Pediatric cardiology education requires mastery of physiology, anatomy, and spatial reasoning. There is limited published data on pediatric cardiology education for medical trainees. Mobile applications have emerged as a...Pediatric cardiology education requires mastery of physiology, anatomy, and spatial reasoning. There is limited published data on pediatric cardiology education for medical trainees. Mobile applications have emerged as a new asynchronous option to supplement formal medical education. We performed an acceptability study using a mobile educational app, Heartpedia™ (HP) to assess itsusefulness to learners in CHD education. This was a single center prospective cohort study of pediatric interns during their cardiology rotation. Individuals were given a tutorial on how to install and use HP but were not required. Surveys and tests were administered before and after their month-long rotation. The tests contained questions based on two cardiac lesions: a perimembranous ventricular septal defect (VSD) and Truncus Arteriosus (TA) Type 1. 53 pediatric interns participated in the study with 66% reported to have used HP. App usage correlated with lesion test score improvement for TA but not VSD. >80% of learners described HP as a helpful, easy to use educational resource for learners, and agreed it improved their understanding of CHD lesions and their management. The Heartpedia™ application was used by most residents during their rotation and found it helpful for their understanding of anatomy and physiology and yielded improved understanding of complex CHD. While app usage showed some improvement in knowledge gains on certain cardiac lesions, further study is warranted to assess the efficacy of mobile learning applications for CHD education.
A standardized fetal cardiology curriculum is beneficial for categorical pediatric cardiology fellowship training. Understanding of the discipline plays an increasingly important role in clinical practice. Accordingly, k...A standardized fetal cardiology curriculum is beneficial for categorical pediatric cardiology fellowship training. Understanding of the discipline plays an increasingly important role in clinical practice. Accordingly, knowledge of these topics is assessed on national examinations including the Pediatric Cardiology Board Certification Exam. We developed a 3-week fetal cardiology curriculum, which included 6 didactic lectures and 4 case-based learning sessions. We delivered this condensed lecture series annually for our categorical pediatric cardiology fellows from 2018 to 2025. Fellows' self-reported knowledge before and after the course and score changes on the fetal cardiology section of the board examination (BE) were evaluated. Fifty-six fellows completed at least one year of the curriculum, and 22 surveys were received. The fellows felt strongly that "the course met personal expectations" (average 86.8%, standard deviation 16.4%) and "improved current knowledge" (average 95.2%, standard deviation 8.5%). Self-reported scores showed perceived improvement in knowledge in all domains. We found that case-based learning was helpful in improving understanding of topics; we increased this modality in subsequent years. Between 2018 and 2024, the gap between our graduates' BE scores and the national average in percentage of correct answers in fetal topics decreased from - 20.3% to 0%. Implementation of a dedicated, condensed fetal cardiology curriculum demonstrated increased confidence and understanding of fetal cardiology. Concurrently, we noted a positive trend in BE scores.
Isolated mitral valve regurgitation is an extremely rare presentation of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). When left ventricle systolic function is preserved, ALCAPA can be...Isolated mitral valve regurgitation is an extremely rare presentation of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). When left ventricle systolic function is preserved, ALCAPA can be easily overlooked. Herein, we present a case series of 3 patients with ALCAPA and preserved systolic function who presented with isolated mitral valve regurgitation (MVR). This manuscript underscores the importance of considering ALCAPA in any patient presenting with isolated MVR.
Kim ME, Lewin M, Penny DJ
… +19 more, Sadiq M, Tham E, Idorn L, Vieira de Campos C, Sanatani S, Dubin AM, Kim J, Raimoindi F, Johnson JN, Gaies M, Shidhika F, Gajewski K, Kenny D, Gentles T, Voges I, Kumar RK, Pass R, Rossano J, McMahon CJ
Organizational culture is increasingly recognized as a determinant of team performance, patient safety, and clinician well-being, yet remains poorly characterized in pediatric cardiology. Existing approaches typically as...Organizational culture is increasingly recognized as a determinant of team performance, patient safety, and clinician well-being, yet remains poorly characterized in pediatric cardiology. Existing approaches typically assess individual domains rather than culture as a multidimensional construct. We conducted a multinational exploratory study to examine physician perceptions of organizational culture and to develop an integrated conceptual framework. We performed a cross-sectional, anonymized survey of pediatric cardiology physicians across tertiary congenital heart programs internationally. The survey, informed by established constructs including the Safety Attitudes Questionnaire, assessed domains such as psychological safety, leadership, team structure, workload, and medicolegal climate. Analyses were descriptive and hypothesis-generating. A total of 133 physicians from 27 centers participated. Overall, 54.9% agreed that organizational culture influenced professional performance (85.0% agreed or were neutral), with limited disagreement across regions. Additionally, 36.2% reported negative spillover into personal or family life. Workload intensity and administrative burden were consistently identified as dominant stressors (≥ 60% across regions). Structural and team-related challenges were also prominent: 42.9% reported silo mentality in large teams, and 41.4% identified insufficient staffing as detrimental to culture. Response patterns were broadly consistent across geographic regions. Respondents demonstrated receptivity to improvement strategies, including open forums and structured feedback mechanisms. Organizational culture is widely perceived to influence performance and well-being in pediatric cardiology. Across healthcare systems, physicians identified consistent structural and relational drivers, including workload, administrative burden, and team integration. The proposed conceptual framework provides a basis for reflection and future research.
Infants with hypoplastic left heart syndrome undergoing single ventricle (SV) palliation may need early heart transplant, but optimal referral timing is unknown, and referrals may be inequitable. We performed a retrospec...Infants with hypoplastic left heart syndrome undergoing single ventricle (SV) palliation may need early heart transplant, but optimal referral timing is unknown, and referrals may be inequitable. We performed a retrospective study of infants undergoing stage 1 SV palliation (S1P) from 2016-2022 enrolled in the National Pediatric Cardiology-Quality Improvement Collaborative (NPC-QIC). Competing risk methods were used to evaluate predictors of Transplant Referral (TxR) and death without TxR. Neighborhood opportunity was determined through use of Child Opportunity Index (COI). A total of 2,960 infants were included. Infants from lower opportunity neighborhoods had lower median birth weights, were less likely to be prenatally diagnosed, and were more likely to require mechanical ventilation pre-operatively than those from higher opportunity neighborhoods. At one year, an estimated 79% of infants were alive, 16% had died, and 5% had a TxR, with no differences in outcomes by neighborhood opportunity or race. In multivariable analysis, neither neighborhood opportunity nor race was associated with either outcome when controlling for known risk factors. Pre-operative tricuspid regurgitation was most predictive of TxR and was also a major risk factor for death without TxR. Multiple non-cardiac risk factors were predictive of death without TxR. One-year survival after S1P approached 80%, though death without TxR was three times more common than TxR. No clear disparities were seen, though non-cardiac risk factors for death were more prevalent in those from lower opportunity neighborhoods. Standardized guidelines may help identify high risk infants who may benefit from earlier TxR.
Critical congenital heart disease (CCHD) screening using pulse oximetry was mandated by all states between 2011-2018. We sought to evaluate the effect on the age at initial admission to a children's hospital, the hospita...Critical congenital heart disease (CCHD) screening using pulse oximetry was mandated by all states between 2011-2018. We sought to evaluate the effect on the age at initial admission to a children's hospital, the hospital length of stay (LOS), and in-hospital mortality before and after pulse oximetry screening (POS) mandates. A multicenter retrospective cohort study of the Pediatric Health Information System was performed evaluating neonates with CCHD born at ≥34 weeks gestation admitted to a children's hospital after the first day of life. We evaluated changes before and after POS mandates in mean age at initial admission and LOS using interrupted time series (ITS) analysis to identify post-POS mandate level and trend changes. There was inadequate power to analyze in-hospital mortality rates with ITS or logistic regression. 12,298 neonates were included from 39 children's hospitals in 22 states and District of Columbia. After POS mandates, neonates tended to be admitted to children's hospitals earlier, with decreases in level (2.2 days; p<0.001) and trend (annual change: +5% to no change; p=0.03). Post-mandate neonates had longer mean LOS with a level increase of 6 days (p=0.03) and no significant change in trend. Descriptive data showed in-hospital mortality decreased from 7.5% pre-mandate to 6.6% post mandate across the cohort, but could not be further evaluated using ITS modeling. Subgroup analyses with only high sensitivity diagnoses and excluding outflow tract diagnoses yielded similar findings. POS mandates were associated with earlier age of initial admission to a children's hospital and increased hospital LOS.
Intrapericardial administration of the glucocorticoid medication triamcinolone is reported useful in the treatment of recurrent pericardial effusion in adult patients. Nevertheless, the studies on triamcinolone use in pe...Intrapericardial administration of the glucocorticoid medication triamcinolone is reported useful in the treatment of recurrent pericardial effusion in adult patients. Nevertheless, the studies on triamcinolone use in pediatric patients are scarce, and the effectiveness and side effect profile of intrapericardial triamcinolone in children is not well characterized. This single center retrospective case series seeks to expand on the efficacy, side effects, and safety of intrapericardial triamcinolone in pediatric patients suffering from recurrent idiopathic pericardial effusions. Nine pediatric patients who received intrapericardial triamcinolone for recurrent idiopathic pericardial effusion at a single center from 2015 to 2025 were included. The pericardial effusion progression was observed with serial echocardiograms and new symptoms appeared after triamcinolone administration. Five patients demonstrated resolution of pericardial effusion, three had improvement, and one relapsed after intrapericardial triamcinolone administration. Five patients suffered from short-term steroid-like side effects that resolved prior to discharge, and two died of underlying disease rather than treatment with triamcinolone. These findings are consistent with the idea that intrapericardial triamcinolone can be utilized as a safe and effective adjunctive treatment option in children with recurrent idiopathic pericardial effusion.
Fetal arrhythmias, including tachycardias, bradycardias and irregular rhythms, may require close surveillance and management to prevent fetal heart failure, premature delivery and stillbirth. With early detection and app...Fetal arrhythmias, including tachycardias, bradycardias and irregular rhythms, may require close surveillance and management to prevent fetal heart failure, premature delivery and stillbirth. With early detection and appropriate management of pathological fetal rhythms, 90-95% of fetuses with even high-risk arrhythmias are live-born and can be delivered at term gestation. Fetal heart rate monitoring (FHRM) is a feasible method for ambulatory detection of new-onset or recurrent arrhythmias. When indications for FHRM are met, prescribed monitoring, guided and supported by the healthcare team, can empower pregnant individuals in the management of their pregnancies. Despite several studies showing utility of ambulatory FHRM, fetal cardiologists and perinatologists face challenges in successfully implementing FHRM programs. Under the auspices of the Fetal Heart Society, the Fetal Cardiology Program Leaders Committee formed a writing group to develop a consensus-based ambulatory FHRM process to be used as a guide for providers caring for pregnancies complicated by or at risk of manifesting fetal arrhythmias.
Neurodevelopmental impairments are common in congenital heart disease (CHD) and fetal brain volume is an important predictor of outcomes. Social determinants of health (SDOH) and environmental factors influence brain gro...Neurodevelopmental impairments are common in congenital heart disease (CHD) and fetal brain volume is an important predictor of outcomes. Social determinants of health (SDOH) and environmental factors influence brain growth in other populations and likely play a neurodevelopmental role in CHD. This study evaluated the influence of SDOH and environmental factors on fetal and neonatal brain volume, growth, and risk of brain injury in CHD. This prospective single-center longitudinal cohort study enrolled fetuses with severe CHD to undergo third-trimester fetal and preoperative brain MRIs. Controls underwent third-trimester brain MRIs. Participants completed SDOH and environmental exposure surveys. Fetal and neonatal brain volumes, brain growth, and presence of white matter injury (WMI) were assessed. 57 CHD patients and 24 controls were enrolled, resulting in 33 fetal and 44 neonatal MRIs in the CHD group and 21 fetal control MRIs. Several SDOH and environmental factors, including maternal smoking, were associated with smaller brain volume and slower brain growth in CHD but not in controls. With CHD, repeated-measures analysis showed smaller fetal brain volume (coeff: - 13.3, 95% CI - 25.5, - 1.1, p = 0.03) and slower growth (coeff: - 2.5, 95% CI - 5.0, - 0.07, p = 0.04) with exposure to any risk factor. Patients with moderate to severe WMI had significantly lower mean COI scores compared to those with no or minimal WMI (58.28 vs. 37.28, p = 0.04), but lost significance when adjusted for GA. SDOH and environmental exposures influence fetal brain growth and preoperative brain injury risk in CHD. These results highlight additive environmental prenatal risks which may be amenable to early intervention.
The care of patients with pediatric and congenital heart disease is intrinsically complex and relies on coordinated multidisciplinary teamwork. While advances in diagnostics and intervention have improved survival, varia...The care of patients with pediatric and congenital heart disease is intrinsically complex and relies on coordinated multidisciplinary teamwork. While advances in diagnostics and intervention have improved survival, variability in outcomes persists and is increasingly linked to team performance, decision-making, and organizational culture. This Perspective draws on evidence from pediatric cardiology, healthcare systems research, and organizational science to examine the role of team-based care in pediatric and congenital cardiology. Emphasis is placed on multidisciplinary decision-making, team dynamics, leadership, and organizational culture, with the aim of stimulating reflection and informing everyday clinical practice rather than providing a systematic or exhaustive review. Effective teamwork is associated with improvements in patient safety, clinical outcomes, patient and family experience, and staff well-being. Multidisciplinary decision-making forums, empowered nursing roles, and psychologically safe cultures emerge as central to high performance. Leadership, accountability, and transparency of outcomes data strongly influence team behaviour, while evidence from pediatric cardiology highlights the vulnerability of clinical decision-making to cognitive bias, heuristics, and organizational context. Optimising decision-making processes, fostering supportive organizational cultures, and investing in team development represent important opportunities to improve outcomes. We propose a practical Measurement-Feedback-Simulation (MFS) framework to support the measurement and improvement of teamwork as a clinical competency.
The optimal right ventricular outflow tract (RVOT) conduit in the Ross procedure remains controversial. This study addresses outcomes of decellularized (DH) and cryopreserved homografts (CH) for RVOT reconstruction. Pati...The optimal right ventricular outflow tract (RVOT) conduit in the Ross procedure remains controversial. This study addresses outcomes of decellularized (DH) and cryopreserved homografts (CH) for RVOT reconstruction. Patients aged 8 years or older who underwent the Ross procedure using DH or CH were reviewed. Conduit durability and pulmonary valve function were compared between both groups. A total of 59 patients were included (26 DH-patients and 33 CH-patients). The median age at Ross procedure (DH: 19 (IQR: 13-30) vs. CH: 15 (IQR: 12-23) years, p = 0.150) and the homograft diameters (DH: 24 (22-26) vs. CH: 23 (22-24) mm, p = 0.163) were similar between the groups. When comparing postoperative events within the first 5 years between the groups, none of the DH-patients experienced infective endocarditis, homograft explantation, or catheter intervention. Whereas, in the CH group, 2 patients developed infective endocarditis, 2 underwent explantation, and one underwent Melody implantation. Following up with serial echocardiography for up to 5 years, none of the DH-patients developed significant pulmonary stenosis (PS > 40 mmHg), and one patient developed significant pulmonary regurgitation (PR: moderate or worse). In contrast, 2 patients in the CH group demonstrated significant PS, and 5 patients showed significant PR. Freedom from graft dysfunction, defined by endocarditis, explantation, Melody implantation, significant PS, and significant PR, tended to be better in DH than CH (90.9% vs. 79.0% at 5 years, p = 0.155). Our results demonstrated better short-term valve performance with DH than with CH following the RVOT reconstruction during the Ross procedure.
To evaluate whether transcatheter patent foramen ovale (PFO) closure is associated with higher rates of complete migraine remission in children with migraine and a concomitant right-to-left shunt. We conducted a single-c...To evaluate whether transcatheter patent foramen ovale (PFO) closure is associated with higher rates of complete migraine remission in children with migraine and a concomitant right-to-left shunt. We conducted a single-center retrospective cohort study of children with migraine and PFO-mediated right-to-left shunting. The primary exposure was transcatheter PFO closure; the primary outcome was complete migraine remission (no attacks for ≥ 3 consecutive months) assessed at 1, 3, 6, and 12 months post-discharge. Kaplan-Meier and multivariable Cox regression with a 3-month landmark were adjusted for right-to-left shunt (RLS) grade, age, sex, duration of the current headache episode, body mass index, and concomitant flunarizine prophylaxis. Among 66 children (mean age 10.3 years; 67% male), 19 underwent transcatheter PFO closure, and 47 received medical management. Complete migraine remission within 12 months occurred in 17/19 (89.5%) of closure patients versus 20/47 (42.6%) of non-closure patients (log-rank p = 0.002). In the adjusted Cox model, closure was associated with remission (hazard ratio [HR] 3.60, 95% confidence interval [CI] 1.38-9.42, p = 0.009); the association persisted after stratification for RLS (HR 3.18, 95% CI 1.26-8.04). A graded pattern was observed across three strata: no treatment 34.4%, flunarizine 60.0%, closure 89.5% (Kendall τ = 0.45, p < 0.001). All 19 procedures achieved complete closure without complications. Transcatheter PFO closure was associated with higher rates of complete migraine remission after adjustment for shunt grade, prophylaxis, and other covariates. Findings are hypothesis-generating; prospective randomized studies are needed before closure can be recommended for pediatric migraine outside clinical trials.
Endovascular stent implantation is an established treatment for native and recurrent aortic coarctation in children. Stents are generally assumed to become endothelialized by neointima within months after implantation. R...Endovascular stent implantation is an established treatment for native and recurrent aortic coarctation in children. Stents are generally assumed to become endothelialized by neointima within months after implantation. Reported late complications include restenosis, aneurysm formation, and stent fracture. Very late complete stent dislodgement, however, has not been reported to date. We report two pediatric patients who experienced very late dislodgement of previously implanted aortic isthmus stents - each nearly seven years after implantation. In the first case, a stent implanted in infancy for recoarctation dislodged completely during technically difficult retrograde catheterization of the aortic valve in a child with Shone complex. In the second case, the majority of a fractured stent migrated into the ascending aorta during advancement of a long sheath for re-stenting of the isthmus. In both patients, the displaced stents were successfully retrieved percutaneously using snare systems, and definitive re-stenting was performed. Notably, displacement occurred without major aortic wall injury, suggesting limited long-term biological fixation. These cases demonstrate that even years after implantation, aortic isthmus stents placed in early childhood may not be firmly integrated into the vessel wall. Growth-related vascular remodeling, reduced wall apposition, and aneurysmal changes may contribute to progressive loss of anchoring. Catheter manipulation or sheath advancement can act as triggering events. Awareness of potential whole-stent mobilization is essential during re-interventions in pediatric patients with longstanding isthmus stents.
Pulmonary arterial hypertension causes significant morbidity and mortality in pediatric patients. Current guidelines speak about the need for new strategies to diagnose and monitor pulmonary hypertension in pediatrics. D...Pulmonary arterial hypertension causes significant morbidity and mortality in pediatric patients. Current guidelines speak about the need for new strategies to diagnose and monitor pulmonary hypertension in pediatrics. Digital subtraction pulmonary angiography is a useful tool to assess lung vasculature and parenchymal perfusion deficits. A Pulmonary Vascular Underperfusion Score has been previously published and used to assess pulmonary vasoreactivity testing response in premature infants with bronchopulmonary dysplasia and pulmonary hypertension. By adopting this Pulmonary Vascular Underperfusion Score, we performed a retrospective review for evaluating children with pulmonary arterial hypertension. Our study demonstrates that the Pulmonary Vascular Underperfusion Score is strongly associated with pulmonary vascular resistance in pediatric patients with pulmonary arterial hypertension. Specifically, a score of ≥ 1.5 reliably identified patients with elevated pulmonary vascular resistance, both at baseline and during vasodilator testing, with high sensitivity and specificity. These findings suggest that Pulmonary Vascular Underperfusion Score may be a useful tool in risk stratification of children with pediatric pulmonary artery hypertension.
Vascular access for endovascular procedures can be challenging in pediatric patients with congenital heart disease. We present two patients in which a left mini-thoracotomy was utilized to access the descending aorta for...Vascular access for endovascular procedures can be challenging in pediatric patients with congenital heart disease. We present two patients in which a left mini-thoracotomy was utilized to access the descending aorta for endovascular aortic stenting. The first patient was a 5-year-old male who had undergone a Fontan pathway and developed a significant stenosis of the distal arch. The second patient was a 10-month-old born with a coarctation who developed recurrent stenosis and a pseudoaneurysm around an existing aortic stent. Utilizing this hybrid approach, both underwent successful retrograde, aortic stent deployment. Both stents can be dilated to adult size in the future. To our knowledge these are the first published descriptions of this technique in the pediatric population.