Cardiology in the Young最新文献

There is a growing awareness that diversity, health equity, and inclusion play a significant role in improving patient outcomes and advancing knowledge. The Pediatric Heart Network launched an initiative to incorporate diversity, health equity, and inclusion into its 2021 Scholar Award Funding Opportunity Announcement. This manuscript describes the process of incorporating diversity, health equity, and inclusion into the Pediatric Heart Network Scholar Award and the lessons learned. Recommendations for future Pediatric Heart Network grant application cycles are made which could be replicated by other funding agencies.

We report a unique case of an adolescent patient with Fontan physiology presenting with unconjugated hyperbilirubinemia due to dehiscence of a tricuspid valve annuloplasty ring.

Objectives: We sought to examine the relative importance of surgical lesion complexity versus the presence of genetic/syndromic/extracardiac anomalies (GSAs) in determining survival, morbidity or need for reinterventions following repair for aortic arch hypoplasia.

Methods: A single-centre, retrospective cohort study of infants undergoing biventricular aortic arch repair via sternotomy from 2010 to 2021 was conducted. Survival analysis was performed using Kaplan-Meier methods, with additional Bayesian survival modelling for subgroups. Composite morbidity comprised respiratory, renal, neurologic, or sepsis-related complications.

Results: Of 83 included infants, n = 13/83 (15.7%) had complex repairs; 27/83 (32.5%) were GSA+. Operative mortality was significantly higher in GSA+ versus GSA- patients (18.5% vs. 1.8%; p = 0.01), though not for complex versus non-complex repairs. Overall 10-year Kaplan-Meier survival was 86.7%. Bayesian modelling suggested equivalent post-discharge attrition in non-complex/GSA+ and complex/GSA- patients, with the poorest outcomes in complex/GSA+ patients; non-complex/GSA- patients had 100% survival. GSA+ patients exhibited higher composite morbidity (44.4% vs. 7.1% in GSA- p < 0.001), with their mode of death seemingly related to a high incidence of respiratory and neurological morbidity, notably in Dandy-Walker syndrome. The 10-year freedom from arch reinterventions was 87.7%; neither complexity, GSA status, nor post-repair peak arch velocity predicted the need for arch reinterventions.

Conclusions: Whilst anatomic complexity may have been somewhat neutralised as a risk factor for operative mortality, in contrast to GSA+ status, there is further post-discharge attrition attributable to complexity or GSA+ status, with additive risk effects. Morbidity directly related to certain syndromes underlies some of this risk. Non-anatomic substrates represent a persistent limitation to outcomes of surgical aortic arch repair in infants.

The interesting study has limitations that put the results and their interpretation into perspective. m.3243A>G carriers should undergo prospective testing for multisystem disease to avoid missing subclinical multisystem involvement. m.3243A>G carriers with hypertrophic cardiomyopathy require long-term electrocardiogram recordings to determine whether implantable cardioverter defibrillator implantation is necessary or not. To assess the outcome of m.3243A>G carriers, knowledge of heteroplasmy rates and mtDNA copy numbers is required. It is tempting to assign pathogenicity when any pathogenic variant is seen with genotype-phenotype correlation. However, double hits are possible and if genetic information is to be used to screen or risk-stratify other family members, the standard of care would be to ensure that post-mortem genetic autopsy is performed for a panel of causative genes, and that an autopsy is done to exclude other causes of death, if possible.

Left ventricular diastolic dysfunction is associated with poor prognosis in patients with hypertrophic cardiomyopathy and CHD. We report the case of an infant concomitant with hypertrophic cardiomyopathy, an atrial septal defect, and left ventricular diastolic dysfunction, who was successfully managed with fenestrated closure of the atrial septal defect.

Background: The COVID pandemic has had deleterious effects on the mental health of the global population. Parents of children with CHD were particularly vulnerable to negative mental health outcomes such as depression, anxiety, and perceived stress. A better understanding of the CHD parent experiences, needs, and concerns while navigating the healthcare system during a pandemic is needed.

Methods: Online survey responses from 71 parents of young children with CHD representing families across the United States of America and Canada were analysed. Qualitative data were collected one year into the COVID pandemic. Thematic analysis was used to examine responses to the open-ended question "What would you like healthcare professionals (doctors, nurses) to know about your experience of being a parent with a child with CHD during the COVID-19 pandemic?."

Results: Two major themes with subthemes and an umbrella theme emerged from the parents' responses (1) Pandemic Parenting: The Emotional Toll of Hospital Visitation Restrictions, Dealing with Social Distancing, Feeling Isolated, Decision Making in Uncertainty, and Playing it Safe versus Returning to Normal and (2) Unmet Expectations of Care: Needing Information, Wanting Empathy, Requesting Respect, Questioning Care Quality, and the umbrella theme of: Our Lives were Turned Upside Down.

Conclusion: CHD parents describe a negative impact of healthcare-related challenges during the COVID pandemic. These findings may offer insight to how healthcare professionals can better support the mental health and care burden of CHD parents during future pandemics.

Aim: Risk stratification is recommended for patients with ventricular pre-excitation, particularly when sports eligibility is required. Few studies have examined the changes in the electrophysiological properties of the accessory pathway during growth. This study investigates the evolution of electrophysiological properties of the ventricular pre-excitation in young athletes referred for sports eligibility.

Methods: Between January 2011 and July 2022, 44 paediatric patients (32 males; mean age, 10 ± 2.42) with ventricular pre-excitation underwent an electrophysiological study, both at rest and during adrenergic stress at two different times (T0 and T1) within a minimal interval of 2 years. Transcatheter ablation was not performed between the two electrophysiological studies. Electrophysiological data were collected and compared.

Results: Electrophysiological study under basal conditions showed a significant decrease in the anterograde accessory pathway effective refractory period and 1:1 conduction over the accessory pathway from T0 to T1. The shortest pre-excited R-R interval during atrial fibrillation did not significantly change at the basal condition; however, it decreased during the stress test. Furthermore, six patients (13.6%) changed the risk profile of their accessory pathway: two "high-risk" patients at T0 became "low-risk" and four "low-risk" patients became "high-risk" at T1. Atrioventricular re-entry tachycardia inducibility did not differ significantly between the two electrophysiological studies.

Conclusions: This study highlights the importance of repeating electrophysiological study (transesophageal or intracardiac) in paediatric athletes with ventricular pre-excitation because significant and clinically relevant changes in the conduction and refractoriness of accessory pathway can occur. This could influence risk stratification for sports eligibility and the correct indication and timing for accessory pathway ablation.

Background: Giant coronary artery aneurysms and myocardial fibrosis after Kawasaki disease may lead to devastating cardiovascular outcomes. We characterised the vascular and myocardial outcomes in five selected Kawasaki disease patients with a history of giant coronary artery aneurysms that completely regressed.

Methods: Five patients were selected who had giant coronary artery aneurysm in early childhood that regressed when studied 12-33 years after Kawasaki disease onset. Coronary arteries were imaged by coronary CT angiography, and coronary artery calcium volume scores were determined. We used endocardial strain measurements from CT imaging to assess myocardial regional wall function. Calprotectin and galectin-3 (gal-3) as biomarkers of inflammation and myocardial fibrosis were measured by enzyme-linked immunosorbent assay.

Results: The five selected patients with regressed giant coronary artery aneurysms had calcium scores of zero, normal levels of calprotectin and gal-3, and normal appearance of the coronary arteries by coronary computed tomography angiography. CT strain demonstrated normal peak systolic and diastolic strain patterns in four of five patients. In one patient with a myocardial infarction at the time of Kawasaki disease diagnosis at the age of 10 months, CT strain showed altered global longitudinal strain, reduced segmental peak strain, and reduced diastolic relaxation patterns in multiple left ventricle segments.

Conclusions: These patients illustrate that regression of giant aneurysms after Kawasaki disease is possible with no detectable calcium, normal biomarkers of inflammation and fibrosis, and normal myocardial function. Individuals with regressed giant coronary artery aneurysm still require longitudinal surveillance to assess the durability of this favourable outcome.

Background: 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.

Methods: 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.

Results: 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.

Conclusion: 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.