History Of Congenital Heart Disease Research Articles

A Nationwide Analysis of the Impact of Cardiopulmonary Anomalies on Cleft Palate Surgical Outcomes.

To increase awareness and improve perioperative care of patients with cleft palate (CP) and coexisting cardiopulmonary anomalies. Retrospective cohort. Multi-center. Patients who underwent surgical repair of CP between 2012-2020 identified in the American College of Surgeons National Surgical Quality Improvement Program Pediatric Data File. Chi-squared analysis and Student's t-test were implemented to make associations between congenital heart disease (CHD) and congenital pulmonary disease (CPD) and postoperative complications. Multiple logistic regression was performed to identify associations between CP and CHD/CPD while controlling for age, gender, and ASA class. C2 values were used to assess the logistic regressions, with a significance level of 0.05 indicating statistical significance. Length of stay (LOS), perioperative complications (readmission, reoperation, reintubation, wound dehiscence, cerebrovascular accidents, and mortality). 9 96 181 patients were identified in the database, 17 786 of whom were determined to have CP, of whom 16.0% had congenital heart defects (CHD) and 13.2% had congenital pulmonary defects (CPD). Patients with CHD and CPD were at a significantly greater risk of increased LOS and all but one operative complication rate (wound dehiscence) relative to patients with CP without a history of CHD and CPD. This study suggests that congenital cardiopulmonary disease is associated with increased adverse outcomes in the setting of CP repair. Thus, heightened clinical suspicion for coexisting congenital anomalies in the presence of CP should prompt referring providers to perform a comprehensive and multidisciplinary evaluation to ensure cardiopulmonary optimization prior to surgical intervention.

Newly formed and ruptured infectious intracranial aneurysm in few days following first intracranial aneurysm embolization concurrent with middle cerebral artery occlusion: A regrettable case in a baby child

AbstractInfectious intracranial aneurysm (IIA) and embolic cerebral infarction are well‐known devastating complications of children suffering infective endocarditis. In this report, we describe a successfully embolized IIA concurrent with bilateral middle cerebral artery (MCA) occlusion. Unfortunately, a newly formed IIA located in the contralateral MCA bifurcation ruptured at the seventh day following embolization. A 6‐month‐old female child was admitted to hospital 3 days following acute right limb mobility disorder. An interventional surgery history of congenital heart disease was confirmed. She was immediately started on antibiotic therapy and the computed tomography agiography (CTA) scan showed occlusion of the upper branch of the left MCA. Unfortunately an IIA was located in the distal artery region (DAR) of the ipsilateral anterior cerebral artery. Angiography (digital subtraction angiography) was performed and the DAR IIA was embolized by OnyX‐18 with Magic 1.2 Fr. microcatheter. On the sixth day, magnetic resonance imaging during the hospital stay showed reduced infarction area with no other special sign. Desperately, a major seizure with opisthotonos attacked the baby on the seventh day after embolization. An immediate CTA scan showed massive hematoma in the right basal ganglia and a ruptured bifurcate aneurysm of the right MCA. The parents refused positive treatment and discharged in considering the critical situation. It should be noted that IIA can be fast formed anywhere in cerebral artery and dynamic angio‐image should be performed as supervision.

FEASIBILITY OF SCHOOL-BASED SCREENING FOR HIGH BLOOD PRESSURE IN CHILDREN: THE HEALTHY HEARTS @ SCHOOL STUDY

Objective: High blood pressure (BP) in childhood is associated with end organ damage and cardiovascular events in mid-adulthood. Community-based BP screening may improve equitable access and awareness, but robust methodology is required. Accordingly, our aim was to assess the acceptability and feasibility of a two-stage school-based BP screening program incorporating targeted 24-hour ambulatory blood pressure monitoring (ABPM). Design and method: One-hundred-ninety-eight children (59% male, 7.6–12.7 years) in grades 3-6 were recruited from three primary schools in Melbourne, Australia. Following an education session for all children in these grades, BP was measured five times in students after parental consent, using the auscultatory method, alternating between arms and after five minutes of rest. Those with an average BP >85th percentile (AAP thresholds) or a risk factor for high BP had these measurements repeated two weeks later, along with ABPM. Families and students also completed an optional questionnaire post-program. Results: At the first assessment, 11 (5.6%) and 10 (5.0%) had elevated or hypertensive BP respectively (14.6% combined if using just the first measure). Fifty-nine (29.8%) were selected for the second assessment (including 10 with obesity, 15 born premature, four with a history of congenital heart disease, and nine with BP >85th percentile, but <90th percentile), of which 52 attended. ABPM indicated that 11 (5.6%) and five (2.5%) had white coat hypertension based on assessment one and two respectively, three (1.5%) had sustained hypertension (based on either assessment stage), and two (1%) had masked hypertension. Three additional participants each had either high BPs during school assessment but did not undertake, or had inconclusive, ABPMs. Overall, 11 (5.6%) were referred to a doctor for medical follow-up. The majority of students (67%) reported that ABPM was tolerable (‘bothersome’ score <8/10 for day and/or night). 96% of families approved of the program and 90% welcomed it as part of their child's schooling. Conclusions: These data indicate that a school-based BP screening program is acceptable and feasible, and that measuring BP on multiple occasions, and with targeted ABPM, is pivotal for minimising false positives and unnecessary referrals.

Imaging Evaluation of Clinical and Subclinical Myocarditis in Multisystem Inflammatory Syndrome of Children with COVID-19 Using Cardiac Magnetic Resonance: The Results and Prognosis

Background: Coronavirus disease 2019 (COVID-19) has emerged as the most significant health crisis in recent years, leading to over 6 million deaths globally due to the disease. Objectives: Given the prevalence of multisystem inflammatory syndrome in children (MISC) following the COVID-19 pandemic, this study aims to examine the imaging findings and prognoses of clinical and subclinical myocarditis in children with MISC through cardiac magnetic resonance (CMR) imaging. Patients and Methods: This prospective cohort study carried out over eighteen months from May 2021 to November 2022, included 14 patients who underwent CMR imaging. A census of all eligible patients during the study period served as the sampling method. Inclusion criteria were patients with confirmed COVID-19 infection through serological tests, polymerase chain reaction (PCR), or recent exposure to COVID-19 patients. Exclusion criteria included patients with a history of congenital heart disease (CHD) or pulmonary disease. Additional diagnostic tests performed included blood sample tests, chest X-ray (CXR), electrocardiogram (ECG), and echocardiography. CMR imaging was conducted on patients with cardiac involvement. A diagnosis of myocardial inflammation was made if a patient met at least two of the Lake Louise Criteria. The Chi-square, Fisher's exact, and Mann-Whitney tests were used to examine the relationship between quantitative variables and treatment outcomes. Additionally, the Wilcoxon signed rank, and McNemar’s tests assessed changes in echocardiography findings from admission to follow-up. A significance level of 0.05 was set. Results: Among the 14 patients studied, 8 (57.10%) were girls and 6 (42.90%) were boys. The average age was 6.03 ± 3.71 years. The median time to CMR imaging after symptom onset was 4 weeks (interquartile range (IQR): 2 - 12, range: 30). Global function assessment using left ventricular ejection fraction (LVEF) showed that 5 (35.70%), 3 (21.40%), and 6 (42.90%) patients had normal function, mild dysfunction, and significant LV dysfunction, respectively. 71.40% of patients who recovered had mild tricuspid regurgitation (TR) and no cardiomegaly. Significant differences in mean values of polymorphonuclear neutrophil (PMN) (37.71 ± 11.75 vs. 81.44 ± 13.06), lymphocytes (48.71 ± 20.08 vs. 12.51 ± 7.26), hemoglobin (Hb) (12.60 ± 1.55 vs. 10.10 ± 1.62), mean corpuscular volume (MCV) (85.90 ± 5.67 vs. 79.37 ± 5.23), erythrocyte sedimentation rate (ESR) (8.86 ± 13.60 vs. 30.29 ± 21.33), and C-reactive protein (CRP) (18.91 ± 27.25 vs. 100.57 ± 85.67) were observed between non-recovered and recovered patients, respectively (P < 0.05). However, no statistically significant association was found between other variables, including N-terminal pro–B-type natriuretic peptide (NT-proBNP), D-dimer, and Troponin I (TPI), with treatment outcomes (P > 0.05). Conclusion: Our findings indicate that a negative COVID-19 test does not exclude an established clinical COVID-19 infection in children with MISC. The results suggest that all children with MISC and a history of COVID-19 infection should undergo assessment for myocardial fibrosis, regardless of ejection fraction (EF) as determined by Echocardiography, laboratory tests, and COVID-19 test results. Strain analysis, conducted during both the acute phase and subsequent follow-ups through CMR imaging or Echocardiography, is recommended to enhance the understanding of the prognosis.

Bartonellosis (Oroya Fever, Peruvian Wart): A Case Report

Bartonellosis (also called Carrión's Disease, Oroya Fever or Peruvian Wart) is caused by the bite of mosquitoes of the Lutzomyia genus belonging to the Psychoid family. They are nocturnal blood-sucking insects, with complete metamorphosis. About 450 species are known, distributed throughout the American continent, mostly in tropical and subtropical areas. In Ecuador, sporadic cases have been reported, with certain characteristics such as, for example, not presenting the blood phase and, in the eruptive phase, not being aggressive. Here, we present a unique case of a 13-year-old boy with a history of congenital heart disease, who presented a clinical picture of one year of evolution suggestive of dermatosis, characterized by papules and nodules that occasionally bleed. The mother reports living in an area of lush vegetation and being in contact with bites. The results of blood count, peripheral blood smear, liver function and coagulation: normal. Blood sample: Negative Bartonella spp. PCR biopsy ribC gene: Positive B. bacilliformis. This case demonstrates the importance of maintaining an entomo-epidemiological surveillance system in patients exposed to arthropod bites that allows for timely detection of cases in order to make a diagnosis and provide timely treatment.

Challenging Case: A Multidisciplinary Approach to Demystifying Chronic Sleep Impairment in an Infant with a Complex Medical and Behavioral Profile.

Challenging Case: A Multidisciplinary Approach to Demystifying Chronic Sleep Impairment in an Infant with a Complex Medical and Behavioral Profile.

Newborn Pulse Oximetry Screening for Detecting Congenital Heart Disease: Experience at a Tertiary Care Center.

Congenital heart disease (CHD) remains the number one birth defect worldwide. Pulse oximetry screening (POS) is a widely used CHD screening modality effective in detecting critical lesions. This study is aimed at assessing the accuracy and cost-effectiveness of POS in a cohort of term well-babies admitted to a regular nursery in a tertiary care center. We reviewed the charts of term babies admitted to our regular nursery over a period of one year. The results of POS and the findings of echocardiography were collected. Similarly, we explored the records of our fetal echocardiography program to identify the fetuses screened for CHD during the same period. 900 term babies were born and admitted to newborn nursery at our center, and 69 fetuses were evaluated by our fetal cardiology team during the study period. None of our term babies had a positive POS at birth or 24 hours of age. However, 56 babies had a cardiac echo before hospital discharge due to suspicious findings on physical examination or a family history of CHD. A simple noncritical CHD was noted in 10 of them. Additionally, 53 babies underwent echocardiography within the first five years of life; a simple CHD was noted in 6 of them. In parallel, 21 of our fetuses were found to have CHD: 16 simple CHD and 5 critical CHD (CCHD). Despite its cost-effectiveness and efficacy in screening for CCHD, POS is suboptimal for detecting simple CHD. In the absence of a proper prenatal screening and fetal echocardiography program, POS remains a cost-effective modality for detecting CCHD.

Frontal Lobe Growth impairment in fetuses with congenital heart disease using Ultrasound parameters; A Comparative Cross-sectional study

Introduction and the Αim: 2D and 3D biometry measures can record the increased development of the fetal brain in the later part of gestation. The objective of this study is to determine whether or not the frontal brain regions of fetuses with congenital heart disease (CHD) are smaller than those of healthy controls. Patients and Μethods: The fetal Medicine unit of the Ain Shams University Maternity Hospital carried out a comparative cross-sectional study, including 140 normal fetuses and 140 fetuses with isolated CHD evaluated between 20 and 39 gestational weeks at our fetal medicine unit in the period November 2021–September 2023 Results: Our results showed the occipito-frontal diameter (OFD) and frontal antero-posterior diameter (FAPD) utilising ultrasonography. FAPD Mean±SD: 26.53 ± 10.44, OFD Mean±SD: 89.14 ± 28.34, and FAPD/OFD Mean±SD: 0.29 ± 0.05. The OFD Mean±SD was found to be 82.18 ± 18.42, the FAPD Mean±SD to be 31.68±6.58, and the FAPD/OFD Mean±SD to be 38.70±2.15. Our results showed that 0.7% of patients exhibited anomalies related to the central nervous system, such as dilated posterior fossa, vermis = 25 mm, and aberrant posterior cranial fossa. The percentage of those with a history of CHD was 3.9%.The history of CHD showed a statistically significant difference between the research groups. When examining comorbidities, surgical history, and the relationship between the other parameters and FAPD, OFD, and FAPD/OFD, there was no statistically significant difference between the groups under investigation, except for CNS abnormalities and gestational age. Conclusion, compared to normal fetuses, fetuses with CHD had shorter FAPDs and a lower FAPD/OFD ratio. Despite the fact that different types of CHD affect hemodynamics differently, there appears to be a common deficiency in the development of the frontal cortex.

Changes in the Etiological Structure and Specifics When Choosing Antibacterial Therapy Against Infective Endocarditis in Pediatric Practice

Infective endocarditis in children is a rare disease, which often has an extremely serious prognosis. The most common risk factor for infective endocarditis in this category of patients is the history of congenital heart disease. At the same time, currently, the disruption of the integrity of the peripheral vascular bed is increasingly noted, among the predisposing causes, due to invasive diagnostic and therapeutic medical procedures, as well as severe somatic diseases. In accordance with the developed recommendations, the appointment of antibacterial therapy for infective endocarditis should be based on etiotropic orientation. Taking these circumstances into account, the article presents an overview of the data reflecting the issues of etiology and choice of antibacterial therapy for infective endocarditis in pediatric practice.

Cardiovascular risk factors and their relationship to age of onset of Alzheimer’s disease in people with Down syndrome

AbstractBackgroundPeople with Down syndrome (DS) are at ultra‐high risk of developing Alzheimer’s dementia (AD). This is primarily due to the triplication of amyloid precursor protein on chromosome 21. Despite common genetic drivers there are large variations in the age of AD onset. In sporadic AD poor cardiovascular health has been associated with increased risk of AD. It has not been established whether the same risks apply to people with DS.MethodLarge population‐based cohort study utilising data from UK‐based primary care electronic health records. Measurements of nine cardiovascular parameters were used to analyse patterns across adulthood. These were systolic blood pressure (SBP), diastolic blood pressure (DBP), pulse pressure (PP), body mass index (BMI), total cholesterol, high and low density lipoproteins, triglycerides, and glycated haemoglobin (HbA1c). Survival curves were fitted with hazard ratios (HR) and crude incident rates calculated using cardiovascular parameters measured between age 25‐34 years as predictor variables and incidence of dementia as the outcome. Age, sex, level of intellectual disability, history of congenital heart disease (CHD), cardiovascular disease, hypothyroidism and diabetes were included as covariates.Result6,424 adults with DS were identified, matched to 23,143 controls. Low PP (<40mmHg), HR 1.45; low DBP (<62mmHg), HR 1.77, and low SBP (<100mmHg), HR 1.83 increased risk of dementia incidence. High SBP (>140mmHg), HR 2.04 increased risk but few people had high values. Raised BMI >25kg/m2 increased incidence dementia risk (HR 1.63). The risk was the same whether someone was overweight or obese. Hypothyroidism, history of CHD, male sex were significant contributors in the models. There was a cumulative effect of risk. People with 1 or 2 risk factors had HRs of 3.15 compared to those with zero risks, whilst people with 3 or more cardiovascular risk factors had HRs of 5.69.ConclusionCardiovascular health in earlier adulthood affects age of AD onset in people with DS. Cardiovascular multi‐morbidity was associated with increased incidence dementia risk suggesting that risks interact in a cumulative fashion. Reducing the number of risks by actively screening and managing medical co‐morbidities in people with DS may therefore help delay the onset of AD. Multi‐domain interventions may be particularly important.

Cochlear Implantation with Fontan Circulation: A Rare Case of Non-Cardiac Pediatric Surgery

Background: Fontan circulation is a cardiac circulation following the Fontan procedure in patients with a single ventricle congenital heart defect. This circulation depends on the difference in pressure between pulmonary capillaries and central venous pressure to achieve optimal cardiac output. This case report aims to discuss intraoperative anesthesia management in a patient with Fontan circulation undergoing a non-cardiac surgical operation.
 Case presentation: The patient is a 7-year-old male with a history of congenital heart disease who has undergone the Fontan circulation procedure and is now undergoing Cochlear implantation surgery. The patient was managed with general anesthesia - endotracheal intubation and controlled ventilation during the operation, following the principle of minimizing intrathoracic positive pressure, low PEEP, and low tidal volume with a target peripheral saturation > 95%. Cardiac output monitoring during the intraoperative period is done using intra-arterial blood pressure. After 5 hours of surgery, the patient was extubated with stable hemodynamics. 
 Conclusion: The primary goal of intraoperative anesthesia management in patients with Fontan circulation is to maintain an optimal transpulmonary gradient by ensuring optimal transpulmonary blood flow, thus preserving cardiac output.

Abstract 17386: Pulmonary Vein Stenosis After Pediatric Heart Transplant

Introduction: Pulmonary vein stenosis (PVS) is a rare condition in which neointimal proliferation of the pulmonary veins leads to venous and arterial hypertension. Little is known about the development of PVS after heart transplant (HTx) in children, which is a rare but known complication of HTx. Research Question: To describe the clinical characteristics, progression of disease, and outcomes of children who develop PVS after HTx. Methods: We performed a retrospective review of patients ≤18 years old who underwent HTx at two pediatric centers, Children’s Healthcare of Atlanta and Lurie Children’s Hospital, between April 2012-April 2022. Patients with a diagnosis of PVS were identified through queries of PVS databases at each center. Details related to the underlying cardiac diagnosis, location and extent of PVS, and post-HTx outcomes were recorded. Results: During the study period, 374 patients underwent HTx across both centers. Seventeen PVS patients (9 male) were identified, 15 of whom had de novo PVS. Fifteen had underlying congenital heart disease (CHD) and 2 had cardiomyopathy. Among CHD patients, 2 had a history of anomalous pulmonary venous return and 14 had prior cardiac surgery. Two patients had undergone catheter based PVS intervention prior to Tx. PVS was diagnosed at a median of 2 months (range 2 weeks-14 years) after HTx. At initial diagnosis, 12 patients had one-vessel PVS, most commonly the left upper pulmonary vein (n=8). At final follow-up, 2 patients had 2-vessel PVS and 7 had 3-4 vessels involved. Surgical intervention was required in 8 unique patients, most commonly for the left veins. Stent or balloon angioplasty were used in all left-sided veins, most commonly in the left lower pulmonary vein (n=8). Two patients were medically treated for pulmonary hypertension following PVS diagnosis. There were 3 deaths in the cohort, 2 of which were thought to be caused by right heart failure secondary to PVS. Conclusions: This is the first study to describe the characteristics of post-HTx PVS in children. PVS occurs in 4.5% of HTx and history of CHD is a strong risk factor. Multiple vessels can be involved and may require catheter-based or surgical intervention. Clinicians must be vigilant in monitoring for the development of PVS in this population.

Abstract 13945: Evaluation of a Deep Neural Network for Detection of D-Transposition of the Great Arteries on Fetal Echocardiograms

Introduction: While prenatal detection of congenital heart disease (CHD) has improved over the years, a significant proportion of CHD remains unrecognized on fetal ultrasound. Prenatal identification of d-transposition of the great arteries (d-TGA) in particular would significantly alter delivery planning and postnatal course. Research Questions: We investigated whether a deep neural network (DNN) can accurately detect d-TGA on fetal echocardiograms. Aim: To evaluate a DNN for detection of d-TGA on second (2T) or third (3T) trimester fetal echocardiograms. Methods: Patients with a singleton pregnancy with a 2T or 3T fetal echocardiogram performed between Jan 1, 2022 and May 1, 2023 at one center were retrospectively evaluated. Cases of d-TGA and negative cases referred for family history of CHD or increased nuchal translucency were included. Cases were reviewed by 3 pediatric cardiologists to confirm that the recorded ultrasound clips were sufficient to diagnose the presence or absence of d-TGA, and were excluded otherwise. The DNN was trained on patients not included in the evaluation to detect the absence of the great artery cross-over pattern, a distinct finding in d-TGA. The DNN outputs the presence or absence of this finding using all recorded video clips of an examination. Results: We included 18 positive and 99 negative cases. The DNN detected suspicion for d-TGA with an AUC of 91.4%, sensitivity of 83.3% (60.8-94.2, 95%CI) and specificity of 92.9% (86.1-96.5, 95%CI). Conclusions: D-TGA can be accurately detected on fetal echocardiograms using a DNN. These promising results lay the foundation for reliable, computer-aided detection of CHD on screening fetal ultrasounds.

Correlation between Ferritin Level and NT-ProBNP in Thalassemia Patient at M. Djamil Hospital Padang

Background: Thalassemia is a hereditary haemolytic anaemia caused by a genetic defect in the formation of globin chains. Routine blood transfusion is part of the management of thalassemia in Indonesia to maintain haemoglobin levels above the target haemoglobin. The consequence of this therapy is secondary excess of iron (ferritin), which adversely affects cardiac function. NT-pro BNP is one of the cheaper, more accessible, and non-invasive biomarkers that can be used to detect abnormalities in cardiac function. This study aims to analyse the correlation of ferritin levels with NT-proBNP in thalassemia patients. Method: This is an observational analytic study with a cross sectional approach. The inclusion criteria in the study were thalassemia patients who were ≤18 years old and routinely received repeated transfusions. Patients with a history of congenital heart disease, heart failure, hyperthyroidism, overt valvular disease, sepsis, or chronic kidney disease were excluded from this study. Correlation analysis was carried out by analysing the relationship between ferritin levels and NT-proBNP levels in children with thalassemia at RSUP Dr. M Djamil Padang. Result: A total of 27 children were included in this study. The median ferritin level of the samples was 3,465.00 (range 1,100-13,600) ng/ml. The median NT-Pro BNP level of the samples was 4.7767.00 (range 3.503-11.575). The results of the correlation analysis showed a significant positive correlation (p = 0.039) with a weak strength level (r = 0.399) between ferritin levels and NT-ProBNP levels. Conclusion: There is a significant correlation between ferritin levels and NT-proBNP. Keywords: Ferritin, NT-proBNP, Thalassaemia [PDF Full Text]

Effects of aprepitant on post-operative nausea and vomiting in patients with congenital heart disease undergoing cardiac surgery or catheterization procedures: a retrospective study with subjects as their own historical control

IntroductionFor patients undergoing cardiac surgery and catheterization procedures, severe post-operative nausea and vomiting (PONV) can occur despite standard anti-emetic interventions. Aprepitant, a neurokinin-1 (NK-1) receptor blocker, is safe and effective at preventing PONV resistant to standard therapies.MethodsPatients with a history of severe PONV presenting for cardiac surgery or catheterization procedures from January 1, 2018 to January 6, 2021 were identified. After pharmacist approval, patients received aprepitant pre-operatively (Dose: 80 mg for weight >50 kg, 40 mg for weight 30–50 kg). A retrospective chart review was performed. Primary outcomes of the incidence of PONV and PONV-related complications were evaluated.ResultsSeventeen patients were included with a mean age of 16.0 years at the time of their initial procedure, which acted as the “control” procedure, and 17.5 years when they received aprepitant. After the control procedure 64.7% of patients required rescue anti-emetics. When this group of patients received aprepitant pre-operatively at their subsequent procedure, only 17.6% required rescue medication (p = 0.005). Similarly, 64.7% of patients suffered at least one PONV-related complication after the control procedure. With aprepitant use pre-operatively, 5.9% of the same patients experienced a PONV-complication (p = 0.0003). Specifically, unplanned ICU admission due to severe PONV after catheterization procedures decreased from 55.6% (5/9) in the control group to 0 after these patients were treated pre-emptively with aprepitant (p = 0.01). For surgical patients, there were significant decreases in PONV-related complications including delayed oral intake and delayed ambulation (p = 0.04) in the aprepitant group compared to the control group.DiscussionThis small, retrospective study supports the conclusion that preoperative aprepitant administration in patients undergoing cardiac catheterization or cardiac surgery with a history of congenital heart disease and severe PONV significantly reduces the incidence of PONV and PONV-related complications. Decreasing these complications will likely improve the surgical experience for patients and families while also decreasing hospital costs and improving efficiency.

Repeat pediatric heart transplantation: A united network for organ sharing database analysis.

A history of congenital heart disease and previous transplantation are each independently associated with worse survival following pediatric heart transplantation. This study aimed to evaluate the characteristics and outcomes of children undergoing repeat heart transplantation in the United States based on the underlying diagnosis. The United Network for Organ Sharing database was used to identify 8111 patients aged<18 years undergoing isolated heart transplantation from 2000 to 2021, including 435 (5.4%) repeat transplants. Restricted cubic spline analysis assessed the non-linear relationship between inter-transplant interval and the primary outcome of all-cause mortality or re-transplantation. Multivariable Cox regression assessed the impact of re-transplantation on the primary outcome. Median follow-up was 5.0 (interquartile range 1.9-9.9) years. Repeat transplant patients were older (median age 12vs. 4 years; p<.001), and less likely to be in UNOS status 1A (66.0%, n=287vs. 81.0% n=6217; p<.001) than primary transplant patients. Freedom from the primary outcome was 51.4% (95% confidence interval [CI] 45.5-57.2) among repeat transplants and 70.5% (95% CI 69.2-71.8) among primary transplants at 10 years (p<.001). Among repeat transplant patients, the relative hazard of the primary outcome became non-significant when the inter-transplant interval>3.6 years. Congenital heart disease was an independent predictor of mortality among primary (HR 1.8, 95% CI 1.6-1.9) but not repeat transplant (HR 1.1, 95% CI .8-1.6) patients. Long-term outcomes remain poor for patients undergoing repeat heart transplantation, particularly those with an inter-transplant interval<3.6 years. Underlying diagnosis does not impact outcomes after repeat transplantation, after accounting for other risk factors.

Incidence and risk factors of congenital ventricular septal defect in Qingdao

Objective: To analyze the incidence and risk factors of ventricular septal defect (VSD) in Qingdao. Methods: A prospective cohort study design was used to include pregnant women who underwent prenatal screening in Qingdao between August 2018 and June 2020 (the whole population coverage). VSD was diagnosed according to the pulse oxygen saturation and heart auscultation, and the final diagnosis was made according to the echocardiography of VSD positive newborns within postnatal day 7. Results: The study included 115 238 live births, among which 388 were diagnosed as VSD, with an incidence of 3.37‰. The results of multivariate logistic regression analysis showed that mother with postgraduate level (OR=1.61, 95%CI: 1.00-2.58, P=0.049) (compared with junior high school and below), preterm birth history (OR=2.90, 95%CI: 1.47-5.70, P=0.002), and pregnancy history of congenital heart disease (OR=5.98, 95%CI: 2.63-14.73, P<0.001) were risk factors for VSD. Compared with female infants, the overall risk of VSD in male infants was relatively low (OR=0.74, 95%CI: 0.60-0.91, P=0.005). Conclusions: The incidence of VSD in Qingdao is 3.37‰. The risk factors of VSD include higher maternal education level, pregnancy history of congenital heart disease and preterm birth history. Moreover, the overall risk of VSD in male infants is low.

Epidemiological and clinical aspects of congenital heart disease in Fallujah Maternity and Children Hospital, Iraq

Objective: This study aimed to analyze the descriptive characteristics of children diagnosed with Congenital Heart Disease (CHD) at Fallujah Maternity and Children Hospital (FMCH).&#x0D; Methods: The prospective study included 1,025 pediatric patients born between 1st December 2018 and 30th September 2022, admitted to the newborns and children's wards, and diagnosed with CHD. Data were collected through interviews with patients' guardians using a registration and questionnaire form, capturing variables related to the child (e.g., name, sex, birth weight, type of CHD) and the mother (e.g., age, occupation, consanguinity, previous CHD births, chronic diseases during pregnancy). Residence (rural or urban) was also recorded.&#x0D; Results: Among the CHD cases, Atrial Septal Defect (ASD) was the most common (72%), followed by Ventricular Septal Defect (VSD) (25%) and Patent Ductus Arteriosus (PDA) (21%). Most diagnoses occurred in children aged 1-28 days. The male-to-female ratio was 1.2:1, and 0.2% had an ambiguous gender. About 58% of children weighed ≥ 2.5kg at birth, and 20% had a family history of CHDs.&#x0D; Conclusion: The increasing prevalence of congenital heart diseases in Fallujah requires preventive strategies targeting modifiable risk factors, better monitoring of high-risk pregnancies, and increased awareness of genetic counseling for families with congenital anomalies. The cardiology department should receive more attention and resources to improve diagnostic and therapeutic capabilities. Further research is needed to understand the reasons behind the recent rise in CHD cases in newborns.

PO-04-156 INSIGHTS REGARDING OUTCOMES FOR COVID-19 HOSPITALIZATIONS WITH ADULT CONGENITAL HEART DISEASE (ACHD)

As of December 2022, SARS-CoV-2 coronavirus resulted in over 6 million deaths worldwide.[1] It was realized early into the pandemic, that COVID-19 significantly impacts the Cardiovascular system. [2] Patients with pre-existing cardiovascular comorbidities were particularly at higher risk of adverse outcomes during their hospitalizations. [3] Similarly, it can be safe to assume patients with adult congenital heart disease (ACHD) should considered a high-risk population for the development of severe COVID infection with increased rates of significant cardiovascular complications. Based on this reasoning and the paucity of data available on this topic using a large database, we sought to investigate the outcomes of patients with ACHD who were admitted to the hospital with COVID-19. The National Inpatient Sample database for 2020 was queried to identify adult hospitalizations with a primary diagnosis of COVID-19 and a secondary diagnosis of ACHD using International Classification of Diseases – 10 Clinical Modification (ICD-10-CM) codes. The primary outcome studied was inpatient mortality, while secondary outcomes included inpatient complications, mean length of stay (LOS), and total hospital charge (THC). Multivariate logistic and linear regression analyses were used to adjust for possible confounders and analyze the variables. Out of 1,050,045 COVID-19 hospitalizations registered, 2,425 (0.23%) had ACHD as a secondary diagnosis. Encounters with ACHD who were hospitalized with COVID-19 had significantly higher adjusted odds of inpatient mortality (Adjusted Odds Ratio [aOR]: 1.4, [95% CI: 1.05-1.88], p=0.022), Longer LOS (Mean 2.4 days, [95% CI: 1.35-3.40], p <0.001), and higher Total Hospital Charges (Mean $53,000, [95% CI: 20811-85190], p <0.001). A Forrest plot (Figure 1) demonstrates a graphical representation of the multivariate analysis of the significant in-hospital complications when adjusted for patient demographics, comorbidities, and hospital characteristics. Among COVID-19 hospitalizations, those with a history of congenital heart diseases had significantly worse outcomes in terms of in-hospital mortality, sepsis; the need for endotracheal intubation, mechanical ventilation, and vasopressors; developing acute kidney injury and pulmonary embolism, in addition to the longer length of stay, and higher total hospital charges.

Surrogates of Muscle Mass on Cardiac MRI Correlate with Exercise Capacity in Patients with Fontan Circulation.

Sarcopenia is an increasingly recognized marker of frailty in cardiac patients. Patients with a history of congenital heart disease and Fontan procedure have a higher risk of developing progressive muscle wasting. Our objective was to determine if we could use routine cardiac MRI (CMR) for the surveillance of muscle wasting. A retrospective study of all Fontan patients (n = 75) was conducted at our institution, with CMR performed from 2010 to 2022 and exercise stress testing performed within 12 months (4.3 ± 4.2 months). The skeletal muscle area (SMA) for the posterior paraspinal and anterior thoracic muscles were traced and indexed for body surface area (BSA). Patients were stratified by percentile into the upper and lower quartiles, and the two groups were compared. Multivariable regression was performed to control for sex and age. There was a significant positive association of both anterior (r = 0.34, p = 0.039) and paraspinal (r = 0.43, p = 0.007) SMA to peak VO2. Similarly, paraspinal but not anterior SMA was negatively associated with the VE/VCO2 (r = -0.45, p = 0.006). The upper quartile group had significantly more males (18/19 vs. 8/20; p = 0.0003) and demonstrated a significantly higher peak VO2 (32.2 ± 8.5 vs. 23.8 ± 4.7, p = 0.009), a higher peak RER (1.2 ± 0.1 vs. 1.1 ± 0.04, p = 0.007), and a significantly lower VE/VCO2 (32.9 ± 3.6 vs. 40.2 ± 6.2, p = 0.006) compared to the lowest quartile. The association of SMA to VO2 peak and VE/VCO2 was redemonstrated after controlling for sex and age. Thoracic skeletal muscle area may be an effective surrogate of muscle mass and is correlated to several measures of cardiorespiratory fitness post-Fontan. CMR would be an effective tool for the surveillance of sarcopenia in post-Fontan patients given its accessibility and routine use in these patients.