable HRA analysis.

The Pediatric Heart Center Melting Pot: Sharing Recipes for Success: Proceedings from the 8th World Congress of Pediatric Cardiology and Cardiac Surgery.

The challenges of present-day healthcare are urgent; there is a shortage of clinicians, patient care is increasingly complex, resources are limited, clinician turnover seems ever-increasing, and the expectations of providers and patients are monumental. To transform problems into innovative opportunities, diverse perspectives and a sense of possibility are needed. The following is a collaborative manuscript authored by the speakers of the 8th World Congress of Pediatric Cardiology and Cardiac Surgery session, "Teamwork, Culture Change, and Strategy." Although this panel was diverse in the clinical roles, nationalities, and genders represented, several consistent themes emerged which are explored in this work.

Mitigating Alarm Fatigue and Improving the Bedside Experience by Reducing Nonactionable Alarms.

OBJECTIVE: To assess whether conditional bedside alarm triggers can reduce the frequency of nonactionable alarms without compromising patient safety and enhance nursing and family satisfaction. STUDY DESIGN: Single-center, quality improvement initiative in an acute care cardiac unit and pediatric intensive care unit. Following the 4-week preintervention baseline period, bedside monitors were programmed with hierarchical time delay and conditional alarm triggers. Bedside alarms were tallied for 4 weeks each in the immediate postintervention period and 2-year follow-up. The primary outcome was alarms per monitored patient day. Nurses and families were surveyed preintervention and postintervention. RESULTS: A total of 1509 patients contributed to 2034, 1968, and 2043 monitored patient days which were evaluated in the baseline, follow-up, and 2-year follow-up periods, respectively. The median number of alarms per monitored patient day decreased by 75% in the pediatric intensive care unit (P < .001) and 82% in the acute care cardiac unit (P < .001) with sustained effect at the 2-year follow-up. No increase of rapid response calls, emergent transfers, or code events occurred in either unit. Nursing surveys reported an improved capacity to respond to alarms and fewer perceived nonactionable alarms. Family surveys, however, did not demonstrate improved sleep quality. CONCLUSIONS: Implemented changes to bedside monitor alarms decreased total alarm frequency in both the acute care cardiac unit and pediatric intensive care unit, improving the care provider experience without compromising safety.

Pediatric reference values for myocardial contraction fraction and global function index of the left ventricle: A cardiovascular magnetic resonance study.

BACKGROUND: Cardiovascular magnetic resonance (CMR) derived global function index (GFI) and myocardial contraction fraction (MCF) were identified as useful imaging markers to assess left ventricular (LV) cardiac performance and can provide prognostic information for several cardiac diseases. As pediatric reference values are lacking, the aim of this retrospective study was to establish these values. METHODS: 154 CMR examinations of healthy children and adolescents (4-18 years) were included. LV end-diastolic, end-systolic and stroke volumes, ejection fraction (LVEF) and myocardial mass were measured using short axis stacks. Results were used to calculate LVGFI and LVMCF. Statistically, the Lambda-Mu-Sigma (LMS)-method was applied to create percentile curves and tables. RESULTS: The mean age (standard deviation) of the subjects was 13.8 (2.8) years, 102 were male (66%). Mean LVGFI was 46.3 (6.0)% and mean LVMCF was 110.6 (19.9) %. Both, LVGFI and LVMCF decreased significantly with age (LVGFI: r = -0.30, p < 0.001; LVMCF: -0.30, p < 0.001). There was no statistical difference between girls and boys (p all >0.05). Strong correlations between LVGFI and LVMCF (r = 0.78, p < 0.001) as well as between LVGFI and LVEF (r = 0.80, p < 0.001) were documented whereas the correlation of LVMCF and LVEF was weaker (r = 0.32, p < 0.001). Univariable and multivariable regression analysis demonstrated that LVGFI was strongly associated with age whereas LVMCF was associated with weight. Percentile curves and tables were created accordingly. CONCLUSION: We provide pediatric CMR reference values for the new cardiac functional markers LVGFI and LVMCF. These may improve the interpretation of clinical CMR studies and can be used for future research studies.

Nutrition Provision in Children with Heart Disease on Extracorporeal Membrane Oxygenation (ECMO).

Nutrition provision for children with heart disease supported with extracorporeal membrane oxygenation (ECMO) involves nuanced decision making. We examined nutrition provision while on ECMO in the CICU and the relationship between energy and protein adequacy and end organ function as assessed by pediatric sequential organ failure assessment (pSOFA) scores in children with heart disease supported with ECMO. Children (≤ 21 years-old) with congenital or acquired heart disease who received ECMO in the cardiac intensive care unit were included. There were 259 ECMO runs in 252 patients over an 8-year study period (2013-2020). Median energy delivery and adequacy were 26.1 [8.4, 45.9] kcal/kg/day and 58.3 [19.8, 94.6]%, respectively. Median protein delivery and adequacy were 0.98 [0.36, 1.64] g/kg/day and 35.7 [13.4, 60.3]%, respectively. pSOFA increased by a median of four points during the ECMO run. Change in pSOFA score was not associated with energy or protein adequacy (p = 0.46 and p = 0.72, respectively). Higher energy and protein adequacy-from parenteral nutrition-correlated with increased hospital-acquired infections (HAIs, p = 0.031 and p = 0.003, respectively). Achieving nutritional adequacy was dependent on the use of parenteral nutrition. Similar clinical outcomes with regard to end organ function but with an increased incidence of HAIs suggests the need to explore the role of optimal enteral nutrition delivery on ECMO.

Comparison of Risk-Scoring Systems in Predicting Kawasaki Disease Associated Coronary Artery Dilation in a North American Cohort.

Scoring systems used to predict morbidity in children with Kawasaki disease (KD) have been developed and validated in Asian populations. The objective of this study was to assess their utility in predicting the development of coronary artery dilation in children with KD in North America. This was a secondary analysis of a National Institutes of Health / National Heart, Lung, and Blood Institute (NIH/NHLBI) Pediatric Heart Network public use dataset from a multicenter, randomized controlled trial of pulse steroid therapy in KD in a North American cohort. The primary outcome of interest was development of coronary artery dilation. The Harada, Kobayashi, Egami, and Sano scoring systems, originally developed to predict risk of intravenous immunoglobulin (IVIG) resistance in Kawasaki patients in Japan, were applied to this cohort. Subjects were kept in the analysis only if there were complete data for every element of each scoring system-Harada (n = 132), Kobayashi (n = 88), Egami (n = 139), and Sano (n = 82). Patients classified as high-risk by the Harada score were more likely to have significant coronary artery dilation (p = 0.042), were more likely to require IVIG retreatment (p = 0.002), and were more likely to require hospital readmission (p < 0.001). The Egami, Kobayashi, and Sano scores were not predictive for any measured outcome. The Harada score can be useful in identifying KD patients at risk for developing coronary artery dilation and IVIG resistance. The Harada score has demonstrated higher sensitivity but lower specificity, making it a valuable screening tool that may benefit from supplementary diagnostic methods.

Atrial Arrhythmia and Bradycardia as a Presentation of Congenital Long QT Syndrome.

We present a term newborn with atrial arrhythmia on the first day of life (DOL). An echocardiogram showed normal structure and normal function; laboratory testing showed normal electrolytes and thyroid function. After initiation of flecainide, the EKG on DOL 2 showed significant and increasing bradycardia with atrial arrhythmia and extremely prolonged QTc interval. Flecainide was stopped and esmolol started. After 6 h of treatment, atrial tachycardia was suppressed, and the rhythm converted to sinus. Genetic testing found variants of unknown significance in the ALPK3 gene and KCNQ1 gene, which has been associated with long QT syndrome (LQTs). LQTs in infants can present as bradycardia, 2:1 AV block, or torsades de pointes. Our review of the literature found only one other case report of atrial arrhythmia in a newborn with congenital LQTs. Diagnosis of LQTs via EKG alone is difficult in neonates since the ST segment and T wave on the first DOL are usually flattened, making correct measurement of the QTc interval difficult. ß-blockers, the first line of treatment for LQTs, are known to shorten QTc intervals and prevent arrhythmia events. As in our patient, ß-blockers may be helpful for atrial arrhythmia prevention in patients with adrenergically mediated atrial tachycardia. In conclusion, atrial arrhythmia with bradycardia can be a presentation of congenital LQTs and be difficult to recognize. For neonates with this presentation with no evidence of myocarditis, congenital heart disease, or significant respiratory illness, genetic congenital LQTs should be highly suspected, especially when associated with low resting heart rates.

A Novel Approach to Mentorship in Pediatric Cardiology: A Group for Women.

Gender disparities for female physicians in academic medicine are longstanding. Female pediatric cardiologists experience inequities in scholarship opportunities, promotion, leadership positions, and compensation. Mentorship groups have been successfully implemented in other subspecialities with promising results. We created a peer mentorship group for female pediatric cardiologists in the Northeast and completed a needs assessment survey of eligible participants. Our goal was to better understand the current challenges and identify resources to overcome these barriers. Our objectives were to (1) describe the creation of a novel mentorship program for female pediatric cardiologists and trainees in the Northeast United States, and (2) report the results of a formal needs assessment survey of all eligible participants. All female pediatric cardiology fellows and practicing pediatric and adult congenital heart disease specialists from 15 academic centers in New England were invited to join a free group with virtual meetings. A formal needs assessment survey was provided electronically to all eligible members. The vast majority of respondents agreed that the Women in Pediatric Cardiology (WIPC) group is a valuable networking and mentorship experience (90%) and would recommend this group to a colleague (95%). Members have witnessed or experienced inequities in a broad range of settings. Common challenges experienced by respondents include dependent care demands, lack of mentorship, inadequate research support, and inequitable clinical responsibilities. Resources suggested to overcome these barriers include mentorship, sponsorship, transparency in compensation, and physician coaching. Mentorship groups have the potential to address many challenges faced by women in medicine. The WIPC Northeast program provides a forum for community, collaboration, education, and scholarship.

4-Dimensional Right Ventricular Outflow Tract Segmentation to Evaluate Candidacy for Transcatheter Pulmonary Valve Replacement.

Candidacy for transcatheter pulmonary valve replacement utilizing newer valve platforms is aided by industry-driven fit analyses. We report augmentation of this decision-making process by visualization of 4-dimensional cine imaging of patient-specific right ventricular outflow tract anatomy and a virtual valve model within a virtual reality platform.

Cognitive biases in pediatric cardiac care.

Medical practitioners are entrusted with the pivotal task of making optimal decisions in healthcare delivery. Despite rigorous training, our confidence in reasoning can fail when faced with pressures, uncertainties, urgencies, difficulties, and occasional errors. Day-to-day decisions rely on swift, intuitive cognitive processes known as heuristic or type 1 decision-making, which, while efficient in most scenarios, harbor inherent vulnerabilities leading to systematic errors. Cognitive biases receive limited explicit discussion during our training as junior doctors in the domain of paediatric cardiology. As pediatric cardiologists, we frequently confront emergencies necessitating rapid decision-making, while contending with the pressures of stress, fatigue, an earnest interest in "doing the right thing" and the impact of parental involvement. This article aims to describe cognitive biases in pediatric cardiology, highlighting their influence on therapeutic interventions for congenital heart disease. Whether future pediatric cardiologists or experienced professionals, understanding and actively combating cognitive biases are essential components of our ongoing medical education. Furthermore, it is our responsibility to thoroughly examine our own practices in our unwavering commitment to providing high-quality care.

Single Center Retrospective Evaluation of Coronary Artery Fistula Outcomes.

There is a paucity of literature describing long-term outcomes of patients with coronary artery fistula with most manuscripts focusing on those requiring interventions. We describe single-center outcomes of coronary artery fistulas including those not requiring intervention. We performed a retrospective review of the electronic medical record and identified all patients with a diagnosis of coronary artery fistula over the last 10 years. 158 patients were identified with a coronary artery fistula. The mean age at diagnosis was 5.8 years (SD ± 5.9). There was a male (55%, n = 87) predominance. Concomitant congenital heart lesion was present in 49% (n = 77) and a genetic anomaly was found in 18% (n = 29). No ischemic changes on electrocardiogram or ECG-stress test were observed. The mean follow-up was 5.0 (SD ± 3.8) years. Most patients (94%, n = 149) did not undergo an intervention. Of those 63% (n = 94) had at least one follow-up echocardiogram. There was spontaneous coronary artery fistula closure in 44% (n = 41), 8% (n = 8) decreased in size, and 48% (n = 45) were unchanged. No patient had enlargement of the coronary artery fistula over time. Additionally, tiny and small coronary artery fistulas showed no significant clinical changes in coronary artery dimensions, left ventricle dimensions and function over time. Seven patients required intervention; two patients underwent surgical ligation and five underwent catheter-based intervention. Most patients with coronary artery fistula in our cohort did not require intervention and over half either closed spontaneously or decreased in size with routine follow-up.