This study aims to evaluate the clinical characteristics and outcomes of children diagnosed with sinus node dysfunction.

This was a retrospective review of patients diagnosed with sinus node dysfunction in two tertiary paediatric cardiology centres in Turkey from January 2011 to June 2022.

In all, 77 patients (50, 64.9% males) were included, with a mean age of 8.2 ± 6.3 years and a mean weight of 28.2 ± 18.8 kg. While age-incompatible bradycardia and pauses were the most common rhythm disturbances, syncope, presyncope, and dizziness (n:33, 43%) were the most frequent initial symptoms. Structural heart disease was present in 58 (75.3%) of the 77 patients, 47 (61%) of whom were congenital. The most commonly associated CHDs were transposition of the great arteries (n:8), atrial septal defect (n:7), and atrioventricular septal defect (n:5). Seven of them also had left atrial isomerism. The remaining 19 patients were isolated. Four patients had SCN5A mutation (two of them were siblings) and two of them had Emery–Dreifuss muscular dystrophy.

Although sinus node dysfunction is rare in children, it has been diagnosed with increasing frequency with structural heart disease, especially in patients who have undergone corrective cardiac surgery related to atrial tissue. Since sinus node dysfunction can occur at any time postoperatively, these patients should be kept under constant control. If symptomatic sinus node dysfunction is confirmed, permanent pacing is an effective therapeutic modality.

To highlight veno-venous collaterals and portosystemic shunts as possible causes of cyanosis requiring evaluation or treatment below the diaphragm in patients with single-ventricle physiology during staged palliation.

Cyanosis can complicate staged single-ventricle palliation and is often evaluated with imaging isolated to the thorax. As a result, lesions below the diaphragm, such as veno-venous collaterals and portosystemic shunts, may be missed. Identification is important since these lesions can be treated via transcatheter techniques.

This study is a retrospective, single-centre case series of six patients.

Two patients following stage 2 palliation had multiple small veno-venous collaterals connecting to a reconstituted azygos with retrograde flow to below the diaphragm. Accessing veno-venous collaterals closer to their entry points below the diaphragm was more optimal for occlusion. Two patients following stage 3 palliation had veno-venous collaterals originating below the diaphragm and connecting to the pulmonary veins. Both patients required infrarenal inferior vena cava contrast injection to identify the veno-venous collateral. The final two patients had heterotaxy with either stage 2 or 3 palliation and were found to have bilateral pulmonary arteriovenous malformations secondary to a portosystemic shunt diverting hepatic factor away from the lungs. All patients had successful device closure of their veno-venous collaterals or portosystemic shunts.

The six patients discussed demonstrate the importance of evaluating below the diaphragm in single-ventricle physiology patients presenting with cyanosis.

Danon disease is an X-linked disorder caused by variants in the lysosome-associated membrane protein-2 (LAMP2) gene located on Xq24. Due to its inheritance in an X-chromosome dominant manner, males typically experience more severe manifestations than females.

The whole exome sequencing was conducted on a cohort of 218 children diagnosed with hypertrophic cardiomyopathy; four children with hypertrophic cardiomyopathy carrying the LAMP2 variants were diagnosed at our centre. Variants in the LAMP2 gene were summarised, and their pathogenicity and conservation were analysed using bioinformatics methods. A retrospective analysis of genotype-phenotype associations was also conducted in conjunction with previously reported cases.

Four patients with Danon disease were diagnosed in our single centre by gene sequencing; they all presented with myocardial hypertrophy as the initial manifestation. Both male patients manifested symptoms from infancy, while disease onset in the two female cases occurred below the average age reported for females. Through gene sequencing, a total of four variants were identified in these four patients, including one splicing variant: c.865-1G>C, one loss of heterozygosity variant: loss1 exon:4-9), one frameshift variant: c.973delG(p.(L325Wfs×21)), and one stop codon variant: c.467T>G(p.(L156*)).

This study identified four patients with LAMP2 gene variants, thereby enriching the documented genetic landscape of LAMP2-associated disorders. Bioinformatics analyses corroborated the pathogenicity of these variants. Additionally, we emphasised that women with suspected Danon disease should be thoroughly evaluated, and the possibility of implantable cardioverter defibrillator implantation and heart transplantation should be considered and discussed as early as possible.

Surgical pulmonary valve replacement is commonly required to palliate patients with CHD affecting the right ventricular outflow tract; however, concerns remain about mid- and long-term durability. Post-operative short-term anticoagulation has been hypothesised to improve valve durability.

This is a single-centre, retrospective study of paediatric patients who underwent surgical pulmonary valve replacement and received a direct oral anticoagulant in addition to aspirin post heart valve insertion. The primary objective was a composite safety score consisting of clinically relevant non-major bleeding, major bleeding, bleeding-related readmission, and medication discontinuation.

The study analysed 34 patients with a median age 14 years (Interquartile range (IQR): 11, 15) and weight 45 kg (IQR: 35, 55). Ten patients met the composite endpoint (10/34, 29%), with 4 patients experiencing major bleeding (4/34, 12%), 6 experiencing clinically relevant non-major bleeding (6/34, 18%), and 3 patients being readmitted within 90 days of surgical pulmonary valve replacement for bleeding (3/29, 8.8%) resulting in 10 patients discontinuing medication early (10/34, 29%). Lower weight was identified as a significant risk factor for adverse event development (p = 0.04).

We observed a higher overall bleeding rate, driven predominately by clinically relevant non-major bleeding events, than other studies using short-term anticoagulation after surgical pulmonary valve replacement. Additional studies should be aimed at evaluating the dosing and safety of direct oral anticoagulants in children in the post-operative period.

Endomyocardial biopsy remains the gold standard for cardiac cellular rejection surveillance after heart transplantation. We studied a novel non-invasive index of left ventricular relaxation to detect cardiac cellular rejection in paediatric heart transplant patients.

This is a single-centre retrospective study of paediatric heart transplant patients who underwent endomyocardial biopsy from June 2014 to September 2021. Left ventricular relaxation index was calculated as the sum of diastolic tissue Doppler imaging velocities (E) of the left ventricular lateral, septal, and posterior walls divided by the percentage of the left ventricular posterior wall thinning by M-mode. Statistical analysis included t-tests and Mann-Whitney tests to compare means and medians between treatment and non-treatment groups. We used the cut-off with the maximum Youden index to compare the sensitivity and specificity of left ventricular relaxation index to detect rejection.

The study included 65 patients who underwent 246 cardiac catheterizations and endomyocardial biopsies. Out of 246, 192 procedures were included and 54 were excluded due to recent transplants or lack of echocardiographic data. A total of 114 demonstrated Grade 0R, 68 Grade 1R, 8 Grade 2R, and 2 Grade 3R allograft rejection. The difference in mean left ventricular relaxation index between treatment versus non-treatment groups (2R, 3R vs. 0R, 1R) was not statistically significant (p = 0.917). A left ventricular relaxation index cut-off of 0.73 had the highest Youden index with good sensitivity (100%) and poor specificity (23%) for detecting rejections with grades 2R and 3R.

Left ventricular relaxation index, a novel index of left ventricular relaxation, was not a sensitive or specific predictor of cardiac cellular rejection in paediatric heart transplants.

Epicardial fat thickness also increases in insulin resistance and diabetes mellitus patients. In our study, we aimed to compare the epicardial fat thickness and interventricular septum diameter in infants of mothers with diabetes with infants of mothers without diabetes. Also, to determine the effect of birth weight on the epicardial fat thickness and the interventricular septum diameter in infants of mothers with diabetes.

Between November 2022 and June 2023, infants of mothers with diabetes and healthy infants were compared. According to birth weight, infants of mothers with diabetes were divided into two groups, macrosomic infants (≥ 4000 g) and non-macrosomic infants (2500–4000 g).

A total of 78 infants of mothers with diabetes and 56 infants of mothers without diabetes were evaluated. Epicardial fat thickness and interventricular septum diameter were found to be statistically significantly higher in infants of mothers with diabetes than in healthy infants. Epicardial fat thickness and interventricular septum diameter measurements were found to be statistically significantly higher in the macrosomic of infants than in the non-macrosomic of infants of mothers with diabetes. Asymmetric septal hypertrophy was found to be more common in macrosomic infants of diabetic mothers than in non-macrosomic infants, although not statistically significant. We also found a positive correlation between epicardial fat thickness and asymmetric septal hypertrophy.

Epicardial fat thickness is observed to increase along with neonatal macrosomia and heart mass in gestational diabetes mellitus. The lack of a significant increase in epicardial fat thickness and interventricular septum diameter in non-macrosomic infants of mothers with diabetes may be indicative of good glycaemic control in non-macrosomic infants of mothers with diabetes. Therefore, good glycaemic control during pregnancy and long-term follow-up of infants of mothers with diabetes after birth is important.

Fear of cardiac arrest among parents of infants with heart disease can cause stress and anxiety. Literature is scarce on the effects of cardiopulmonary resuscitation training (CPRt) on anxiety and stress of parents. We analysed the impact of CPRt on anxiety, stress, and comfort levels on parents of infants with heart disease.

Cardiopulmonary resuscitation (CPR) and choking relief manoeuvre (CRM) comfort level, Parental State-Trait Anxiety Inventory (STAI), and Parenting Stress Index (PSI) scores were prospectively collected pre-, immediately post-, and 3 months post-CPRt.

There were 97 participants: 80% (n = 78) mothers/grandmothers and 20% (n = 19) fathers. The mean (SD) age of participants was 28.7 (5.6) years old. There was a significant decrease in STAI across the three time points collected; STAI decreased by 12% from baseline to immediately post-CPRt and 19% from baseline to 3 months post-CPRt (p < .0001). There were no significant changes in PSI across the time points. Baseline to immediately post-teaching, we found that CPRt significantly increased comfort performing CPR, CRM, and comfort in knowing what to do (p=< .001, p=< .001, p=< .001, respectively). Comfort levels persisted elevated when comparing pre- to 3 months post-CPRt (p=< .001, p= .002, p= .001, respectively), maintaining at least a 177% average increase up to 3 months post-CPRt for all aspects.

CPRt can aid in improving anxiety and comfort levels of parents of infants with heart disease around hospital discharge. Parental preparedness and reassurance to know what to do in emergency situations can be enhanced by a simple intervention such as CPRt.

Interprofessional teams in the pediatric cardiac ICU consolidate their management plans in pre-family meeting huddles, a process that affects the course of family meetings but often lacks optimal communication and teamwork.

Cardiac ICU clinicians participated in an interprofessional intervention to improve how they prepared for and conducted family meetings. We conducted a pretest–posttest study with clinicians participating in huddles before family meetings. We assessed feasibility of clinician enrollment, assessed clinician perception of acceptability of the intervention via questionnaire and semi-structured interviews, and impact on team performance using a validated tool. Wilcoxon rank sum test assessed intervention impact on team performance at meeting level comparing pre- and post-intervention data.

Totally, 24 clinicians enrolled in the intervention (92% retention) with 100% completion of training. All participants recommend cardiac ICU Teams and Loved ones Communicating to others and 96% believe it improved their participation in family meetings. We exceeded an acceptable level of protocol fidelity (>75%). Team performance was significantly (p < 0.001) higher in post-intervention huddles (n = 30) than in pre-intervention (n = 28) in all domains. Median comparisons: Team structure [2 vs. 5], Leadership [3 vs. 5], Situation Monitoring [3 vs. 5], Mutual Support [ 3 vs. 5], and Communication [3 vs. 5].

Implementing an interprofessional team intervention to improve team performance in pre-family meeting huddles is feasible, acceptable, and improves team function. Future research should further assess impact on clinicians, patients, and families.

To evaluate the prognostic value of electrocardiographic ventricular repolarisation parameters in children with dilated cardiomyopathy.

A retrospective study was conducted involving 89 children with dilated cardiomyopathy [age 5.24 (4.32, 6.15) years] as the research group, and a control group consisting of 80 healthy children matched for age and sex. Within the research group, there were 76 cases in the survival subgroup and 13 cases in the death subgroup. Ventricular repolarisation parameters were measured.

(1) Compared to the control group, both QTcmax and QTcmin were significantly prolonged in the research group (P < 0.01). Additionally, Tp-Te /QT ratios for leads III, aVL, V1, V2, and V3 showed an increase (P < 0.05), while T-wave amplitudes for leads I, II, aVL, aVF, V4, V5, and V6 exhibited a decrease (P < 0.05). (2) In comparison to the survival subgroup, the diameters of the LV, RV, LA, and RA in the death subgroup were enlarged, while the left ventricular ejection fraction and eft ventricular fractional shortening were decreased (P < 0.05). The Tp-Te /QT ratios for leads aVR, V5, and V6 also increased notably (P < 0.05 or P < 0.01). The T-wave amplitude readings from leads II, aVF, and V6 demonstrated significant reductions (P < 0.05).

Abnormal ventricular repolarisation parameters were found in dilated cardiomyopathy children. Increased Tp-Te /QT ratios in aVR, V5, and V6 leads and decreased T-wave amplitudes in II, aVF, and V6 leads were risk factors for predicting mortality in children with dilated cardiomyopathy.

The impact of CHD on safe driving for adolescents is currently unknown. A prospective, qualitative descriptive study was conducted among adolescents with CHD to describe perceived barriers, facilitators, and impacts of CHD on safe, independent driving among adolescents.

Twenty-eight adolescents aged 15–19 years with CHD participated in virtual, semi-structured interviews in 2023. Adolescent interview data were analysed with conventional content analysis refined by Theoretical Domains Framework in NVivo software.

Mean participant age was 16.4 ± 0.23 years (57% male). Single ventricle physiology (25%) and septal defects (32%) were prevalent diagnoses among the study population. Most participants (92%) did not have driving restrictions.

Driving as a normal rite of passage for adolescents with CHD; and confident—but curious—about the impacts of CHD on driving. Adolescents felt confident that driving is not impacted by CHD. They were curious about the likelihood of cardiovascular emergencies and related symptoms while driving. Perceived barriers and facilitators to safe, independent driving were like what has been described in published literature among adolescents without CHD.

These findings celebrate the normalcy of driving during adolescence and reveal curiosities about the impacts of stress, anxiety, fatigue, and risks of heart attack and stroke on driving. Adolescents may look to CHD healthcare providers to help them learn about driving. These findings may inform the development of tools to facilitate meaningful conversations with adolescents regarding driving safety as part of the transition to adult CHD care.

Ventricular tachycardia is a rare condition in the fetus and neonate with a paucity of data regarding the management and outcomes.

We reviewed the clinical histories, associated CHD and syndromes, diagnostic investigations, management strategies, and outcomes for all fetuses and neonates with ventricular tachycardia encountered between 2005 and 2020.

Five fetal and 8 neonatal cases of ventricular tachycardia were encountered. Two (40%) fetal cases, compared to 5 (62%) neonatal cases had CHD (p = 0.59), and only 1 fetus had cardiomyopathy with findings suggesting restriction before ventricular tachycardia onset. The median age at ventricular tachycardia presentation was 32 (26–37) weeks for fetal and 11 (1–27) days for neonatal cases. Of the fetal cases, 2 were initially treated trans-placentally with propranolol and 2 with amiodarone. Two fetuses (40%) had ventricular tachycardia suppression prenatally that recurred during the neonatal period necessitating propranolol therapy, 2 (40%) had resolution before birth with no postnatal recurrence, and the cardiomyopathy case never achieved full control, developed hydrops and demised. Of the neonatal cases, 4 received intravenous antiarrhythmics on admission: 3 amiodarone and 1 esmolol, and 2 of these were converted to propranolol and 2 to sotalol. Three other neonates initially received propranolol, with 1 discharged on propranolol, 1 on sotalol, and 1 on mexiletine after failed propranolol and sotalol treatment. The 8th neonate was discharged on no medication.

Most fetal and neonatal ventricular tachycardia is manageable with pharmacologic therapy. Given its rarity, larger studies are needed to identify optimal management strategies.

Due to the high postoperative mortality, tools for an adaequate risk stratification are important to identify high-risk patients undergoing the Norwood procedure. As a marker of ventricular wall stress, NT-proBNP might be of particular interest in these children.

This study evaluated whether NT-proBNP’s age-adjusted z-score (“zlog-NT-proBNP”) predicts outcomes after stage I Norwood procedure.

Patients who underwent the Norwood procedure between 1 January 2011 and 31 December 2022, with perioperative NT-proBNP measurements available were enrolled. Since reference intervals of NT-proBNP are highly age-dependent, age-adjusted zlog-NT-proBNP was used. Serial zlog-NT-proBNP values were analysed to predict the occurrence of major adverse cardiovascular events after the Norwood procedure. Major adverse cardiovascular events was defined as death, resuscitation, or mechanical circulatory support. Secondary endpoints were re-operation and re-intervention for shunt.

A total of 139 patients underwent the Norwood procedure and had at least one NT-proBNP measurement available. Preoperative zlog-NT-proBNP measurements (median 3.7, interquartile range 3.1–4.19) showed no association with the occurrence of major adverse cardiovascular events or mortality. Zlog-NT-proBNP early after ICU admission (3.2, interquartile range 2.4–3.8) was predictive of mortality but showed no association with the occurrence of major adverse cardiovascular events. Zlog-NT-proBNP before ICU discharge (3.2, interquartile range 2.8–3.8) was significantly associated with the occurrence of both major adverse cardiovascular events (hazard ratio 1.83, 95% confidence interval 1.25–2.67, P = 0.002) and death (hazard ratio 2.1, 95% CI 1.4–3.2, P < 0.001).

High zlog-NT-proBNP levels after the Norwood surgery were strongly associated with the occurrence of major adverse cardiovascular events and death. Therefore, zlog-NT-proBNP has the potential to identify high-risk patients before life-threatening complications occur.

Racial disparities in healthcare have been well documented in the United States. We hypothesise that there will be a racial variance in different clinical variables in single-ventricle patients through stages of palliation.

Retrospective single-centre study stratified all single-ventricle patients who reached stage 2 palliation by race: Black and White. Other races were excluded. Demographic and clinical characteristics were compared, alongside follow-up survival data. Primary outcomes were progression to Fontan and overall survival.

Among 526 patients, 325 (61.8%) were White, and 201 (38.2%) were Black. Median age at stage 2 palliation was 150 days for White and 165 for Black patients (p = 0.005), with similar weights. Black patients exhibited higher median cardiopulmonary bypass times (87 vs. 74 minutes, p = 0.001) and a greater frequency of genetic syndromes (30.1% vs. 22.1%, p = 0.044). No significant differences were observed in outcomes between groups from stage 2 to stage 3, pre-stage 3 cardiac catheterisation variables, or perioperative outcomes. Multivariable regression analysis identified hypoplastic pulmonary arteries as the risk factor for mortality after stage 2. Survival analysis showed no difference in survival by race; however, occurrence of combined cardiovascular event was significantly higher in Black race.

Significant racial disparities exist among single-ventricle patients regarding the timing of stage 2 palliation, cardiopulmonary bypass duration, and frequency of genetic syndromes. Black race was a risk factor for sub-optimal long-term outcome, although perioperative mortality was similar. These race-related factors warrant further studies to improve our understanding of the impact of race on outcomes.

In recent years, the importance of telemedicine has increased significantly. Especially in the field of echocardiography, virtual reality glasses offer the possibility of real-time data transmission without restrictions in the examination process. In particular, the care of critically ill newborns with suspected CHD might be improved by allowing a specialized paediatric cardiologist to remotely guide an echocardiographic examination. The current study aims to prove whether novices, under Google Glass guidance by a paediatric cardiologist, can perform an appropriate neonatal echocardiography.

The current study is a prospective monocentric single-blinded pilot study. Participants were supposed to perform two test runs: The first test run was “unguided” and the second test run was instructed via Google Glass. A validated training simulator for neonatal echocardiography “EchocomNeo, Echocom GmbH” was used. The study took place at the Leipzig Heart Center, Department of Pediatric Cardiology from April 2022 to November 2022.

A total of 21 medical students were enrolled. In total 252 views (126 views in each test run) were recorded. The overall performance was significantly higher in the Google Glass guided test run compared to “unguided” (structure score: 77.6% vs. 63.2%. p < 0.001 and quality score: 58.7% vs. 47.2%, p < 0.001). Also, the time was significantly lower in the Google Glass guided test run than in the unguided test run, p = 0.014.

Google Glass guidance by a paediatric cardiologist could optimize the performance of novices in echocardiography using a standardized neonatal echo-simulator with structural normal cardiac anatomy.

Children with CHD are at increased risk for neurodevelopmental disabilities and neuropsychological impairments throughout their life span. The purpose of this report is to share our experience building a sustainable, novel, inpatient, interdisciplinary Neurocardiac Critical Care Program to mitigate risks and optimize outcomes during the ICU stay.

A descriptive review was chosen to identify meaningful characteristics, challenges and lessons learned related to the establishment, expansion of and sustainability of Neurocardiac Critical Care Program in a 26-bed pediatric cardiac ICU.

We successfully launched, expanded, and sustained an interdisciplinary Neurocardiac Critical Care Program. Here, we share the foundation, framework, challenges, and lessons learned as we established and sustained the Neurocardiac Critical Care Program. The key elements of our program are (1) consistent engagement by pediatric neurologists in the cardiac ICU, (2) comprehensive education initiatives, (3) evidence-based clinical practice changes, and (4) quality improvement and research projects.

The development of a pediatric Neurocardiac Critical Care Program is feasible and sustainable. This program was informed by recent research related to perioperative and psychosocial risk factors that impact brain development and neurodevelopmental outcomes in this vulnerable population. By aligning our efforts, our multidisciplinary team is helping shift the paradigm in pediatric cardiac critical care to actively manage complex heart disease, while simultaneously and proactively mitigating risks to the developing brain and family unit.

Describe the hemodynamic implications of anaesthetic choice among children with heart disease undergoing cardiac catheterisation.

Study 1 was a secondary analysis of data obtained during catheterisation-based hemodynamic assessment of infants with hypoplastic left heart syndrome following Stage 1 palliation, randomised in the Single Ventricle Reconstruction trial. Measured and calculated hemodynamics including pulmonary and systemic vascular resistance indexed to body surface area (PVRi and SVRi respectively) and pulmonary/systemic blood flow (Qp/Qs) were analysed with respect to anaesthetic employed during catheterisation, classified as moderate sedation or general anaesthesia. Study 2 consisted of a single centre, prospective analysis of patients requiring percutaneous closure of a patent ductus arteriosus or endomyocardial biopsy after orthotopic heart transplant. Participants underwent hemodynamic assessment first using inhaled volatile anaesthesia (IA), and then transitioned to total intravenous anaesthesia, comparing hemodynamic measures with respect to anaesthetic approach.

In Study 1, independent of shunt type, PVRi, and patient size, moderate sedation was associated with a greater than two-fold odds of a Qp/Qs >1 (OR 2.12, 95%CI 1.18–3.87, p = 0.013). In Study 2, while PVRi was similar, SVRi was significantly higher using total intravenous anaesthesia. Among the patent ductus arteriosus subgroup, Qp/Qs increased significantly with a total intravenous anaesthesia relative to IA (p = 0.003); additionally, among the orthotopic heart transplant subgroup, left ventricular end diastolic pressure increased following a transition to total intravenous anaesthesia (p = 0.002).

Analyses of hemodynamics during catheterisation support a significant impact of anaesthetic type on hemodynamic values including SVRi, left ventricular end diastolic pressure, and Qp/Qs. Anaesthesia choice and intraprocedural management of SVRi are important considerations when making clinical decisions based on hemodynamic data.

Medical and surgical advancements have enabled a 95% survival rate for children with CHD. However, these survivors are disproportionately affected by neurodevelopmental disabilities. In particular, they have behavioural problems in toddlerhood. Because there is a known relationship between behavioural problems and early language delay, we hypothesise that children with critical CHD have early detectable language deficits. To test our hypothesis, we performed a retrospective study on a cohort of children with critical CHD to visualise their early language developmental trajectories.

We identified a cohort of 27 children with two diagnoses: single ventricle physiology (19) and transposition of the great arteries (8). As part of their routine clinical care, all of these children had serial developmental evaluations with the language subsection of the Capute Scales. We visualised their developmental language trajectories as a function of chronologic age, and we used a univariate linear regression model to calculate diagnosis-specific expected developmental age equivalents.

In each group, language development is age-appropriate in infancy. Deviation from age-appropriate development is apparent by 18 months. This results in borderline-mild language delay by 30 months.

Using the Capute Scales, our team quantified early language development in infants and toddlers with critical CHD. Our identification of deceleration in skill acquisition reinforces the call for ongoing neurodevelopmental surveillance in these children. Understanding early language development will help clinicians provide informed anticipatory guidance to families of children with critical CHD.

Children with single ventricle physiology and transposition of the great arteries have measurable early language delays.

Lipid levels in paediatric patients with anomalous aortic origin of a coronary artery (AAOCA) have not previously been explored. Patients with CHD have an increased risk of atherosclerotic cardiovascular disease later in life compared to the general population. We aim to characterise the lipid profiles in paediatric patients with AAOCA and explore its relation to diagnosis, race/ethnicity, and exercise.

Single institution retrospective cohort of 180 AAOCA paediatric patients (median age 13.7 years interquartile range 9.7–15.6, 66% male). Total cholesterol, HDL, LDL, triglycerides, total cholesterol to HDL ratio, and non-HDL cholesterol were evaluated across race/ethnicity, sex, type of AAOCA, documented ischaemia on imaging, exercise level, and surgery status. Normality of the data distribution for each lipid parameter was evaluated using Kolmogorov–Smirnov testing. Accordingly, Mann–Whitney U and t-tests were used to compare variables. The proportion of abnormal lipid levels by sex and race/ethnicity was calculated.

Total cholesterol was elevated in 29%, (51/177) of patients, HDL 37% (64/174), triglycerides 44% (72/165), LDL 16% (28/170), total cholesterol-HDL ratio 29%, (48/163), and non-HDL cholesterol 28% (47/165). Across subgroups categorised on the basis of surgery status, exercise level, AAOCA type, and sex, the mean and median levels for individual lipid parameters were normal. By race/ethnicity, Hispanic patients had significantly higher triglyceride (median 99, interquartile range 71–136.5, p = <0.001) and total cholesterol to HDL ratios (median 3.2, interquartile range 2.7–4.5, p = 0.014) versus non-Hispanic White and Black patients. Two-thirds of patients exercise recreationally.

Hispanic patients have significantly elevated triglycerides and total cholesterol to HDL ratios compared to others. Longitudinal follow-up evaluating differences in long-term lipid status in patients with AAOCA and risk for cardiovascular events is warranted.