Significant Congenital Heart Disease Research Articles

Abstract 4135928: Externally Validated Deep Learning Model for Patent Ductus Arteriosus Detection by Echocardiography in Preterm Infants

Introduction: Patent ductus arteriosus (PDA) is a common form of congenital heart disease in premature infants and a source of significant morbidity. Echocardiography is the mainstay modality for diagnosis and monitoring but is resource-intensive and requires expertise for interpretation. Aims: To develop and externally validate a deep learning model to identify PDA in preterm infants by echocardiography. Methods: We trained, validated, and tested a convolutional neural network to detect PDA in raw echocardiogram clips from infants (gestational age <37 weeks) with and without PDAs and no significant additional congenital heart disease at the Medical University of South Carolina (MUSC). For training and validation, we employed a 12-fold cross-validation procedure. Experienced cardiologists defined the ground truth labels. We assessed classification performance on an internal model naïve dataset using the area under the receiver operator curve (AUROC), sensitivity, specificity, positive predictive value, and negative predictive value. For external multicenter testing, we used studies from Children’s Hospital of Orange County (CHOC) and Boston Children’s Hospital (BCH), both of which utilize a different ultrasound system vendor. Results: Training and validation were completed using 1,145 color/color-compare clips (661 clips with PDA and 484 no PDA) from 66 patients. The internal test dataset consisted of 142 clips from 16 patients. The external CHOC and BCH cohorts consisted of 146 clips from 50 patients and 294 clips from 68 patients, respectively. Model performance was similar for internal and external testing (Table 1). Conclusions: Our deep learning model detected the presence of PDA with acceptable performance. This performance generalized well across multiple institutions and ultrasound vendors. This work shows promise for the future development of an automated tool for PDA diagnosis enabling the democratization of pediatric cardiology resources.

Invasive testing leads to significantly altered (surgical) management strategy in coronary anomalies with interarterial course: 3 year interim analysis of MuSCAT

Abstract Introduction Diagnostic work-up of patients with an anomalous aortic origin of a coronary artery (AAOCA) includes an anatomical and functional assessment. The approach to functional evaluation currently varies widely and mainly includes noninvasive assessment. Intracoronary hemodynamic assessment can potentially enhance risk assessment for myocardial ischemia and sudden cardiac death and guide management of AAOCA patients. Hence, this study aims to evaluate invasive detection of ischemia on top of the standard of care diagnostic work-up for interarterial or intraseptal AAOCA and the impact on patient management. We report the first clinical outcomes. Methods In this multicenter prospective cohort study on AAOCA, all consecutive patients of 16 years and older with AAOCA with a newly diagnosed interarterial or intraseptal course in whom diagnostic work-up according to the protocol of the MuSCAT trial was performed between January 2021 and January 2024, were included for analysis. Exclusion comprised incomplete diagnostic evaluation, hemodynamically significant concomitant congenital heart disease or obstructive coronary artery disease in the AAOCA effluence. The work-up included invasive ischemia testing using fractional flow reserve (FFR) and or instantaneous wave-free ratio (iFR)/resting full-cycle ratio (RFR) at baseline and during pharmacological stress using adenosine, adrenaline and dobutamine. Results Fifty-eight patients (50% female, median age at AAOCA diagnosis 51.5 years (IQR 44.0-59.0)) were included, Table 1. The right coronary artery was anomalous in 88%, 93% patients had an interarterial and 69% an intramural course. Non-invasive ischemia detection was positive in 12%, negative in 84% and inconclusive in 4%. Invasive hemodynamic assessment was positive for ischemia in 22%, negative in 74% and inconclusive in 3%. Class of recommendation for surgery based on ESC guidelines was I or IIa in 28% patients and IIb, III or unclassified in 72%. Invasive hemodynamic assessment changed the treatment decision in 24% of the patients; 50% with initially recommended surgical treatment and 14% with initially a conservative treatment adjudication, Figure 1. Median follow-up after initial diagnosis was 20 [IQR 10-28] months and in 56% of all patients who completed 6 months follow-up the initial symptoms alleviated. One (2%) patient underwent a percutaneous re-intervention of the operated AAOCA. Conclusions This national prospective study of invasive hemodynamic assessment in patients with AAOCA showed clinically significant impact on the adjustment of initial management strategy in 24% of the patients. No cardiac death of myocardial infarction in the AAOCA supply area occurred during follow-up. Analysis of the complete follow-up of the MuSCAT trial can further substantiate the role of additional invasive functional imaging in AAOCA.

Detection of congenital heart disease by neonatologist performed cardiac ultrasound in preterm infants

ObjectiveWe aimed to assess the frequency of de novo congenital heart disease (CHD) detection via neonatologist-performed cardiac ultrasounds (NPCU) in premature infants born at <30 weeks of gestation.Study designIn this cross-sectional study (2004–2023) clinicians completing NPCU flagged de novo suspected CHD. All flagged NPCUs were cross-checked with cardiologists to confirm CHD diagnosis.ResultsThere were 2088 out of 3739 infants (56%) with at least one NPCU; 294 (14%) with cardiology referral. CHD diagnosis was confirmed in 109 of the 2088 (5.2%) infants. All major and critical CHD on NPCU imaging were suspected during NPCU and had prompt referral to the cardiology department.ConclusionDe novo presentation of significant CHD continues to occur in the preterm population, emphasizing the need for recognizing CHD during NPCU. Optimizing NPCU training may benefit patients with early cardiology referral and review.

Cardiac murmurs in children.

Approximately 50% of children experience a cardiac murmur at some point in their lives; <1% of these murmurs are attributed to congenital heart disease (CHD). Cardiac murmur might be the first clinical sign of a significant CHD in children. Despite careful routine medical examinations at birth, approximately 50% of CHD cases could remain unrecognised. Cardiovascular symptoms and signs could be specific or non-specific in neonates and children with heart murmurs. Knowledge about red flags in history and physical examinations, and syndromic associations of common CHDs are important. Auscultatory skills to identify systolic, diastolic and continuous murmurs and heart sounds are essential. Differential diagnosis should be formulated based on the location of maximum intensity of murmurs. Younger infants and children with pathological murmurs and red-flag signs should be promptly referred to local paediatric cardiology services for further investigations. Significant skill and knowledge are required for the identification of critical murmurs and associated cardiovascular problems. This review provides a simplified comprehensive update on cardiac murmurs and associated conditions in neonates and children.

Cost-utility analysis of palivizumab for preventing respiratory syncytial virus in preterm neonates and infants in Colombia

AimPalivizumab has proven effective in reducing hospitalizations, preventing severe illness, improving health outcomes, and reducing healthcare costs for infants at risk of respiratory syncytial virus (RSV) infection. We aim to assess the value of palivizumab in preventing RSV infection in high-risk infants in Colombia, where RSV poses a significant threat, causing severe respiratory illness and hospitalizations.MethodsWe conducted a decision tree analysis to compare five doses of palivizumab with no palivizumab. The study considered three population groups: preterm neonates (≤ 35 weeks gestational age), infants with bronchopulmonary dysplasia (BPD), and infants with hemodynamically significant congenital heart disease (CHD). We obtained clinical efficacy data from IMpact-RSV and Cardiac Synagis trials, while we derived neonatal hospitalization risks from the SENTINEL-1 study. We based hospitalization and recurrent wheezing management costs on Colombian analyses and validated them by experts. We estimated incremental cost-effectiveness ratios and performed 1,000 Monte Carlo simulations for probabilistic sensitivity analyses.ResultsPalivizumab is a dominant strategy for preventing RSV infection in preterm neonates and infants with BPD and CHD. Its high efficacy (78% in preventing RSV in preterm infants), the substantial risk of illness and hospitalization, and the high costs associated with hospitalization, particularly in neonatal intensive care settings, support this finding. The scatter plots and willingness-to-pay curves align with these results.ConclusionPalivizumab is a cost-saving strategy in Colombia, effectively preventing RSV infection in preterm neonates and infants with BPD and CHD by reducing hospitalizations and lowering healthcare costs.

The mechanics of congenital heart disease: from a morphological trait to the functional echocardiographic evaluation.

Advances in pediatric cardiac surgery have resulted in a recent growing epidemic of children and young adults with congenital heart diseases (CHDs). In these patients, congenital defects themselves, surgical operations and remaining lesions may alter cardiac anatomy and impact the mechanical performance of both ventricles. Cardiac function significantly influences outcomes in CHDs, necessitating regular patient follow-up to detect clinical changes and relevant risk factors. Echocardiography remains the primary imaging method for CHDs, but clinicians must understand patients' unique anatomies as different CHDs exhibit distinct anatomical characteristics affecting cardiac mechanics. Additionally, the use of myocardial deformation imaging and 3D echocardiography has gained popularity for enhanced assessment of cardiac function and anatomy. This paper discusses the role of echocardiography in evaluating cardiac mechanics in most significant CHDs, particularly its ability to accommodate and interpret the inherent anatomical substrate in these conditions.

Prenatal detection rates for congenital heart disease using abnormal obstetrical screening ultrasound alone as indication for fetal echocardiography.

To determine the live born prenatal detection rate of significant congenital heart disease (CHD) in a large, integrated, multi-center community-based health system using a strategy of referral only of patients with significant cardiac abnormalities on obstetrical screening ultrasound for fetal echocardiography. Detection rates were assessed for screening in both radiology and maternal fetal medicine (MFM). The impact on fetal echocardiography utilization was also assessed. This was a retrospective cohort study using an electronic health record, outside claims databases and chart review to determine all live births between 2016 and 2020 with postnatally confirmed sCHD that were prenatally detectable and resulted in cardiac surgery, intervention, or death within 1year. There were 214,486 pregnancies resulting in live births. Prenatally detectable significant CHD was confirmed in 294 infants. Of those 183 were detected for an overall live-born detection rate of 62%. Detection rates in MFM were 75% and in radiology were 52%. The number of fetal echocardiograms needed to detect (NND) sCHD was 7. A focus on quality and standardization of obstetrical screening ultrasound with referral to fetal echocardiography for cardiac abnormalities alone achieves benchmark targets for live-born detection of significant CHD requiring fewer fetal echocardiograms.

Risk stratification by systemic manifestations secondary to hemodynamic disorders of patients with severe tricuspid regurgitation

BackgroundTricuspid regurgitation (TR) is a prevalent disease that triggers systemic pathological changes including cardiac, respiratory, hepatic and digestive, hematopoietic, renal and skin issues. The burden of extra-cardiac manifestations has not been well described in TR patients and the clinical impact is unknown.MethodsPatients with severe or more-than-severe TR during hospitalization, who did not have any previous cardiac procedures, hemodynamically significant congenital heart disease or concomitant severe aortic or mitral valve disease, were retrospectively analyzed. Pre-specified criteria and diagnosis of baseline characteristics were used to evaluate the presence of extra-cardiac manifestations secondary to TR after excluding comorbidities that may also lead to corresponding abnormalities. Extra-cardiac involvements encompass respiratory, hepatic and, digestive, renal, hematopoietic and dermatic system. Staging criteria are defined as no extra-cardiac system involvement in Stage 1, one in Stage 2, at least two extra-cardiac involvements in Stage 3 and any end-stage organ failure in Stage 4. A telephone follow-up was conducted to record the composite endpoint namely all-cause death or cardiac rehospitalization after the index hospitalization.ResultsA total of 258 patients were identified with a median age of 73 (interquartile range [IQR]: 62–83) years and 52.3% were female. Severe TR and more-than-severe TR patients accounted for 92.6% and 7.4% of the cohort. There were 20.5%, 27.5%, 37.6% and 14.3% of patients from Stage 1 to 4 respectively. The follow-up time was at a median of 251 (IQR: 183–324) days. TR Patients in Stage 3&4 were at an increased risk with borderline statistical significance to experience the composite endpoint compared to patients in Stage 1&2 (odds ratio [OR] 1.9, 95% confidence interval [CI] 1.0 to 3.7, P = 0.049).ConclusionsApproximately half of patients with at least severe TR presented with two or more extra-cardiac systemic manifestations, which may incur a 1.9-fold higher risk of all-cause death or cardiac rehospitalization than TR patients with one or less extra-cardiac involvement.

Social Determinants of Health Including Child Opportunity Index Leading to Gaps in Care for Patients With Significant Congenital Heart Disease.

Gaps in care (GIC) are common for patients with congenital heart disease (CHD) and can lead to worsening clinical status, unplanned hospitalization, and mortality. Understanding of how social determinants of health (SDOH) contribute to GIC in CHD is incomplete. We hypothesize that SDOH, including Child Opportunity Index (COI), are associated with GIC in patients with significant CHD. A total of 8554 patients followed at a regional specialty pediatric hospital with moderate to severe CHD seen in cardiology clinic between January 2013 and December 2015 were retrospectively reviewed. SDOH factors including race, ethnicity, language, and COI calculated based on home address and zip code were analyzed. GIC of >3.25 years were identified in 32% (2709) of patients. GIC were associated with ages 14 to 29 years (P<0.001), Black race or Hispanic ethnicity (P<0.001), living ≥150 miles from the hospital (P=0.017), public health insurance (P<0.001), a maternal education level of high school or less (P<0.001), and a low COI (P<0.001). Multivariable analysis showed that GIC were associated with age ≥14 years, Black race or Hispanic ethnicity, documenting <3 caregivers as contacts, mother's education level being high school or less, a very low/low COI, and insurance status (C statistic 0.66). One-third of patients followed in a regional referral center with significant CHD experienced a substantial GIC (>3.25 years). Several SDOH, including a low COI, were associated with GIC. Hospitals should adopt formal GIC improvement programs focusing on SDOH to improve continuity of care and ultimately overall outcomes for patients with CHD.

Hemodynamics During Development and Postnatal Life.

A well-developed heart is essential for embryonic survival. There are constant interactions between cardiac tissue motion and blood flow, which determine the heart shape itself. Hemodynamic forces are a powerful stimulus for cardiac growth and differentiation. Therefore, it is particularly interesting to investigate how the blood flows through the heart and how hemodynamics is linked to a particular species and its development, including human. The appropriate patterns and magnitude of hemodynamic stresses are necessary for the proper formation of cardiac structures, and hemodynamic perturbations have been found to cause malformations via identifiable mechanobiological molecular pathways. There are significant differences in cardiac hemodynamics among vertebrate species, which go hand in hand with the presence of specific anatomical structures. However, strong similarities during development suggest a common pattern for cardiac hemodynamics in human adults. In the human fetal heart, hemodynamic abnormalities during gestation are known to progress to congenital heart malformations by birth. In this chapter, we discuss the current state of the knowledge of the prenatal cardiac hemodynamics, as discovered through small and large animal models, as well as from clinical investigations, with parallels gathered from the poikilotherm vertebrates that emulate some hemodynamically significant human congenital heart diseases.

The Impact of Integration of a Genetic Clinic Into a Pediatric Cardiac Unit.

Previously published studies suggest that genetic or environmental causes can be observed in 20-30% of congenital heart disease (CHD) patients, which include aneuploidy, single gene defects, pathological copy number variations, and de novo autosomal dominant and recessive inheritance. Moreover, the genetic background of childhood cardiomyopathies (CMs) has not been elucidated well. The study highlights the value of genetic assessment in diagnosing and family counseling for CHD and pediatric CM patients referred to the genetic clinic in a pediatric cardiology department. The study involved patients less than 18 years of age attending the cardiogenetic clinic in the pediatric cardiology department between December 2010 and February 2019. The following patient categories who had genetic evaluation were included: CHD in the presence of a syndromic phenotype, patients with CHD having extracardiac congenital anomalies or delayed development, hypertrophic and dilated CM patients, patients with dilated aortic root and ascending aorta,significant CHD in siblings or first-degree relatives,suspected channelopathies; and interrupted aortic arch abnormalities. A total of 285 patients were evaluated in the cardiogenetic clinic. The mean age was 20.2 months, with a range of 0-168. Females and males constituted 153 (53.7%) and 132 (46.3%), respectively. The most commoncause of referral to the genetic clinic was the presence of CM (N=134 (46.3%)): hypertrophic CM in 24% and dilated CM in 20% of cases. Seventy-six patients (26.7%) had positive genetic results. The most common genetic abnormality was familial infantile hypertrophic CM-causing gene ELAC2 in 19 (23.5%) cases. It may be beneficial for any pediatric cardiology unit to provide an established genetic clinic. Using a genetic clinic will enhance understanding of CHD pathophysiology, family education, and genetic counseling. Agreement on a well-written protocol and the way forward to specify what congenital heart conditions require genetic investigation should be clarified.

Abstract 14599: A Pilot Rheumatic Heart Disease Screening Program Using Portable, Handheld Echocardiography in Rivas, Nicaragua

Introduction: Portable, handheld echocardiography offers several advantages for rheumatic heart disease (RHD) screening in low-resource settings. Analyses of focused protocols using handheld echocardiography have yielded acceptable sensitivity and specificity for diagnosis of RHD, including in those performed by non-expert sonographers. Previous studies estimate high burden of RHD in Nicaragua. We conducted a pilot community-based RHD screening program using handheld echocardiography in Rivas, Nicaragua using contemporary RHD diagnostic criteria. Methods: Non-expert sonographers were trained using the GE Vscan Extend TM device and a simplified version of the 2012 World Heart Federation criteria for diagnosis of RHD. Referral criteria for complete echocardiogram included the presence of a mitral regurgitant jet of &gt; 2.0 cm, aortic regurgitant jet &gt; 1.0 cm, or any morphologic abnormality of the mitral or aortic valve, as well as left ventricular dysfunction or significant congenital heart disease. Participants were recruited by convenience-based sampling in communities throughout Rivas, Nicaragua. Participants were notified at the point-of-care of need for complete echocardiogram. All studies were overread asynchronously by expert readers for diagnostic accuracy. Results: Between March 2022 and April 2023, 494 screening echocardiographic studies were performed and evaluated for diagnostic accuracy. The median age was 36.3 years (IQR 19.2, 56.2), 22% of participants were &lt;18 years of age, and 60% of participants were female. There were 17.6% of participants referred for complete echocardiogram at the point-of-care compared with 13.0% meeting referral criteria on expert review. Negative and positive predictive values for non-expert vs. expert assessment were 92.4% and 62.1%, respectively. Cohen’s kappa for inter-rater reliability was 0.338, suggesting fair agreement. Conclusions: This pilot program demonstrated the feasibility of a screening program for RHD in rural Nicaragua with non-expert sonographers. Lessons from this program are intended to inform future epidemiological studies to determine the population prevalence and longitudinal outcomes of RHD in this region.

Abstract 19083: AI-Enabled ECG Detection of Atrial and Ventricular Septal Defects in Pediatric Patients

Background: Atrial septal defects (ASD) and ventricular septal defects (VSD) are among the most common forms of congenital heart disease (CHD). Early detection and intervention for these defects are crucial to prevent complications. Given advancements in AI technology, the development of AI-enabled ECG models for detecting these defects is of significant interest. Aims: This study aimed to create AI models using pediatric ECG data for the detection of ASD, VSD, and a combined model detecting either ASD or VSD. Methods: We analyzed a dataset of 7,795 pediatric patients from Mayo Clinic who had undergone echocardiography between 2002 and 2022, with corresponding 10-second, 12-lead surface ECGs taken within 14 days of echocardiography. This dataset included 259 patients with ASDs and 139 patients with VSDs (single or multiple). The control group was comprised of 7,397 patients without significant or complex CHD. For cases and controls, we included patients with all forms of electrocardiographic abnormalities and those with valvular lesions who had less than moderate stenosis or regurgitation. The dataset was divided into training, validation, and testing subsets to develop convolutional neural network (CNN) models for ASD, VSD, and either ASD or VSD detection. Results: Our ASD and VSD detection models achieved test AUCs of 0.82 (95% CI 0.76, 0.88) and 0.80 (95% CI 0.70, 0.90), respectively. Meanwhile, a combined model that detected either ASD or VSD demonstrated a test AUC of 0.81 (95% CI, 0.76, 0.86), sensitivity of 0.73, specificity of 0.76, PPV of 0.14, and NPV of 0.98 (Figure 1). Conclusions: AI-enabled ECG models demonstrate promising performance in detecting ASD and VSD in pediatric patients. A combined model that detects either ASD or VSD also shows robust results. Future research should focus on multi-center validation of these models, refining their diagnostic capacities, and investigating their potential for early intervention in CHDs.

Neighborhood location and nutritional resources as a risk factor for congenital heart disease in the fetus.

Congenital heart disease (CHD) is the most common birth defect, influenced by maternal health, environmental conditions, and genetics. Maternal health and nutrition, particularly maternal diabetes, is a modifiable risk factor for development of CHD in the fetus. However, the importance of food access during pregnancy on the development of CHD remains unknown. The objective of this study was to investigate the association between maternal neighborhood characteristics, particularly food access, and occurrence of prenatally diagnosed CHD. A retrospective case series studied maternal-fetal dyads with prenatally diagnosed CHD between 2019 and 2021 in Washington, DC. Moran's I of maternal addresses evaluated geographic clustering of disease. Negative binomial regression assessed association between census tract demographics and population-adjusted CHD rate. A total of 307 dyads were analyzed. Global Moran's I showed significant CHD clustering (p-value = .004). However, degree of clustering was not clinically meaningful. After adjusting for neighborhood socioeconomic status, residing in food deserts was not a predictor for CHD. However, neighborhoods with a higher percentage of households receiving Supplemental Nutrition Assistance Program (SNAP) benefits were associated with higher rates of conotruncal heart defects (Incident Rate Ratio [IRR] = 1.04, CI = 1.01-1.08) and aggregate CHD (IRR = 1.03, CI = 1.01-1.05). Neighborhood location and food access were not associated with CHD. However, increased enrollment in SNAP was associated with higher rates of CHD. The association between CHD and SNAP benefits warrants further exploration. Understanding food access and maternal nutrition may illuminate disparities in the burden of CHD.

Unexpected death involving the spleen - an overview.

The spleen is located in the left upper quadrant of the abdominal cavity and has both immunological and haematological functions. A variety of quite different entities may be associated with sudden death due to splenic involvement. These range from simple traumatic disruption of the parenchyma of a normal spleen with marked intra-abdominal haemorrhage, to conditions such as malaria and infectious mononucleosis where splenomegaly predisposes to rupture. Haematological diseases such as malignancies and haemoglobinopathies may causes sudden death due to haemorrhage or red cell sequestration. Asplenia or polysplenia may be associated with significant congenital heart disease. Hyposplenia, both structural and functional, may also result in rapid demise from sometimes unusual bacterial infections. Vascular abnormalities causing death include entities such as splenic artery aneurysms. Thus, deaths from splenic pathology may be due to localised issues or may be part of more generalised disease. For this reason the autopsy in cases of splenic-associated deaths must be comprehensive and include special testing such as microbiological screening and/or haematologic/genetic evaluation.

Timing of the narrowest neoLVOT with trans-catheter mitral valve implantation, a cardiac computed tomography study

Abstract Funding Acknowledgements Type of funding sources: None. Background Trans-catheter mitral valve replacement (TMVR) procedures had emerged as an alternative solution for patients who have surgical high risk for mitral valve surgery. One of the main challenges faced during the percutaneous approach is the degree of left ventricular outflow tract (LVOT) narrowing after implanting the new percutaneous mitral prosthesis. The remaining LVOT area (NeoLVOT) can be estimated by a commercially available CT analysis software that allows the insertion of a virtual valve within the CT dataset in the native annulus and/or the degenerated bioproshesis and hence allows measurement of the NeoLVOT using the multi-planar reconstruction (MPR) mode. The systolic phase that shows the narrowest NeoLVOT is not fully understood, and hence there is a considerable variability between operators in choosing the best phase in systole at which they should insert the virtual valve within the mitral annulus in the CT dataset to measure the narrowest NeoLVOT. Purpose To detect the systolic phase that shows the narrowest NeoLVOT and hence standardize the way to use the software to decrease variability among operators. Methods A retrospective observational single center study, including 20 patients (age 62± 4 years, 70% men), who had undergone a clinically indicated cardiac computed tomography (CT). All these patients were in sinus rhythm, had no significant coronary artery disease bases on CT report, and had normal left ventricular systolic function with no regional wall motion abnormalities, we excluded patients who had previous cardiac surgeries, hypertrophic cardiomyopathy, significant pulmonary hypertension or congenital heart disease that may affect septal motion. CTs were performed using a 128 slice dual-source (Somatom Definition Flash, Siemens Healthineers, Erlangen, Germany) with a retrospective ECG-gated acquisition CT analysis was done by a single operator using the commercially available software (3mensio Structural Heart 10.3, Pie Medical Imaging, The Netherlands). Multiphase scans were used and mid-diastole (80%), early-systole (10%), mid-systole (20%), late-systole (30–40%) were identified and analysed. Measurements done were; mitral annulus (MA) antero-posterior (AP) diameter, inter-commissural (IC) diameter, annulus area (Area), annulus perimeter (Peri), aorto-mitral angel, and the NeoLVOT area. (Figure 1). Results All measurements were feasible in all phases for all patients. The neoLVOT area was significantly narrower at end-systole (224 ± 62mm²), compared to early-systole (299 ± 70mm²) &amp; mid-systole (261 ± 75 mm²), (p = 0.005). All other measurement did not show statistically significant changes despite taking certain trends. (Figure 2). Conclusions End-systole is the cardiac phase that shows the narrowest predicted Neo-LVOT area and hence it should be the standard phase to be used during CT analysis before TMVR to predict the narrowest NeoLVOT.

Positive mindset and exercise capacity in school-aged children and adolescents with congenital heart disease.

Patients with congenital heart disease (CHD) have variable degrees of peak oxygen consumption (VO2) that can be improved with supervised fitness training. The ability to exercise is affected by anatomy, hemodynamics, and motivation. Motivation is in part related to mindset, or personal attitudes and beliefs, and a more positive mindset around exercise has been associated with better outcomes. It is unknown whether variations in measured peak VO2 in patients with CHD are related to having a positive mindset. Patient's ages 8-17 years with CHD were administered quality of life and physical activity questionnaires at the time of their routine cardiopulmonary exercise test. Those with severe hemodynamic burden were excluded. Patients were grouped based on disease classification. Mindset was evaluated via validated questionnaires including a PROMIS Meaning and Purpose (MaP) survey and an Anxiety survey. Pearson correlation coefficients were calculated to estimate the magnitude of the association between percent predicted peak oxygen consumption (pppVO2) and questionnaire scores overall and within CHD subgroups. Eighty-five patients participated; median age was 14.7 years, 53% were female, 66% had complex CHD, 20% had simple CHD, and 14% had single ventricle heart disease. Mean MaP scores were significantly lower in all CHD groups compared to population norms (p < 0.001). As a group, MaP scores were positively associated with the amount of reported physical activity (p = 0.017). In patients with simple CHD, MaP scores were positively associated with pppVO2 (p = 0.015). The association was even stronger for MaP:Anxiety, with worse ratios associated with lower pppVO2 (p = 0.005). Patients with complex and single ventricle CHD did not show a similar association. Patients with CHD, regardless of severity, had lower meaning and purpose scores than the general population, and these scores were associated with amount of reported physical activity. In the simple CHD subset, having a more positive mindset was associated with higher peak VO2 and a more negative mindset with lower peak VO2. This relationship was not seen with more significant CHD. While underlying CHD diagnoses are not modifiable, mindset and peak VO2 are, and consideration should be given to measuring both as each may be a target for intervention.

Ventricular pre-excitation in young patients: long term follow-up and natural history in a contemporary cohort

Abstract Funding Acknowledgements Type of funding sources: None. Background Ventricular preexcitation (VPE) due to atrio-ventricular accessory pathways (AP) has been associated to a higher incidence of sudden cardiac death (1,2). The incidence of major arrhythmic events (MAE) may be higher in children (1,3), but most of the past studies recruited a high proportion of patients with congenital heart disease (CHD), treated with outdated pharmacological therapy (1). Transcatheter ablation (TCA) of the AP is a safe and effective therapy and it is nowadays the standard of care (1,2). However, it may be less effective and more dangerous in smaller and younger children (1,3). It is described that patients may spontaneously lose VPE during childhood (1), but the natural history of VPE is not well understood yet. Purpose To study the natural history of VPE. We focused especially on the likelihood of spontaneous resolution of VPE during growth, the possible associated factors, and the incidence of MAE during a long follow up in a contemporary paediatric cohort. Methods We retrospectively recruited patients diagnosed with VPE at age 0 to 12, referred from 1993 to 2021 to two tertiary care hospitals. Significant CHD and double AP were excluded. The primary outcome was the spontaneous resolution of VPE, defined by the loss of short PR and delta wave seen in at least two consecutive ECG and/or on 24-hour Holter monitoring. The follow up stopped when the patients reached 16 years of age, met the primary outcome or effective TCA was performed. Given the time-to-event dependency of the condition, the survival analysis of VPE considered the left truncation resulted from the recruitment of patients after the first days of life. Results We recruited 153 patients with a median age of diagnosis of 4.9 (0.2 – 8.4). The median follow-up time was 4.9 (1.6 – 8) years. During follow-up, 42 (28%) patients spontaneously loss VPE. The left truncated Kaplan-Meier survival curve of VPE is shown in Picture 1. It estimates that VPE would persist in 53% (40.1-70.2) and 33.8% (23.7-48.4) of patients at the age of 1 and 16, respectively. Symptoms and intermittent preexcitation were the only variables significantly associated with lower (OR 0.2) and higher probability (OR 3.45) of SR of VPE. The vast majority of patients with symptoms was treated with drug therapy before the age of 8, and TCA was safely delayed thereafter. None of the patients experienced a MAE during follow up. Conclusion According to our data, a large proportion of paediatric patients are likely to spontaneously lose VPE within the first year of life, and a spontaneous resolution may occur later during childhood in a non-negligible number of cases. The absence of symptoms and/or intermittent preexcitation are significant predictors of loss of VPE. The results of our study are of the utmost clinical relevance, since they support a wait-and-see strategy, especially in very young children with VPE.

Improvement in Palivizumab Administration Prior to Discharge for Hospitalized Infants with Hemodynamically Significant Congenital Heart Disease: A Quality Improvement Initiative.

In this quality improvement initiative, we aimed to increase provider adherence with palivizumab administration guidelines for hospitalized infants with hemodynamically significant congenital heart disease. We included 470 infants over four respiratory syncytial virus (RSV) seasons from 11/2017 to 03/2021 (baseline season: 11/2017-03/2018). Interventions included the following: education, including palivizumab in the sign-out template, identifying a pharmacy expert, and a text alert (seasons 1 and 2: 11/2018-03/2020) that was replaced by an electronic health record (EHR) best practice alert (BPA) in season 3 (11/2020-03/2021). The text alert and BPA prompted providers to add "Need for RSV immunoprophylaxis" to the EHR problem list. The outcome metric was the percentage of eligible patients administered palivizumab prior to discharge. The process metric was the percentage of eligible patients with "Need for RSV immunoprophylaxis" on the EHR problem list. The balancing metric was the percentage of palivizumab doses administered to ineligible patients. A statistical process control P-chart was used to analyze the outcome metric. The mean percentage of eligible patients who received palivizumab prior to hospital discharge increased significantly from 70.1% (82/117) to 90.0% (86/96) in season 1 and to 97.9% (140/143) in season 3. Palivizumab guideline adherence was as high or higher for those with "Need for RSV immunoprophylaxis" on the problem list than for those without it in most time periods. The percentage of inappropriate palivizumab doses decreased from 5.7% (n = 5) at baseline to 4.4% (n = 4) in season 1 and 0.0% (n = 0) in season 3. Through this initiative, we improved adherence with palivizumab administration guidelines for eligible infants prior to hospital discharge.

Respiratory Syncytial Virus is the Most Common Causative Agent of Viral Bronchiolitis in Young Children: An Updated Review.

Viral bronchiolitis is a common condition and a leading cause of hospitalization in young children. This article provides readers with an update on the evaluation, diagnosis, and treatment of viral bronchiolitis, primarily due to RSV. A PubMed search was conducted in December 2021 in Clinical Queries using the key terms "acute bronchiolitis" OR "respiratory syncytial virus infection". The search included clinical trials, randomized controlled trials, case control studies, cohort studies, meta-analyses, observational studies, clinical guidelines, case reports, case series, and reviews. The search was restricted to children and English literature. The information retrieved from the above search was used in the compilation of this article. Respiratory syncytial virus (RSV) is the most common viral bronchiolitis in young children. Other viruses such as human rhinovirus and coronavirus could be etiological agents. Diagnosis is based on clinical manifestation. Viral testing is useful only for cohort and quarantine purposes. Cochrane evidence-based reviews have been performed on most treatment modalities for RSV and viral bronchiolitis. Treatment for viral bronchiolitis is mainly symptomatic support. Beta-agonists are frequently used despite the lack of evidence that they reduce hospital admissions or length of stay. Nebulized racemic epinephrine, hypertonic saline and corticosteroids are generally not effective. Passive immunoprophylaxis with a monoclonal antibody against RSV, when given intramuscularly and monthly during winter, is effective in preventing severe RSV bronchiolitis in high-risk children who are born prematurely and in children under 2 years with chronic lung disease or hemodynamically significant congenital heart disease. Vaccines for RSV bronchiolitis are being developed. Children with viral bronchiolitis in early life are at increased risk of developing asthma later in childhood. Viral bronchiolitis is common. No current pharmacologic treatment or novel therapy has been proven to improve outcomes compared to supportive treatment. Viral bronchiolitis in early life predisposes asthma development later in childhood.