Significant Congenital Heart Disease Research Articles

PO-03-146 PREMATURE VENTRICULAR CONTRACTIONS IN CHILDREN AND YOUNG ADULTS: RISK FACTORS FOR LEFT VENTRICULAR DYSFUNCTION

Premature ventricular contractions (PVCs) are common in children and are often perceived as benign. However, frequent PVCs can lead to left ventricular (LV) dysfunction. Risk factors for PVC-mediated LV dysfunction are not well defined in children. Characterize the frequency of and risk factors for LV dysfunction in children with frequent PVCs. Retrospective cohort study from 2003-18 including patients aged 1-21 years with frequent PVCs (>0.5% on Holter monitoring), a 12-lead ECG with 1+ PVC, and an echocardiogram. Risk factors for LV dysfunction (LVEF <50%) at time of presentation and during follow up in the subset of patients with echocardiographic follow up were evaluated using logistic regression and Cox proportional hazards analysis, respectively. Exclusion criteria included significant congenital heart disease and known personal or family history of primary arrhythmic or cardiomyopathic disease. We identified 202 patients and four were excluded due to diagnoses made after presentation with PVCs: Andersen-Tawil syndrome (2 siblings), and cardiomyopathy (2), leaving 198 for analysis with a median age 12.3 years. LV dysfunction was present at time of presentation in 4%, developed in an additional 6% during follow up, and was mild in most (LVEF <40% in only 1). Risk factors for LV dysfunction included older age; higher PVC burden; longer PVC duration; couplets, triplets, NSVT, and polymorphic ventricular ectopy (table 1). A PVC burden threshold of ≥15% was 100% sensitive and 76% specific for LV dysfunction at time of presentation (fig. 1) while a PVC duration threshold of ≥151ms was 100% sensitive and 74% specific. No risk factors were identified for developing LV dysfunction during follow up. In children who never received PVC-directed therapy or had serial Holter monitors before therapy was started, PVC burden decreased from a median of 10.2 to 1.8% (p < 0.001) over a median follow up period of 3.2 years. PVC-mediated LV dysfunction is rare in children and is mild in nearly all cases. Younger patients with <15% PVCs, PVC duration <151ms, and without complex ventricular ectopy are at extremely low risk for LV dysfunction. The natural history shows a reduction in PVC burden over time in most, but not all, patients. A minority of children who come to attention due to frequent PVCs have a serious underlying cardiovascular diagnosis.Tabled 1Table 1No LV Dysfunction (n = 191)LV Dysfunction (n = 7)OR [95% CI]p-valueDemographicsMale sex108 (57)6 (86)4.6 [0.5-39.0]0.161Age (years)12.1 (7.8-15.6)17.6 (16.2-17.6)4.5 [1.3-16.1] per 5 year increase0.021CHD8 (4)1 (14)3.8 [0.4-35.5]0.240PresentationPVCs incidentally noted140 (73)6 (86)2.2 [0.3-18.6]0.474Initial Holter Results%PVCs8.6 [3.9-14.9]33.6 [18.5-36.8]1.6 [1.3-2.2] per 5% increase<0.001Sustained VT3 (2)1 (14)10.4 [0.9-115.7]0.056NSVT11 (6)4 (57)21.8 [4.3-109.8]<0.001*Couplets73 (38)7 (100)*0.001Triplets26 (14)4 (57)8.5 [1.8-40.0]0.007Polymorphic ectopy9 (5)2 (29)8.1 [1.4-47.5]0.021ECG PVC CharacteristicsInferior QRS axis156 (82)6 (86)1.3 [0.2-11.5]0.786LBBB morphology141 (74)5 (71)0.9 [0.2-4.7]0.888Outflow tract morphology118 (62)5 (71)1.5 [0.3-8.2]0.608PVC coupling interval (ms)477 (420-531)467 (399-497)0.9 [0.7-1.1] per 25 ms increase0.420PVC duration (ms)135 (119-151)160 (157-168)4.0 [1.5-10.9] per 25 ms increase0.007ETT results (n = 99)PVC suppression during test78 (85)4 (57)0.2 [0.1-1.2]0.080VT during test3 (3)1 (14)4.9 [0.4-55.0]0.194∗Odds ratio cannot be estimated as all patients with dsyfunction are in one group; p-value estimated from Fisher's exact test. CHD = congenital heart disease, VT = ventricular tachycardia, NSVT = non-sustained ventricular tachycardia, LBBB = left bundle branch block, ETT = exercise tolerance test Open table in a new tab

PO-04-124 ARRHYTHMIAS INCLUDING ATRIAL FIBRILLATION IN KLEEFSTRA SYNDROME: A POSSIBLE EPIGENETIC LINK

Kleefstra syndrome (KS), often diagnosed in early childhood, is a rare genetic disorder (EHMT1 gene: an important epigenetic modulator) that is characterized by hypotonia, developmental delay and distinct facial features. Although congenital heart disease (CHD) is frequently reported, there is a paucity of data on the prevalence of CHD subtypes and arrhythmias in KS; no known reports of Atrial Fibrillation (AF). To systematically assess the prevalence of CHD and arrhythmias in KS. Cross-sectional analysis of the two largest known KS registries: Radboudumc (50 patients, median/IQR age: 13.5/13 years) based on health record review at Radboud University Medical Center in Netherlands, and GenIDA (163 patients, median/IQR age: 10/12 years) based on surveys of patient families from around the world. Case series of 3 additional patients with KS and atrial tachyarrhythmias. Both registries independently report 40% (20 patients in Radboudumc and 65 in GenIDA) prevalence of a cardiovascular system abnormality and 2-8% prevalence of arrhythmias (4 in Radboudumc and 3 in GenIDA). Radboudumc registry included 14% patients with atrial septal defect (ASD), 12% with ventricular septal defect (VSD), 4% patients each with Tetralogy of Fallot and pulmonary valve stenosis, and none with left ventricular dysfunction. The 8% patients with arrhythmias in this registry did not report significant CHD and included one patient with AF, two with supraventricular tachycardias (SVTs), and one with ventricular tachycardia (VT). GenIDA registry reported 1% patients with cardiomyopathy, 10% with VSD, 13% with ASD, 16% with valvular disease, and 9% with vascular malformations. This registry also included one patient with AF and another with chronic ectopic atrial tachycardia with neither patient reporting CHD. A case series of 3 additional KS patients not included in these registries with atrial tachyarrhythmias reveals two patients with AF and one with atrial tachycardia, in the absence of CHD. One patient with symptomatic AF refractory to medications underwent successful pulmonary vein isolation (first known report) at age 23 without recurrence at 16 months. The largest database of KS patients compiled to-date reveals a high prevalence of CHD and early-onset atrial tachyarrhythmias including AF, even in the absence of structural heart disease. These findings suggest a role for regular electrocardiograms and ambulatory rhythm monitoring as well as a possible novel epigenetic mechanism for AF.

Evaluation of Foetal Cardiothoracic Ratio in Normal Pregnancy: Correlation with Gestational Age, Foetal Weight and Established Nomogram

Background: An important sign of congenital heart disease (CHD), myocardial dysfunction and foetal congestive cardiac failure is cardiomegaly, which is detected using the assessment of the cardiothoracic (CT) ratio.&#x0D; Objective: To evaluate the foetal CT ratio and its relationship with gestational age in the second half of pregnancy, and produce nomograms for the foetal CT ratio.&#x0D; Subjects and Methods: This descriptive, cross‐sectional study enrolled women with a normal pregnancy in their second and third trimesters at the two tertiary health facilities, one secondary facility and one radiodiagnostic facility in Bayelsa State, Nigeria. The study was conducted from April–December 2022. Pearson's correlation and logistic regression analyses were used to assess the relationship and correlation between the CT ratio, gestational age, and estimated foetal weight. The p&lt;0.05 at 95%CI level of significance was set.&#x0D; Results: The mean bi-parietal diameter estimated foetal weight and CT diameter ratio was 66.5 ± 18.3 mm, 1.24 ± 0.87 kg and 0.46 ± 0.02 cm respectively. The foetal CT ratio correlates very strongly with estimated gestational age (ɼ = 0.98; p – 0.001), bi-parietal diameter (ɼ = 0.96; p – 0.001), estimated foetal weight (ɼ = 0.92; p – 0.001), Maternal age (ɼ = 0.36; p – 0.001), maternal weight (ɼ = 0.19; p – 0.001) and maternal height (ɼ = 0.20; p – 0.001).&#x0D; Conclusion: We have demonstrated that there does exist a very strong correlation between the foetal cardiothoracic ratio and the foetal gestational age, biparietal diameter and foetal weight, which slightly, but steadily increased throughout pregnancy.

Disparate Clinical Characteristics and Prognosis of HFpEF versus HFrEF Phenotype of Diabetic Cardiomyopathy

Diabetic cardiomyopathy (DCM) is an ill-defined entity. This study aims to explore the clinical characteristics and prognosis of diabetic patients that disparately develop heart failure (HF) with preserved ejection fraction (HFpEF) other than HF with reduced ejection fraction (HFrEF). A total of 911 patients diagnosed with diabetes mellitus were identified in the ChiHFpEF cohort (NCT05278026). DCM was defined as diabetic patients diagnosed with HF, absent from flow obstructive coronary artery disease (CAD), uncontrolled refractory hypertension and hemodynamics significant heart valvular diseases, arrhythmia and congenital heart diseases. The primary endpoint was a composite of all-cause death and rehospitalization due to HF. As compared to DCM-HFrEF patients, DCM-HFpEF patients had a longer duration of diabetes, were older and more noticeable in hypertension and non-obstructive CAD. After a median follow-up of 45.5 months, survival analysis showed that DCM-HFpEF patients had a better composite endpoint. Cox regression implicated that non-obstructive CAD was a negative (HR 0.101, 95% CI 0.028-0.373, p = 0.001) predictor for the composite endpoint of DCM-HFrEF patients. Age was a positive predictor for the composite endpoint of DCM-HFpEF patients (HR 1.044, 95% CI 1.007-1.082, p = 0.018). DCM-HFpEF is a disparate entity from DCM-HFrEF. Additional phenomic studies are needed to explore the molecular mechanisms and develop targeted therapies.

The implications of airway anomalies on children with congenital heart disease and bronchiolitis.

Bronchiolitis is a common airway infection in young children. Hemodynamically significant congenital heart disease (CHD) predicts a more complicated course. However, the role of airway anomalies remains unknown. We retrospectively reviewed the records of patients under 2 years old, diagnosed with CHD, and admitted between January 2011 and December 2013, before the palivizumab era. Records of bronchiolitis admissions were also extracted. Patients were grouped according to CHD condition and airway anomalies. A total of 230 patients with CHD were enrolled. A total of 180 (78%) and 71 (31%) patients had hemodynamically significant CHD and airway anomalies, respectively. A total of 52 (22.6%) patients were admitted for bronchiolitis 78 times. Among them, 33 (63.5%) had hemodynamically significant CHD, and 28 (53.8%) had airway anomalies. In patients with bronchiolitis admissions, the mean ventilator use, intensive care unit stay, and hospital stay were 1.08, 4.08, and 15.19 days, respectively. When compared, the mean hospital stay for bronchiolitis patients with airway anomalies was significantly longer than that of those without airway anomalies (19.8 vs. 9.9 days, p = 0.008). When further divided the patients by the presence hemodynamic significance, patients with hemodynamically significant CHD and airway anomaly had longer hospital stay than those who had neither. (21.7 vs. 8.3 days, p = 0.004) Airway anomaly was a significant risk factor for longer hospital stay in linear regression model (p = 0.007). Airway anomalies are common in children with CHD and are associated with longer hospital stays on bronchiolitis admission. An active survey for airway anomalies and adequate prophylaxis for bronchiolitis infection might be important in the care of children with CHD associated with airway anomalies.

Prophylaxis protects infants with congenital heart disease from severe forms of RSV infection: an Italian observational retrospective study

BackgroundIn children with congenital heart disease (CHD) respiratory syncytial virus (RSV) infection may have a severe course, with increased risk of morbidity and mortality, requiring hospital admission and intensive care. The aim of the present study was to evaluate the effect of prophylaxis with palivizumab in preventing RSV-associated hospitalization in infants with CHD.MethodsWe carried out an observational, retrospective study in a paediatric cardiology division at a secondary-care centre in Italy, extracting from the database children with CHD who, from November 2004 to March 2022, matched the criteria for palivizumab prophylaxis, to evaluate the hospitalization rate in CHD patients with and without palivizumab prophylaxis and their RSV-related hospitalization characteristics compared with a group of children without CHD and no other underlying clinical conditions (control group, CG), hospitalized for RSV infection.ResultsOne hundred twenty-eight children with CHD were enrolled in the study, mainly (71.9%) with increased pulmonary flow, and received palivizumab prophylaxis. Twenty-seven received hospital care for bronchiolitis. Almost all CHD patients hospitalized for bronchiolitis (26 out of 27) received partial prophylaxis (≤ 3 doses). CHD patients with bronchiolitis stay longer in the hospital than control (14.4 ± 21.7 days vs 6.2 ± 2.3 days) some of which require intensive care (n = 4).ConclusionsOur study provides evidence of the efficacy of palivizumab in protecting patients with hemodynamically significant CHD under the age of 2 years from RSV disease and its life-threatening complications. Reducing hospitalisation rate, morbidity, and mortality in this category of patients, passive immune prophylaxis with palivizumab may impact healthcare resource availability and utilisation.

Comparison of Echocardiography and Computed Tomography Angiography in the Evaluation of Pulmonary Arteries and Pulmonary Annulus in Congenital Heart Diseases with Right Ventricular Outflow Tract Obstruction

Abstract Background: Congenital heart diseases (CHDs) are the most commonly occurring birth defects. Clinically significant CHD necessitates percutaneous or operative intervention early in life. Accurate knowledge of the morphology and caliber of the pulmonary arteries and pulmonary valve is important in surgical planning, postoperative complications, and long-term outcome of various CHDs. This study aimed to compare echocardiography and computed tomography angiography (CTA) in the evaluation of the pulmonary arteries and pulmonary annulus in children with cyanotic CHD with right ventricular outflow obstruction. Materials and Methods: Our study was a cross-sectional study involving 39 children under the age of 2 years with diagnosed cyanotic CHD. Spectrum included were tetralogy of Fallot (TOF), TOF with pulmonary atresia, double-outlet right ventricle-TOF type, pulmonary atresia, and pulmonary stenosis. All patients underwent transthoracic echocardiography, followed by CTA. Images were reconstructed and analyzed, and the results were compared with echocardiography. Results: There was a good correlation between echocardiography and CTA in the evaluation of the caliber of pulmonary arteries and pulmonary valve, with a Pearson’s correlation coefficient of 0.85, 0.69, 0.62, and 0.73 for the main pulmonary artery (MPA), right pulmonary artery (RPA), left pulmonary artery (LPA), and pulmonary valve, respectively. There was an underestimation of the caliber of the main, right, and left pulmonary arteries with echocardiography (with a bias of 0.73 ± 1.96 mm, 0.01 ± 2.3 mm, and 0.17 ± 1.3 mm for the MPA, RPA, and LPA, respectively) and overestimation of the caliber of the pulmonary annulus (with a bias of −0.07 ± 2.20 mm). Conclusions: Despite a good correlation and agreement in the measurement of vessel diameters, echocardiography may misdiagnose certain cases of valvular or arterial stenosis as atresia, or may fail to visualize the structures entirely. In such patients, further evaluation with CTA may be required.

The high-density grid catheter: A safe adjunctive tool in pediatric and complex congenital heart disease patients.

The safety and utility of the Advisor™ High-Density Grid mapping catheter (HDGC) in children and congenital heart disease (CHD) patients are not well described. A single-center retrospective cohort study of pediatric and CHD patients undergoing electrophysiology study and ablation to determine the effect of HDGC use on outcomes including complications, fluoroscopy use, procedure duration, acute ablation success, and arrhythmia recurrence. HDGC was used in 74/261 (28.3%) cases. HDGC subjects differed by median age (17 vs. 13 years; p < 0.001),weight (68 vs. 50 kg; p < 0.001), and prevalence of significant CHD (42% vs. 3%; p < 0.001). Arrhythmia substrates were dissimilar: HGDC cases had higher frequencies of intra-atrial re-entrant tachycardia (25.7% vs. 0.5%), atrial flutter (8.1% vs. 1.1%), ectopic atrial tachycardia (13.5% vs. 3.7%), and premature ventricular contractions (9.5% vs. 0.5%), and lower incidences of atrioventricular re-entrant tachycardia (16.2% vs. 46.1%). Complications were rare (n = 5, 1.9%) with no significant difference between groups (p = 1.00). Procedure duration-but not fluoroscopy exposure-was significantly longer in HDGC cases (median 256 vs. 216 min, p < 0.001). Acute success was lower (93.2% vs. 99.4%; p = 0.01) and recurrences higher (13.2% vs. 3.8%; p = 0.016) in HDGC compared to non-HDGC cases. HDGC use in children and CHD patients is safe and not associated with higher complication rates. The lower acute success and higher recurrence rates in HDGC cases likely reflect a bias toward HDGC use in more complex arrhythmia substrates rather than less favorable ablation outcomes.

Evaluation of neonatal presurgical echocardiographic requests for noncardiac surgery at the University of Uyo Teaching Hospital, Nigeria

Abstract Context: There is a well-established association of congenital heart disease (CHD) with surgically correctable malformations particularly those of the gastrointestinal tract, genitourinary and central nervous systems. These malformations are typically corrected during the newborn period. Clinically significant and severe CHDs may worsen surgical outcomes. Aims: This study aimed to determine the prevalence of CHD diagnosed by echocardiography in neonates having noncardiac surgery at the University of Uyo Teaching Hospital. The study also aimed to assess if echocardiographic findings correlated with the severity of clinical presentation. Settings and Design: This was a retrospective review of the clinical and echocardiographic records of neonates who underwent noncardiac surgery between October 2020 and March 2023 at the University of Uyo Teaching Hospital. Subjects and Methods: Echocardiographic studies were done on all participants on a GE Vivid Iq® machine using standard views. M-mode and two-dimensional data were recorded. Statistical Analysis: Data were analyzed using the Statistical package for socials sciences (SPSS) version 25. Results: Thirty patients had presurgical echocardiography done in the period accounting for 41% of all neonatal echocardiography requests. Twenty percent of the patients had a CHD identified. Atrial Septal Defects were the commonest CHD identified in 50% of patients. The association between clinical symptoms, gender, surgical diagnosis and the presence of CHDs were not statistically significant. Conclusions: Neonatal surgical anomalies are commonly associated with CHDs. The nature of most of the CHDs however appears to be mild. A preoperative assessment by a pediatric cardiologist is recommended to avoid undue delays.

Surgical correction of congenital heart disease (atrial septal defect) in HIV patient

Presented clinical case demonstrates a successful surgical treatment results of long-term existing hemodynamically significant congenital heart disease (secondary atrial septal defect and pulmonary stenosis with secondary tricuspid regurgitation) in a patient with HIV infection after correction the immunodeficiency status and reducing the viral load.

Fifteen-minute consultation: How to spot serious heart disease in the newborn

Congenital heart disease (CHD) is common and important as it remains a leading cause of neonatal morbidity and appreciable mortality. Prenatal diagnosis, the presence of a murmur, cyanosis, tachypnoea and/or...

Impact of repaired aortic coarctation in left ventricular myocardial work

AimsMyocardial strain analysis enables more precise assessment of cardiac performance but is relatively load dependent. New tools have been developed with afterload adjustment. Our objective was to assess myocardial work (MW) in patients with repaired aortic coarctation (rACo). MethodsProspective study of consecutive patients with rACo who underwent a routine transthoracic echocardiogram in 2018 and 2019 at our center. Patients with significant aortic valve disease, pacemaker, or other congenital heart diseases (except for mild bicuspid aortic valve disease) were excluded. Global longitudinal strain with two dimensional speckle tracking analysis and MW were obtained (GWI:Global Work Index; GCW: Global Constructive Work; GWW: Global Wasted Work; GWE: Global Work Efficiency). Blood pressure was measured in the patient's right arm. ResultsWe included 42 patients in the analysis, mean age of 37±10 years, 38% males. In this group, 52% had hypertension and 64% had a concomitant bicuspid aortic valve. In comparison to previously published reference values, patients with rACo had significantly lower GWI (1807 vs. 1896 mmHg%) and GCW (2173 vs. 2232 mmHg%) (p<0.001), particularly in males. Systolic blood pressure is an independent predictor for GWI (β=0.432) and for GCW (β=0.534) and GLS an independent predictor of all MW parameters (β>0.594). Neither age nor gender were independent predictors. ConclusionsIn patients with rACo, there are some signs of left ventricular dysfunction with a reduction in GCW and GWI and with preserved GWE, despite normal ejection fraction and strain.

Long QT syndrome: Identification of a novel de novo mutation of calmodulin in a newborn girl

Background: Long QT syndrome (LQTS) is a genetically and phenotypically heterogeneous disorder of ventricular myocardial repolarization that is typically characterized by prolongation of the heart rate-corrected QT interval on a 12-lead electrocardiogram (ECG). LQTS has a complicated etiology since great genotypic and phenotypic overlapping among LQTSs limits the establishment of a diagnosis based solely on clinical features. Case presentation: Here, we describe a Vietnamese newborn with a novel calmodulin (CALM2) variant. The patient was referred to the Department of Cardiology at Children's Hospital 2 in Ho Chi Minh City, Vietnam for multiple clinical cardiac presentations. The patient's family history was negative for cardiovascular diseases; however, she suffered from recurrent syncope and QT interval prolongation on resting in a 12-lead surface ECG. A fetal echocardiogram at 29 weeks detected signs of congenital heart disease. The newborn patient then suffered from sudden cardiac arrest and required cardiopulmonary resuscitation. Two weeks later, the patient suffered from torsade de pointes for the third time and required electrical shocks. An endotracheal tube was placed through the mouth into the trachea to facilitate breathing. Unfortunately, the patient expired at 3 months of age due to serious inflammatory issues. Whole exome sequencing (WES) — a technique used to determine variations in all coding regions (exons) of the known genes — validated a total of four rare variants in four cardiomyopathy (CM) genes, the CALM2, DSP, MYBPC3, and SPEG genes. We identified a novel de novo heterozygous missense mutation, c.280G &gt; T (p.Asp94Tyr), in the CALM2 gene (NM_001305626) encoding calmodulin, which is a ubiquitous calcium-binding protein that is important for a myriad of intracellular signaling events of cardiac function. Conclusion: The results obtained in this study support the ``pan-cardiomyopathy panel'' approach, in which the molecular diagnosis of LQTS, the early identification of arrhythmia development, and the improved clinical management of cardiovascular disease patients are applied. Trial registration: The procedures were reviewed and approved by the Ethical Committee of the University of Medicine and Pharmacy, Ho Chi Minh City, Vietnam, under the number UMP-VN 2018-10a12.

Reappraisal of the Subtropical Guidelines on Palivizumab Prophylaxis in Congenital Heart Disease.

Objective: To define the impact of associated abnormalities on the efficacy of the novel subtropical guidelines for palivizumab prophylaxis on respiratory syncytial virus (RSV)-related hospitalizations in patients with hemodynamically significant congenital heart disease (hsCHD).Method: This prospective study enrolled every patient seen at a tertiary care center for hsCHD, who was born between 2014 and 2018 and received at least 1 dose of palivizumab, according to the subtropical guidelines. The patients were followed until the age of 2 years.Results: A total of 772 patients (49% male) were enrolled. Cyanotic CHD was seen in 46% of patients, of whom 23% had associated abnormalities. Lung/airway abnormalities (14%) were the most common followed by the genetic syndromes associated with CHD (7.3%). Among the 772 patients, RSV-related hospitalizations occurred in 3.2 and 2.2% children aged ≤ 12 and 13–24 months, respectively. Most of the RSV infections occurred in patients no longer satisfying the criteria for palivizumab prophylaxis. The patients with associated abnormalities but not the type of CHD, patient age, and patient sex were risk factors for RSV-related hospitalizations. The rates of RSV-related hospitalizations, admission to the intensive care unit, and endotracheal intubation were higher for patients with associated anomalies than for other patients before 24 months of age (10.2 vs. 4.0%, 67 vs. 33%, and 39 vs. 4.2%, p = 0.004, 0.06, 0.013, respectively).Conclusion: Children with abnormalities, especially genetic syndromes and lung/airway problems associated with CHD, are at high risk for RSV-related hospitalization. Our current subtropical guidelines for palivizumab prophylaxis in patients with hsCHD, should be revised to include the results of this study.

Effectiveness of palivizumab immunoprophylaxis in infants with respiratory syncytial virus disease in Colombia.

Respiratory syncytial virus (RSV) is one of the most important childhood infections. To evaluate the effectiveness and safety of palivizumab immunoprophylaxis in preterm infants at a high risk of severe respiratory syncytial virus infection during the RSV season in Colombia. A prospective observational non-comparative multicenter study in six Colombian cities. At the beginning of the RSV infection season, palivizumab prophylaxis, up to five doses, was administered to infants born at ≤32 weeks of gestation, infants younger than six months, infants under one year of age with bronchopulmonary dysplasia (BPD), infants one year or less of age with hemodynamically significant acyanotic and non-acyanotic congenital heart disease (CHD), and with follow-up during the immunoprophylaxis until one month after the last dose. The study enrolled 600 patients, 91.8% of which were born at ≤ 32 weeks of gestation. BPD was observed in 54.9% of infants. 49% were born at < 32 weeks gestation and presented BPD. 6.9% had hemodynamically significant acyanotic and non-acyanotic CHD 53.3% received three or more doses of palivizumab. The mean interval between doses was 39.6 days. 1.8% of patients were hospitalized due to a confirmed RSV infection. Overall mortality was 1.2%, whereas the mortality by RSV in infants undergoing prophylaxis was 0.2%. Palivizumab was a clinically effective, well-tolerated treatment in the Colombian population. The safety profile of palivizumab reflects the findings from previous studies in developed countries.

Prenatal diagnosis of significant congenital heart disease and elective termination of pregnancy in Nevada

Objective We investigated the relationship between prenatal detection of significant congenital heart disease and elective termination of pregnancy over time in Nevada. Methods We identified those prenatally or post-natally diagnosed with significant congenital cardiovascular malformations in Nevada with birth dates or estimated delivery dates between July 2012 and June 2021. Results We identified 1246. Of 1246, 69 underwent fetal demise, 42 had elective termination, and 1135 were live-born. Of the 1135 live-born, 1090 had prenatal care, of which 718 (66%) overall had a prenatal diagnosis of significant congenital heart disease. However, prenatal detection statistically significantly increased over time from 45 to 82%, p = .00001. Termination of pregnancy averaged 10% of those identified within the legal timeframe, and the rate did not statistically significantly increase with increasing prenatal detection rates, p = .56. Of the 42 undergoing elective termination, 23 (55%) had syndromes or comorbidities vs. 280 (25%) of the 1135 live-births, p = .0003. Conclusions In Nevada, despite a statistically significant increase in prenatal detection of significant congenital heart disease over time, termination of pregnancy rates did not increase. Nevertheless, those undergoing elective termination were more likely to have associated syndromes or comorbidities.

Evaluation of the frequency and types of structural heart disease in fetuses of pregnant women according to risk groups by fetal echocardiography

Aim: The aim of this study is to evaluate the frequency of congenital heart diseases (CHD) encountered in low-risk and high-risk pregnant women by fetal echocardiographic (FE) examination. Material and Method: The records of 855 pregnant women with a gestational week greater than 16 who applied to the pediatric cardiology outpatient clinic of our hospital between July 2019- October 2021 and underwent FE were analyzed retrospectively. Results: CHD was detected in 109 (12.7%) of 855 pregnant women who were referred to our center and underwent FE examination. Frequency of CHD was given according to risk groups. The rate of CHD in patients in the high-risk group was 15.2%, while it was 9% in patients in the low-risk group (p=0.008). Significant CHD was 6.2% in the high-risk group versus 2.7% in the low-risk group (p=0.016). The most common structural cardiac anomaly in FE examination was ventricular septal defect (38.5% in 42 fetuses), and the second most common cardiac anomaly was atriyoventricular septal defect (18.3% in 20 fetuses). Conclusion: In our study, it was found a higher rate of CHD in pregnant women in the high-risk group than in the low-risk group and especially significant CHD was detected more common in pregnant women in the high-risk group.

Abstract 11504: Socioeconomic Factors Including Neighborhood SES Leading to Gaps in Care for Patients with Significant Congenital Heart Disease

Introduction: Lifelong continuity of care is crucial for patients with significant congenital heart disease (CHD). Unfortunately, gaps in care (GIC) are common and may be attributed to vulnerable developmental periods, changes in insurance status, lack of symptoms or knowledge regarding follow-up, and inability to access specialty care. There is limited understanding of socioeconomic and demographic factors that contribute to GIC in CHD. Hypothesis: Socioeconomic factors including U.S. neighborhood socioeconomic score(SES) are associated with GIC in patients with significant CHD. Methods and Results: A total of 8674 patients followed at Boston Children's Hospital (BCH) with moderate to severe CHD(as defined by AHA/ACC 2008 guidelines) were seen in clinic between January 2013 and December 2015. Socioeconomic characteristics including language, race, ethnicity, and U.S. neighborhood socioeconomic score (SES) were analyzed. GIC of &gt; 3.25-years were identified in 33% (2829) of patients. GIC were associated with being an adolescent or young adult (13-23 years) (P&lt;0.001), non-English speaking (P&lt;0.002), non-white (P&lt;0.001), Hispanic ethnicity (P = 0.02), and a low SES (P &lt;0.001). Conclusions: A third of patients with significant CHD experienced a substantial GIC (&gt;3.25 years). Adolescent young adult age and socioeconomic factors, including race, ethnicity, language, and a low SES were associated with GIC in patients with significant CHD. Interventions to identify and support these vulnerable populations could improve patient continuity of care and overall outcomes. Further studies are needed to understand the reasons for such GIC in vulnerable populations.

Risk factors for hospitalisation due to respiratory syncytial virus infection in children receiving prophylactic palivizumab.

Respiratory syncytial virus (RSV) is a common pathogen that causes extremely severe respiratory symptoms in the first few weeks and months of life. In infants with cardiopulmonary diseases, RSV infections have a significant clinical impact. Palivizumab, a humanised monoclonal antibody for RSV, has been shown to significantly reduce the rate of hospitalisation of high-risk infants diagnosed with RSV. However, we have experienced a significant number of RSV infections in our institution that required hospitalisation or intensive care, despite the administration of palivizumab. This study aimed to analyse the risk factors associated with severe RSV despite the use of palivizumab. We retrospectively reviewed the medical records of 688 patients who visited or were admitted to our hospital and received palivizumab. Thirty-seven (5.4%) patients required hospitalisation for RSV, despite receiving palivizumab. In addition, 31 of these patients (83.8%) required hospitalisation out of season for palivizumab injection. Preterm birth (≤ 28-week gestation), bronchopulmonary dysplasia (BPD), and trisomy 21 were risk factors for RSV-related hospitalisation in infected patients, despite receiving palivizumab. Furthermore, subgroup analysis of 69 patients with RSV revealed that hemodynamically significant congenital heart disease (CHD) was also a risk factor for RSV-related hospitalisation.Conclusion: Preterm birth (≤ 28weeks of gestation), BPD, trisomy 21, hemodynamically significant CHD, and CHD requiring surgery or cardiac catheterisation/intervention during infancy could be considered when determining whether year-round administration of palivizumab is appropriate. What is Known: • Respiratory syncytial virus causes severe respiratory symptoms in infants, particularly those with cardiopulmonary diseases. • The use of palivizumab has reduced the rate of hospitalisation of infants diagnosed with RSV. Despite this, the rate of hospitalisation is still high. What is New: •We identified that preterm birth (≤ 28-week gestation), bronchopulmonary dysplasia, trisomy 21, andhemodynamically significant congenital heart disease were risk factors for RSV-related hospitalisation, even afterreceiving palivizumab treatment. •High-risk infants should be closely monitored and the prolonged use of palivizumab should be considered.

When children with congenital heart disease become adults – Understanding psychosocial attainments and functioning in comparison to census population norms

BackgroundAdults outnumber children living with congenital heart disease (CHD), but research on outcomes for this population in terms of sociodemographic attainments such as education, employment and relationship status is only starting to emerge. ObjectivesWe examined such outcomes in a UK clinic sample. Determinants of outcome were examined in relation to disease severity and psychological functioning. MethodsUsing a survey with clustered clinic sampling, we evaluated outcomes for a final sample of 116 adults with significant congenital heart disease (ACHD) on common measures of sociodemographic attainment. Outcomes were compared to national population data. ResultsFindings suggested that ACHD compared relatively well with respect to educational attainment and employment. However, they were less likely to be living independently and have offspring than peers and more likely to be single and earning below the average wage. Most outcomes did not vary significantly by disease severity (with the exception of having offspring). Rather, psychological factors – mood, resilience, executive functioning and self-perceptions of health predicted attainment outcomes in various domains. ConclusionsAlthough some outcomes are comparable to peer referenced norms, ACHD deviated from population norms with respect to interpersonal functioning, having offspring and employment earnings. Findings suggested that psychological and neuropsychological factors were more important than disease severity in predicting outcomes. Clinical implications are discussed.