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Background: Infectious endocarditis (IE) is an infectious disease caused by direct invasion of the heart valve, endocardium, or adjacent large artery endocardium by pathogenic microorganisms. Despite its relatively low incidence, it has a poor prognosis and a high mortality. Intracranial infectious aneurysms (IIA) and ruptured sinus of Valsalva aneurysm (RSVA) are rare complications of IE. Case presentation: We report a young male patient with symptoms of respiratory tract infection, heart murmurs and other symptoms and signs. The patient also had kidney function impairment and poor response to symptomatic therapy. Blood culture was negative, but echocardiography was positive, which met the diagnostic criteria for infective endocarditis. Moreover, an echocardiography showed a ruptured sinus of Valsalva aneurysm with a ventricular septal defect. Finally, secondary rupture of an IIA with multiple organ damage led to a poor clinical outcome. Conclusion: Therefore, in the clinical setting, for young patients with unexplained fever, chest pain, or palpitations, we need to be highly vigilant, considering the possibility of infective endocarditis and promptly performing blood culture, echocardiography, cerebrovascular imaging and so on, in order to facilitate early proper diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Background: Left ventricular outflow tract stenosis and atrioventricular valve regurgitation are often problems encountered in adulthood after complete atrioventricular septal defect repair. The surgical approach and indications for managing long-term outcomes such as left atrioventricular valve regurgitation and left ventricular outflow tract stenosis after complete atrioventricular septal defect repair have been discussed. Case presentation: A 23-year-old woman with intellectual disability was diagnosed with complete atrioventricular septal defect and underwent two-patch repair without cleft closure in childhood. Follow-up examination in adulthood demonstrated moderate left-sided atrioventricular valve regurgitation and left ventricular outflow tract stenosis with a circumferential ridge (peak velocity, 3.7 m/s; pressure gradient, 54 mmHg). Intraoperative findings showed a circumferential ridge under the aortic valve, and we removed the ridge. In addition, a cleft was present at the anterior leaflet, and we completely closed the cleft. Anticoagulation therapy was not initiated, and no embolic complications occurred. Follow-up echocardiography demonstrated no ridge under the aortic valve and only mild-range left AVVR. Conclusions: We successfully performed surgical treatment without valve replacement or anticoagulation therapy in a patient with poor medical compliance. Delayed reoperation leads to degeneration of the valve structure and makes more difficult to repair. Atrioventricular valve regurgitation should be evaluated in combination with based on the etiology of the regurgitation especially cleft related or not, in addition to the dilatation annulus, cleft size, and depth of the leaflet coaptation depth, and associated other valve diseases. [ABSTRACT FROM AUTHOR]

Introduction: Reducing cardiopulmonary bypass (CPB) induced inflammatory injury is a potentially important strategy for children undergoing multiple operations for single ventricle palliation. We sought to characterize the soluble receptor for advanced glycation end products (sRAGE), a protein involved in acute lung injury and inflammation, in pediatric patients with congenital heart disease and hypothesized that patients undergoing single ventricle palliation would have higher levels of sRAGE following bypass than those with biventricular physiologies. Methods: This was a prospective, observational study of children undergoing CPB. Plasma samples were obtained before and after bypass. sRAGE levels were measured and compared between those with biventricular and single ventricle heart disease using descriptive statistics and multivariate analysis for risk factors for lung injury. Results: sRAGE levels were measured in 40 patients: 19 with biventricular and 21 with single ventricle heart disease. Children undergoing single ventricle palliation had a higher factor and percent increase in sRAGE levels when compared to patients with biventricular circulations (4.6 vs. 2.4, p = 0.002) and (364% vs. 181%, p = 0.014). The factor increase in sRAGE inversely correlated with the patient's preoperative oxygen saturation (Pearson correlation (r) = −0.43, p = 0.005) and was positively associated with red blood cell transfusion (coefficient = 0.011; 95% CI: 0.004, 0.017; p = 0.001). Conclusions: Children with single ventricle physiology have greater increase in sRAGE following CPB as compared to children undergoing biventricular repair. Larger studies delineating the role of sRAGE in children undergoing single ventricle palliation may be beneficial in understanding how to prevent complications in this high-risk population. [ABSTRACT FROM AUTHOR]

There is limited experience in evaluating abnormal ductus arteriosus (DA) insertion pattern by contrast-enhanced cardiac computed tomography (cardiac CT) in patients with right-sided obstructive cardiac defects. Retrospective review of 38 infants with right-sided obstructive cardiac defects who underwent a preoperative cardiac CT between 2016 and 2021. We reviewed the types of cardiac lesions, patterns of ductal insertion, frequency of pulmonary artery (PA) stenosis requiring intervention, total dose length product (DLP), and effective radiation dose. Of 38 infants, 45% were female, the median gestational age and weight were 37 (range 34–40) weeks and 2.95 (range 2–4) kg. The most common pathologies were pulmonary atresia with ventricular septal defect (24%) and tetralogy of Fallot (24%). The abnormal ductal insertion patterns were DA inserting into the left PA in 39%, DA bifurcating into branch PA in 32%, and DA inserting into the right PA in 13%. Of the 38 infants, 76% developed branch PA stenosis requiring intervention. Among patients with abnormal DA insertion, 44% required branch PA arterioplasty during their index surgery compared to 17% without abnormal DA insertion. Regardless of the type of abnormal DA insertion, 67% developed bilateral branch PA stenosis over time. The mean DLP was 8 mGy-cm and the mean calculated effective radiation dose was 0.312 mSv. The utilization of contrast-enhanced cardiac CT in infants with right-sided obstructive heart defects can offer crucial insights into abnormal ductus arteriosus insertion patterns. This information is valuable for effective procedure planning and for monitoring the development of branch pulmonary artery stenosis. [ABSTRACT FROM AUTHOR]

Congenitally corrected transposition of the great arteries (ccTGA) is an infrequent and complex congenital malformation, which accounts for approximately 0.5% of all congenital heart defects. This defect is characterized by both atrioventricular and ventriculoarterial discordance, with the right atrium connected to the morphological left ventricle (LV), ejecting blood into the pulmonary artery, while the left atrium is connected to the morphological right ventricle (RV), ejecting blood into the aorta. Due to this double discordance, the blood flow is physiologically normal. Most patients have coexisting cardiac abnormalities that require further treatment. Untreated natural course is often associated with progressive failure of the systemic right ventricle (RV), tricuspid valve (TV) regurgitation, arrhythmia, and sudden cardiac death, which occurs in approximately 50% of patients below the age of 40. Some patients do not require surgical intervention, but most undergo physiological repair leaving the right ventricle in the systemic position, anatomical surgery which restores the left ventricle as the systemic ventricle, or univentricular palliation. Various types of anatomic repair have been proposed for the correction of double discordance. They combine an atrial switch (Senning or Mustard procedure) with either an arterial switch operation (ASO) as a double-switch operation or, in the cases of relevant left ventricular outflow tract obstruction (LVOTO) and ventricular septal defect (VSD), intra-ventricular rerouting by a Rastelli procedure. More recently implemented procedures, variations of aortic root translocations such as the Nikaidoh or the half-turned truncal switch/en bloc rotation, improve left ventricular outflow tract (LVOT) geometry and supposedly prevent the recurrence of LVOTO. Anatomic repair for congenitally corrected ccTGA has been shown to enable patients to survive into adulthood. [ABSTRACT FROM AUTHOR]

Background: Human adenovirus (HAdV) is an important pathogen causing acute respiratory infection (ARI) in children. Many countries, including China, have experienced sporadic or outbreaks related to HAdV-4, and death cases were reported. However, there is little research on HAdV-4 and the epidemic situation of HAdV-4 in China is little known. This study was designed to comprehend the prevalence and genetic characteristics of HAdV-4 in ARI children in China. Methods: Respiratory tract samples from ARI children hospitalized in six hospitals of Northern and Southern China from 2017 to 2020 were collected for HAdV detection and typing. Clinical information was collected from HAdV-4 positive patients for clinical characteristics and epidemiological analysis. The main capsid proteins and the whole genome sequences were amplified and sequenced for bioinformatics analysis. Results: There were 2847 ARI children enrolled, and 156 (5.48%) HAdV positive samples were detected. Eleven HAdV-4 positive samples were identified, accounting for 0.39% of the total samples and 7.05% of the HAdV positive samples. The main manifestations were fever and cough. Two children had conjunctivitis. Two children were diagnosed with severe pneumonia and developed respiratory failure. One of them developed hemophagocytic syndrome and checked in pediatric intensive care unit (PICU). This child had ventricular septal defect. All the children recovered. The isolated strains of HAdV-4 obtained in this study and the reference strains from China located in the same phylogenetic branch (HAdV-4a), while the prototype strain and vaccine strains formed another branch (HAdV-4p). Upon comparison with the prototype strain, there were a few amino acid mutations existing in three major capsid proteins. According to recombination analysis, no new recombination was found. Conclusions: The detection rate of HAdV-4 in children hospitalized with ARI was 0.39% in the total samples and 7.05% of all HAdV positive samples. HAdV-4 isolates obtained in this study and other reference strains from China belonged to the HAdV-4a subtype. Our data provided reference for the monitoring, prevention and control of HAdV-4, as well as the research and development of vaccines and drugs. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to examine whether there is a correlation between different types of ventricular septal defects (VSD) and chromosomal abnormalities in the low-risk setting of non-invasive prenatal testing (NIPT) and to evaluate the prognosis of fetuses with varying types of VSD. Methods: Cases of pregnant women who underwent amniocentesis due to fetal VSD were collected by Tianjin Central Hospital of Obstetrics and Gynecology from May 2017 to May 2022. Exclusions were made for those without NIPT, with high-risk NIPT results, genetic disorders, and those lost to follow-up. Data collected included ultrasound classification of VSD, prenatal NIPT results, copy-number variations (CNVs) results, and neonatal outcomes. Results: The prevalence of pathogenic CNVs was investigated in 74 cases of VSDs. Of these cases, 45 were isolated VSDs (9 muscular and 36 non-muscular) and 29 were non-isolated VSDs (10 with intracardiac and 19 with extra-cardiac structural anomalies). The results revealed that the incidence of pathogenic CNVs was lower in isolated VSDs compared to non-isolated VSDs in a low-risk NIPT condition (χ2 = 9.344, P = 0.002). There was no significant difference in the prevalence of pathogenic CNVs between VSDs with intracardiac and extra-cardiac structural anomalies (P = 0.541). Moreover, VSDs associated with intracardiac structural anomalies had the highest rate of surgical intervention. Conclusion: When NIPT is low-risk and VSD is isolated, the likelihood of fetal chromosomal defects is not increased. However, if there are intra- or extra-cardiac structural abnormalities present alongside VSD, the possibility of pathogenic CNV is considerably greater, necessitating invasive prenatal diagnosis. Isolated muscular VSDs usually do not require surgery, which can be used as a basis for prenatal counseling regarding fetal VSD. [ABSTRACT FROM AUTHOR]

Background Residual interatrial shunt following percutaneous patent foramen ovale (PFO) closure is a rare complication that can be associated with persistent migraine. Case summary A 32-year-old woman with a history of percutaneous PFO closure due to a previous coronary paradoxical embolism and a condition of drug-refractory migraine underwent transoesophageal echocardiography (TOE) to investigate further recurrent migraine attacks. A partial displacement of a PFO occluder device was found, and it was successfully treated through the fluoroscopic and TOE-guided positioning of a second occluder in overlap with the previous one. The patient reported no more migraine attacks at the 6-month follow-up visit. Discussion Residual interatrial shunt should be suspected in patients with persistent migraine and a history of percutaneous PFO closure. Fluoroscopic and TOE-guided closure of residual interatrial shunt with a second PFO occlude device seems to be a safe and clinically effective strategy to manage persistent migraine. [ABSTRACT FROM AUTHOR]

Objective: Neurocristopathies play a role as pathogenesis of Hirschsprung's disease and congenital heart diseases (CHDs). This study seeks to identify concomitant deformities, syndromes, and/or associations associated with Hirschsprung's disease warrant evaluation for CHDs through echocardiography. Materials and Methods: A retrospective analysis was conducted on Hirschsprung's disease patients at Siriraj Hospital between January 2006 and December 2022. Echocardiograms were performed when clinical symptoms, abnormal chest X-rays (CXR), desaturation, heart murmurs raised suspicions of cardiovascular anomalies. Results: Among 299 Hirschsprung's disease patients, 43 (14.4%) exhibited CHDs. The sensitivity of CXR (n=268) and echocardiograms (n=51) in diagnosing CHDs was 48.8% and 100%, respectively. Predominant CHD presentations included patent ductus arteriosus (n=29), atrial septal defects (n=18), and ventricular septal defects (n=15). The presence of concomitant deformities, syndromes and/or associations associated with Hirschsprung's disease significantly heightened the likelihood of concurrent CHDs (Odds ratio = 23.56, p < 0.001). Patients with Hirschsprung's disease and concomitant deformities (n=28) (excluding syndromic or chromosomal abnormalities) had 1.73 times the odds of CHDs (p = 0.262) compared to those without concomitant deformities. Patients with Hirschsprung's disease and Down syndrome (n=34) exhibited 77.78 times higher odds of CHDs (p < 0.001), while those with other syndromes and/or associations (n=6) had 13.03 times higher odds of CHDs (p = 0.005) compared to patients lacking these conditions. Conclusion: CHDs were identified in 14.4% of Hirschsprung's disease patients. Echocardiograms should be selectively employed in Hirschsprung's disease associated with Down syndrome, other syndromes, or concomitant deformities. [ABSTRACT FROM AUTHOR]

Background: Cleft in the mitral valve leaflet is a primary cause of congenital mitral regurgitation, stemming from developmental anomalies in the mitral valve and frequently associated with other congenital heart defects. Concurrent presence of cleft in mitral valve leaflet with atrial septal defect and ventricular septal defect is relatively rare. Echocardiography, especially transesophageal echocardiography, is essential in diagnosing cleft mitral valve leaflet and related congenital heart defects, providing critical, detailed imagery for accurate assessment. This study presents a young female patient whose anterior mitral cleft, along with atrial septal defect and ventricular septal defect, was revealed through three-dimensional transesophageal echocardiography. Case presentation: A 25-year-old Iranian female, experiencing progressive dyspnea and diminished physical capacity over 3 months, was referred to our hospital. Initial examination and transthoracic echocardiography indicated severe mitral regurgitation. Further evaluation with transesophageal echocardiography corroborated these findings and identified a cleft in the anterior mitral valve leaflet, coupled with mild left ventricular enlargement and significant left atrial enlargement. The complexity of the patient's condition was heightened by the diagnosis of cleft mitral valve leaflet in conjunction with atrial septal defect and ventricular septal defect, showing the complex nature of congenital defects. Conclusion: This case emphasizes the critical role of transthoracic echocardiography in diagnosing cleft of mitral valve leaflet and associated cardiac anomalies, showcasing its superiority over transthoracic echocardiography for detailed visualization of cardiac structures. The identification of multiple congenital defects highlights the necessity for a comprehensive diagnostic approach to manage and treat patients with complex congenital heart diseases effectively. Future research should aim to refine diagnostic methodologies to enhance patient outcomes for cleft of mitral valve leaflets and related congenital conditions. [ABSTRACT FROM AUTHOR]

Background: Chromosome 7 has regions enriched with low copy repeats (LCRs), which increase the likelihood of chromosomal microdeletion disorders. Documented microdeletion disorders on chromosome 7 include both well-known Williams syndrome and more rare cases. It is noteworthy that most cases of various microdeletions are characterized by phenotypic signs of neuropsychological developmental disorders, which, however, have a different genetic origin. The localization of the microdeletions, the genes included in the region, as well as the structural features of the sequences of these genes have a cumulative influence on the phenotypic characteristics of the individuals for each specific case and the severity of the manifestations of disorders. The consideration of these features and their detailed analysis is important for a correct and comprehensive assessment of the disease. Results: The article describes a clinical case of 7p22.3 microdeletion in a patient with congenital heart defect and neurological abnormalities - epilepsy, combined with moderate mental and motor developmental delay. Conclusions: Through detailed genetic analyses, we are improving the clinical description of the rare 7p22.3 microdeletion and thus creating a basis for future genetic counseling and research into targeted therapies. [ABSTRACT FROM AUTHOR]

This research letter discusses the different phenotypes of ABL1 deficiency syndromes caused by mutations in different isoforms of the ABL1 gene. Somatic mutations in ABL1 are associated with cancer, while germline gain-of-function variants are linked to Congenital Heart Defects and Skeletal Malformations Syndrome (CHDSKM). Biallelic loss-of-function variants in ABL1 are associated with Human ABL1 Deficiency Syndrome (HADS). The letter presents a case study of a Lebanese family with a history of cardiac abnormalities, including a child with a stenotic subaortic membrane. The study highlights the various types of heart defects and genetic mutations in the ABL1 gene, suggesting that different mutations can lead to distinct phenotypes. Further research is needed to fully understand ABL1-associated disorders. The study was funded by the Lebanese American University and the authors have no conflicts of interest. [Extracted from the article]

Phonocardiography (PCG) is used as an adjunct to teach cardiac auscultation and is now a function of PCG-capable stethoscopes (PCS). To evaluate the efficacy of PCG and PCS, the authors investigated the impact of providing PCG data and PCSs on how frequently murmurs, rubs, and gallops (MRGs) were correctly identified by third-year medical students. Following their internal medicine rotation, third-year medical students from the Georgetown University School of Medicine completed a standardized auscultation assessment. Sound files of 10 different MRGs with a corresponding clinical vignette and physical exam location were provided with and without PCG (with interchangeable question stems) as 10 paired questions (20 total questions). Some (32) students also received a PCS to use during their rotation. Discrimination/difficulty indexes, comparative chi-squared, and McNemar test p-values were calculated. The addition of phonocardiograms to audio data was associated with more frequent identification of mitral stenosis, S4, and cardiac friction rub, but less frequent identification of ventricular septal defect, S3, and tricuspid regurgitation. Students with a PCS had a higher frequency of identifying a cardiac friction rub. PCG may improve the identification of low-frequency, usually diastolic, heart sounds but appears to worsen or have little effect on the identification of higher-frequency, often systolic, heart sounds. As digital and phonocardiography-capable stethoscopes become more prevalent, insights regarding their strengths and weaknesses may be incorporated into medical school curricula, bedside rounds (to enhance teaching and diagnosis), and telemedicine/tele-auscultation efforts. [ABSTRACT FROM AUTHOR]

Background: Congenital heart disease (CHD) is the most prevalent congenital anomaly, but its underlying causes are still not fully understood. It is believed that multiple rare genetic mutations may contribute to the development of CHD. Methods: In this study, we aimed to identify novel genetic risk factors for CHD using an ENU-based dominant genetic screen in mice. We analyzed fetuses with malformed hearts and compared them to control littermates by whole exome or whole genome sequencing (WES/WGS). The differences in mutation rates between observed and expected values were tested using the Poisson and Binomial distribution. Additionally, we compared WES data from human CHD probands obtained from the Pediatric Cardiac Genomics Consortium with control subjects from the 1000 Genomes Project using Fisher's exact test to evaluate the burden of rare inherited damaging mutations in patients. Results: By screening 10,285 fetuses, we identified 1109 cases with various heart defects, with ventricular septal defects and bicuspid aortic valves being the most common types. WES/WGS analysis of 598 cases and 532 control littermates revealed a higher number of ENU-induced damaging mutations in cases compared to controls. GO term and KEGG pathway enrichment analysis showed that pathways related to cardiac contraction and neuronal development and functions were enriched in cases. Further analysis of 1457 human CHD probands and 2675 control subjects also revealed an enrichment of genes associated with muscle and nervous system development in patients. By combining the mice and human data, we identified a list of 101 candidate digenic genesets, from which each geneset was co-mutated in at least one mouse and two human probands with CHD but not in control mouse and control human subjects. Conclusions: Our findings suggest that gene mutations affecting early hemodynamic perturbations in the developing heart may play a significant role as a genetic risk factor for CHD. Further validation of the candidate gene set identified in this study could enhance our understanding of the complex genetics underlying CHD and potentially lead to the development of new diagnostic and therapeutic approaches. [ABSTRACT FROM AUTHOR]

Background: The prevalence of celiac disease (CD) and hypothyroidism exhibit significant variation in different studies among patients with congenital heart disease (CHD). This study evaluated the frequency of laboratory test abnormalities in children and adolescents with CHD in Shiraz, Iran. Methods: This prospective case-control study was conducted on 223 children and adolescents with CHD and healthy individuals referred to the heart clinic affiliated with Shiraz University of Medical Sciences between February 2019 and December 2021. They were classified into case and control groups. Blood tests were performed for total IgA antibody, anti-tissue transglutaminase IgA antibody (anti-TTG Ab), T4, and thyroid stimulating hormone (TSH) and anti-thyroid peroxidase antibodies in serum, along with transthoracic echocardiography. Likewise, demographic characteristics of patients, including age, sex, weight, height, and body mass index (BMI), were recorded. Also, anti-TTG Ab levels were compared among CHD patients according to cyanosis status, gender, age (above and below five years), and BMI (under and over 18.5). Results: Ninety-eight CHD patients and 100 healthy individuals with an average age of 5.32 ± 4.05 years (1–18 years) were examined. In children with CHD, atrial septal defect (27%), ventricular septal defect (20%), and tetralogy of Fallot (13%) were the most prevalent disorders. Only one CHD patient had an anti-TTG Ab level of 16.6 unit/mL, considered borderline for seropositive CD diagnosis. There was no difference in anti-TTG Ab levels between age (above and below five years), BMI (under and over 18.5), cyanosis status, and gender groups. Seven CHD patients had high TSH levels, three had cyanotic CHD, and one had Down syndrome. The TSH levels and non-autoimmune hypothyroidism were significantly higher in CHD patients than in normal subjects (p < 0.05). Conclusions: According to the results of this study, the serum level of TSH and prevalence of non-autoimmune hypothyroidism were higher in patients with CHD than in normal subjects, but the serum level of anti-TTG Ab was not different between the two groups. [ABSTRACT FROM AUTHOR]

Background: Ventricular septal defect (VSD) is the most common congenital heart disease. Although a small number of genes associated with VSD have been found, the genetic factors of VSD remain unclear. In this study, we evaluated the association of 10 candidate single nucleotide polymorphisms (SNPs) with isolated VSD in a population from Southwest China. Methods: Based on the results of 34 congenital heart disease whole-exome sequencing and 1000 Genomes databases, 10 candidate SNPs were selected. A total of 618 samples were collected from the population of Southwest China, including 285 VSD samples and 333 normal samples. Ten SNPs in the case group and the control group were identified by SNaPshot genotyping. The chi-square (χ2) test was used to evaluate the relationship between VSD and each candidate SNP. The SNPs that had significant P value in the initial stage were further analysed using linkage disequilibrium, and haplotypes were assessed in 34 congenital heart disease whole-exome sequencing samples using Haploview software. The bins of SNPs that were in very strong linkage disequilibrium were further used to predict haplotypes by Arlequin software. ViennaRNA v2.5.1 predicted the haplotype mRNA secondary structure. We evaluated the correlation between mRNA secondary structure changes and ventricular septal defects. Results: The χ2 results showed that the allele frequency of FLT4 rs383985 (P = 0.040) was different between the control group and the case group (P < 0.05). FLT4 rs3736061 (r2 = 1), rs3736062 (r2 = 0.84), rs3736063 (r2 = 0.84) and FLT4 rs383985 were in high linkage disequilibrium (r2 > 0.8). Among them, rs3736061 and rs3736062 SNPs in the FLT4 gene led to synonymous variations of amino acids, but predicting the secondary structure of mRNA might change the secondary structure of mRNA and reduce the free energy. Conclusions: These findings suggest a possible molecular pathogenesis associated with isolated VSD, which warrants investigation in future studies. [ABSTRACT FROM AUTHOR]

Aim Saline contrast-enhanced electrical impedance tomography (EIT) has been used to identify the respiratory failure etiologies through assessment of regional lung perfusion at the bedside. In this study, we introduce a novel approach to detect right-to-left intracardiac shunt based on the center of heart (CoH) parameter determined from the early phase of impedance–time curve after saline bolus injection. Methods and result The timepoints when the saline bolus enter the heart (T0) and the lung regions (T1) are identified at first. A moving time window from T0 to T1 is then generated with steps of 0.5 s and the slope of the impedance–time curve in each pixel within the window calculated. CoH is calculated as the geometric center of pixel slope values in the right-to-left image direction. To illustrate how this method works in practice, we calculated the CoH values at T0 to T1 in 10 control hypoxic patients with no right-to-left shunt. In addition, we examined two critically ill patients with right-to-left intracardiac shunt. One was postcardiac surgery patient who had a residual atrial septal defect by color doppler of transesophageal echocardiograph. The other patient had a congenital heart disease of ventricular septal defect by color doppler of trans-thoracic echocardiography. A large difference in CoH between T0 to T1 was observed in the two patients with intracardiac shunt than in the control patients (11.06 ± 3.17% vs. 1.99 ± 1.43%, P  = 0.030). Conclusion Saline bolus EIT for lung perfusion might be used as ventriculography to identify the right-to-left intracardiac shunt at the bedside. [ABSTRACT FROM AUTHOR]

Background: Ventricular septal defect (VSD) is a lethal complication of myocardial infarction. Case presentation: A 67-year-old male who presented with a history of chest pain associated with shock... Physical examination was significant for crepitation in lower lung fields. Echo showed a left ventricular ejection fraction (LVEF) of 30-35% and muscular ventricular septal defects with left to right shunting and elevated pulmonary artery systolic pressure. In a short time, he developed respiratory failure during the hospital course. Our patient had VSD due to acute myocardial infarction (MI). The patient was transferred to another hospital on a ventilator for surgical intervention but died before any procedure. Conclusion: Our case highlights an unfortunate patient with a VSD secondary to myocardial infarction (MI) resulting in Death. [ABSTRACT FROM AUTHOR]

The article comments on a study which examined circumferential strain derived from the short axis of the ventricles in neonatal cardiac function. It summarizes findings of the study and suggests the importance of considering the potential variability in strain reference values in relation to the width of the region on interest. It argues for the establishment of reference values in healthy neonates in situations of myocardial ischemia such as perinatal asphyxia.

Infective endocarditis (IE) affects the endothelium of the heart, with the heart valves most commonly involved. It has been documented that the annual incidence of infective endocarditis is 3-10 per 100,000 patient-years1. However, it can be underestimated since the incidence in developing countries cannot be determined accurately. Here, we present a case of a 37-year-old male who was referred from a local health facility with shortness of breath on presentation; the patient was anuric for one day and initial laboratory investigations showed metabolic acidosis, hyperkalemia, sepsis, and deranged renal function tests. The patient had received a three-week course of intravenous (IV) piperacillin-tazobactam at the previous health facility, being diagnosed as a case of infective endocarditis. An initial transthoracic echocardiogram (TTE) showed vegetation on the pulmonary valve; however, the patient was neither an IV drug abuser nor did he have any history of implantation of intracardiac devices or central venous catheters. There was no recent or remote history of dental or surgical procedures. Due to the acute kidney injury, hemodialysis sessions and IV imipenem were started. As the patient’s hemodynamic profile improved by the fifth day of admission, TTE was repeated, revealing a small ventricular septal defect (VSD). This case report highlights the importance of even small VSD that could potentially lead to right-sided IE. Surgical correction of VSD could prevent such a life-threatening condition. [ABSTRACT FROM AUTHOR]

Maternal diabetes has been related to an increased risk of congenital heart disease (CHD) in offspring. However, inconsistent results were retrieved for studies evaluating the association between gestational diabetes mellitus (GDM) and CHD in offspring. We therefore performed a systematic review and meta-analysis for comprehensive investigation. Observational studies were identified by searching PubMed, Embase, and Web of Science according to the aim of the meta-analysis. A randomized-effects model was used to pool the data by incorporating the influence of potential heterogeneity. Twenty-three observational studies, involving 46953078 mother-child pairs, were available for the meta-analysis. Among them, 2131800 mothers were diagnosed as GDM and 214379 newborns had CHD. Overall, maternal GDM was associated with a higher incidence of CHD in offspring [odds ratio (OR): 1.32, 95% confidence interval (CI): 1.21 to 1.45, p<0.001; I2=62%]. Sensitivity analysis limited to studies with adjustment of maternal age and other potential confounding factors showed similar results (OR: 1.40, 95% CI: 1.30 to 1.51, p<0.001; I2=47%). Subgroup analysis suggested that the association between maternal GDM and CHD in offspring was not significantly affected by methods for diagnosis of GDM, methods for confirmation of CHD, or study quality scores (p for subgroup difference all>0.05). Subsequent analysis according to types of CHD showed that maternal GDM was associated with higher risks of atrial septal defect, ventricular septal defect, and Tetralogy of Fallot. Maternal GDM may be associated with a higher risk of CHD in offspring. [ABSTRACT FROM AUTHOR]

Objectives: Congenital heart defects (CHDs) are among the most frequent congenital defects, and they significantly burden the healthcare system due to their high mortality rate and high cost of care for survivors. We aimed to highlight the incidence of CHDs in a tertiary center in Western Romania. Methods: A retrospective study was carried out between 2018 and 2022 at the "Pius Brinzeu" Emergency County Hospital Timisoara. Relevant information about the mothers and the newborns were collected and statistically analyzed. Results: The incidence of CHDs from 2018 to 2022 in our center was 5.3%. Eleven types of malformations were diagnosed postnatally in 541 newborns, with 28.8% of cases having more than one type of CHD. The antenatal detection rate was 28%, with the highest rates for tetralogy of Fallot, hypoplastic left heart syndrome, or significant ventricular septal defects and the lowest for pulmonary stenosis. The lower antenatal detection rate was influenced mainly by incomplete or absent prenatal care. Conclusions: The incidence of CHDs is clearly dependent of a multifactorial approach, and the results highlight this. With an incidence almost 50% lower than reported within the literature and a low rate of prenatal detections, CHDs could be a more of a burden to endure regarding medical treatment. Improvements in patients' education, prenatal care, and screening programs could improve diagnosis, decrease mortality, and optimize postnatal care. [ABSTRACT FROM AUTHOR]

His-bundle pacing has demonstrated feasibility in numerous adult studies to reverse and prevent pacing-induced cardiomyopathy, however, is met with higher capture thresholds with deployment sheaths designed for adults with his-bundles in the typical location. To describe 24 pediatric and adult congenital patients post-physiologic pacing. Patients at the University of Minnesota Masonic Children's Hospital with congenital complete heart block or congenital heart disease and atrioventricular block presented for pacemaker placement between November 2019 and January 2021. Twenty-four patients had attempted his-bundle placement using either Medtronic's C315 or C308 sheaths and 3830 leads except for 3 patients who had Boston Scientific's His system with the Shape 3 sheath and 7842 leads. Twenty-four total patients underwent physiologic pacing (23 his-bundle, 13 female, 11 male) with median age of 14 years (range 8–39 years) with median weight of 51 kg (range 21.2–81 kg) with five right-sided implants performed. Twelve patients had congenital heart disease including atrioventricular canal defects, tetralogy of Fallot, and ventricular septal defect repairs (nine patients with ventricular septal defect repairs). Twelve patients had selective His-bundle pacing (six with congenital heart disease). Median threshold to capture was 0.5 V at 0.4 ms (range 0.4 to 1.1 V at 0.4 ms), impedance 570 ohms (range 456–1140 ohms), and sensing median of 9.7 mV (range 1.5–13.8 mV if present). The median follow-up time was 610 days (range 240–760 days). No complications occurred peri-procedurally or during follow-up. His-bundle pacing is feasible in pediatric and congenital heart disease patients. [ABSTRACT FROM AUTHOR]

Data on concomitant cardiac surgery (CCS) performed during pediatric lung transplantation (LTx) is limited. Therefore, we conducted a multi-institutional analysis to identify the incidence and outcomes of CCS in pediatric (< 18 years) LTx recipients by merging data (2004–2023) from the United Network for Organ Sharing (UNOS) and Pediatric Health Information System (PHIS) databases. Of the total of 596 pediatric LTx recipients, 87 (15%) underwent CCS. The majority of these cardiac surgeries were atrial septal defect (ASD) closure (90%) followed by aortic arch/descending aortic repair (3%), atrial repair (3%), ventricular septal defect closure (2%), patent ductus arteriosus ligation (2%), and tricuspid valve repair (2%). The median age at LTx was 3 years (IQR: 0–12). Pulmonary hypertension (PHT) was the predominant indication for LTx (54%). Survival to discharge was 94% and 5-years survival was 64%. Our findings indicate CCS in children undergoing LTx has acceptable outcomes. [ABSTRACT FROM AUTHOR]

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Background Approximately 25% of the general population has a patent foramen ovale (PFO) that remains asymptomatic in the vast majority. Right-to-left shunt (RLS)-mediated hypoxaemia is a rare associated condition of PFO. Case summary This report describes a case of percutaneous PFO closure for hypoxaemia in a 73-year-old patient showing immediate clinical benefit. She experienced progressive dyspnoea on exertion requiring oxygen therapy. SaO2 was 87% at rest without oxygen therapy, which increased to 98% after percutaneous PFO closure. Discussion Most PFOs remain clinically insignificant but RLS-mediated hypoxaemia is a rare phenomenon that can occur even at advanced age. Percutaneous PFO closure is a safe and effective therapy option that provides immediate improvement of hypoxaemia. [ABSTRACT FROM AUTHOR]

Background Severe aortic paravalvular leaks (PVLs) after surgical mechanical aortic valve replacement (AVR) represent a high risk for congestive heart failure, haemolysis, and infective endocarditis. This is the first reported case of distal radial artery (DRA) access for severe mechanical aortic PVL closure with a sequential double vascular plug guided by computed tomography angiography (CTA), transoesophageal echocardiography (TOE), and 3D TOE in an acute setting. Case summary A 51-year-old male presented with significant mixed aortic valve disease. Aortic valve replacement was performed (Slimline Bicarbon A-25 mm) according to guidelines. Four and 16 days later, a re-exploration was carried out due to pericardial effusion. Four months after discharge from rehabilitation, the patient was readmitted due to worsening dyspnoea on exertion and then at rest. Transthoracic echocardiography, TOE, and consequently, CTA, revealed severe PVL, following which the procedure of transcatheter PVL closure was chosen, with a preference for DRA access. After a CTA scan analysis and angiographic, TOE, and 3D TOE visualization of the leak, a 14/5 mm and a 10/5 mm vascular plug (AVPIII) were deployed to achieve good results. A 9-month clinical, echocardiographic, and CTA follow-up revealed good long-term results. Discussion For transcatheter PVL closure, CTA is helpful for not only vascular access planning, but also a visualization of the magnitude of the leak, location, and device planning. This case report demonstrates that the distal radial approach is feasible in patients with severe mechanical aortic valve PVL retrograde transcatheter closure. DRA access could possibly represent less bleeding and vascular access site complications when compared with femoral access and has some potential advantages over regular radial access. [ABSTRACT FROM AUTHOR]

Air pollution and heavy metal exposure are emerging public health concerns. Prenatal exposure to air pollutants and heavy metals has been implicated in the development of congenital heart disease (CHD). However, the relationship between exposure to airborne heavy metals and CHD has not yet been investigated. Therefore, in this large population-based study, we investigated the association between air pollutants, including airborne heavy metals, and the risk of CHD using national health insurance claims data from South Korea. Data regarding 1,129,442 newborns and their mothers were matched with air pollutant levels during the first 8 weeks of gestation. In the five-air pollutant model, we found significant positive correlations between prenatal exposure to sulfur dioxide (SO2; odds ratio [OR] 6.843, 95% confidence interval [CI] 5.746–8.149) and cadmium (Cd; OR 1.513, 95% CI 1.187–1.930) and the risk of ventricular septal defects in newborns. This study highlights the association between prenatal exposure to air pollutants, including airborne heavy metals, and an elevated CHD risk. Further research is essential to validate and expand these findings, with the ultimate goal of enhancing public health outcomes. [ABSTRACT FROM AUTHOR]

Ventricular septal defects are a rare complication after acute myocardial infarction with a mortality close to 100% if left untreated. However, even surgical or interventional closure is associated with a very high mortality and currently no randomized controlled trials are available addressing the optimal treatment strategy of this disease. This state-of-the-art review and clinical consensus statement will outline the diagnosis, hemodynamic consequences and treatment strategies of ventricular septal defects complicating acute myocardial infarction with a focus on current available evidence and a focus on major research questions to fill the gap in evidence. [ABSTRACT FROM AUTHOR]

Background: Secundum atrial septal defect (ASD) is one of the most common congenital heart conditions. Transcatheter device closure of secundum ASD has become the preferred treatment for patients with suitable anatomy. While, the impact on ventricular function and volume post-closure has been widely studied, data on atrial performance following percutaneous ASD closure remains limited. Objective: This study aimed to assess atrial function using echocardiographic modalities and atrial rhythm through Holter monitoring early after percutaneous ASD closure. Patients and Methods: The study included 40 patients with secundum ASD, evaluated before and shortly after percutaneous closure. Atrial performance was assessed using two-dimensional echocardiography, tissue Doppler imaging (TDI) velocities, and speckle-tracking echocardiography (STE) to measure reservoir, conduit, and contractile functions. Electrical function was monitored with electrocardiograms (ECG) and Holter monitoring to detect heart rate (HR) rhythms and heart rate variability (HRV) before and after ASD closure. Results: Right atrial diameters, volumes, and TDI velocities significantly decreased post-closure. RA reservoir function dropped from 34.58 ± 11.69 before closure to 28.73 ± 11.52 after closure (p<0.001). Similarly, LA reservoir function decreased from 31.4 ± 7.28 to 27.08 ± 6.93 (p<0.001). Average HR decreased from 98.08 ± 13.51 bpm to 92.05 ± 13.23 bpm (p<0.001). The incidence of supraventricular arrhythmia increased significantly post-closure (0.41 ± 0.46%) compared to pre-closure (0.08 ± 0.19%; p<0.001). Conclusion: Atrial diameters and volumes decreased early after ASD device closure, along with reductions in atrial reservoir, conduit, and contractile functions. Supraventricular arrhythmias increased, but there was an improvement in HR and HRV parameters post-closure. [ABSTRACT FROM AUTHOR]

Background: Atrial septal defect (ASD) closure has become an established therapy. Novel echocardiographic methods have been developed to quantify global as well as regional ventricular function and are used for diagnostic and prognostic evaluation of various cardiovascular diseases. Objective: To assess the impact of transcatheter closure of secundum atrial septal defects on the left and right ventricular function using conventional and newer echo-Doppler modalities. Patients and methods: This study included 40 subjects with secundum ASD. Cases were assessed by transesophageal echo pre ASD closure to detect suitability for transcatheter ASD closure, then selected patients were evaluated by transthoracic echo-Doppler using conventional and recent modalities before closure, one month and three months after closure. Results: Post ASD closure there were increase in the Left ventricle (LV) dimensions and volumes, increase in LVEF, and reduction of LV-GLS compared to pre closure. The RV volumes, RV-GLS and RVEF were decreased post closure. Both LV Tei index and RV Tei index showed significant reduction post ASD closure. Conclusion: Transcatheter closure of secundum atrial septal defect leads to structural and functional changes in both ventricles in the form of: (a) reduction in RV dimensions and volumes with apparent deterioration of the RV systolic function, (b) increase in dimensions and volumes of the LV with apparent improvement of LV systolic function, and (c) apparent improvement of both left and right ventricular diastolic function post ASD closure. [ABSTRACT FROM AUTHOR]

Purpose: The volume of surgical services has significantly reduced globally due to the coronavirus disease 2019 (COVID-19) pandemic. This study evaluated the level of recovery in terms of the number of operations performed in Japan in 2021, based on nationwide periodic surveillance. Methods: Information on the weekly and annual volumes of 20 representative procedures in 6 surgical subspecialties in 2021 was extracted from the National Clinical Database. Statistical data for 2018 and 2019 (pre-pandemic era) were compared with those for 2020. Data on waves of infection, peak period, and high-prevalence areas (13 of 47 prefectures) were analyzed individually. Results: The volumes of the 10 procedures, including gastrectomy, hepatectomy, valve replacement and valve plasty, coronary artery bypass grafting, infrarenal abdominal aorta replacement, ventricular septal defect closure, lung lobectomy, inguinal hernia repair (age < 16 years old), and appendectomy (age < 16 years old), did not reach 95% of that in the pre-pandemic era. The most striking decline in the surgical volume of these 10 procedures was observed during the peak period of wave 5 in high-prevalence areas. Conclusion: This near-complete enumeration survey identified the polarization of 20 representative procedures in terms of resumption of surgical service after the pandemic. [ABSTRACT FROM AUTHOR]

Tetralogy of Fallot is the most common heart defect associated with cyanosis characterized by the co-occurrence of pulmonary stenosis, right ventricular hypertrophy, and ventricular septal defect with over-riding of the aorta. The present review purposed to summarize myocardial, valvular and vascular abnormalities, which were described in a series of patients following repair of tetralogy of Fallot. It was also aimed to describe potential differences in these parameter using different surgical strategies. [ABSTRACT FROM AUTHOR]

Aim: To examine whether antenatal diagnosis modifies relationships between neonatal brain volumes and 18‐month neurodevelopmental outcomes in children with transposition of the great arteries (TGA). Method: In a retrospective cohort of 139 children with TGA (77 antenatally diagnosed), we obtained total brain volumes (TBVs) on pre‐ (n = 102) and postoperative (n = 112) magnetic resonance imaging. Eighteen‐month neurodevelopmental outcomes were assessed using the Bayley Scales of Infant and Toddler Development, Third Edition. Generalized estimating equations with interaction terms were used to determine whether antenatal diagnosis modified associations between TBVs and neurodevelopmental outcomes accounting for postmenstrual age at scan, brain injury, and ventricular septal defect. Results: Infants with postnatal diagnosis had more preoperative hypotension (35% vs 14%, p = 0.004). The interactions between antenatal diagnosis and TBVs were significantly related to cognitive (p = 0.003) outcomes. Specifically, smaller TBVs were associated with lower cognitive scores in infants diagnosed postnatally; this association was attenuated in those diagnosed antenatally. Interpretation: Antenatal diagnosis modifies associations between neonatal brain volume and 18‐month cognitive outcome in infants with TGA. These findings suggest that antenatal diagnosis may be neuroprotective, possibly through improved preoperative clinical status. These data highlight the need to improve antenatal diagnosis rates. What this paper adds: Antenatal diagnosis of transposition of the great arteries modified relationships between neonatal brain volume and neurodevelopment.Smaller brain volumes related to poorer cognitive scores with postnatal diagnosis only.There was more preoperative hypotension in the postnatal diagnosis group. [ABSTRACT FROM AUTHOR]

Objective: Extracorporeal membrane oxygenation (ECMO) is a life-saving treatment option providing cardiopulmonary support when standard therapies prove insufficient for reversible diseases. The mean objective of this study was to evaluate our center’s experience with ECMO following pediatric cardiac surgery. Materials and Methods: This retrospective study was conducted in our pediatric intensive care unit (PICU) between November 2014 and March 2021 and included patients who received ECMO following cardiac surgery. Results: Over the 7-year period, 324 patients underwent cardiac surgery, of which 24 (7.4%) required ECMO support. Among them, 13 (54.2%) were female, with a median age of 16.0 (2.0-208) months and a median weight of 7.0 (3.5-70) kg. The mean vasoactive inotrope score (VIS) was 53.9 ± 44.5. Atrioventricular septal defect repair was the most common surgical procedure (n = 8/24, 41.6%). The primary indication for ECMO was low cardiac output syndrome (LCOS) in 14 (58.3%) patients. The median duration of ECMO support was 6.0 (1.0-46.0) days. Nonsurvivors had significantly higher Pediatric Risk Score of Mortality (PRISM) III scores (P = .014) and VIS scores during the pre-ECMO period (P = .004). Early or late neurological complications developed in 12 (50%) patients, with significant differences in lactate levels and pH levels pre-ECMO between those with and without neurological complications (P = .01, P = .02, respectively). We successfully decannulated 16 (66.6%) patients, with a final survival rate of 12 (50%). Conclusion: ECMO plays a crucial role in providing pre- and post-cardiac surgery support for children. LCOS remains the main indication, and high PRISM III and VIS scores are valuable predictors of outcomes. [ABSTRACT FROM AUTHOR]

BACKGROUND: Congenital heart disease surgery is related to significant postoperative complications which have been associated with poor patient outcomes. However, the prevalence, predictors, and effect of complications on outcomes have only been evaluated in a few numbers of studies. This study aimed to assess the magnitude of postoperative complications after congenital heart surgery, their predictors, and the associations between complications and patient outcomes. METHODS: A retrospective, single-center cross-sectional study was done at the Cardiac Center of Ethiopia (CCE) on children who had undergone cardiac surgery between 2009 and 2022. All 919 pediatric patients aged below 18 years who had undergone congenital heart surgery were included in the study. RESULT: Of the 919 patients who underwent surgery in the cardiac center of Ethiopia, Ventricular septal defect( VSD) was the most common diagnosis (41%) and VSD patch closure (39.4%) was the most common surgical procedure. The presence of at least one complication was identified in 39.5% of patients. Of all patients, 11.3% had cardiac, 23.1% had extra-cardiac complications, and 5.3%% had major complications. Higher age at diagnosis, lower weight at admission, cyanotic congenital heart disease, higher Risk adjustment for congenital heart surgery score (RACH-1 score), higher vasoactive inotropic score, Cardiopulmonary bypass use during surgery, higher cardiopulmonary bypass time, higher aortic cross-clamp time, higher duration of surgery, and the higher number of surgeries were associated with complications. The duration of mechanical ventilation, length of intensive care unit stays, and length of hospital stay was significantly prolonged in patients with complications. CONCLUSION: Congenital heart disease surgeries pose a high risk of complications, and these complications are associated with poor patient outcomes in Ethiopia. Therefore, predicting complications based on risk factors and early detection and treatment is crucial to improve the patient's outcome. [ABSTRACT FROM AUTHOR]

Key Clinical Message: In this study, we introduced one of the rarest concomitants of the absence of left pulmonary artery (LPA), which was seen in our patient along with patent ductus arteriosus (PDA) and ventricular septal defect (VSD). Unilateral absence of pulmonary artery (UAPA) is a congenital heart disease in association with other abnormalities such as tetralogy of Fallot and septal defects or isolated in 30% of cases and occurs in the right lung in two thirds of cases. Our case is a 33‐year‐old man who was hospitalized with symptoms of cough, shortness of breath, and hemoptysis. The echocardiography revealed a large ventricular septal defect, absent left pulmonary artery, and severe pulmonary hypertension (PH) along with patent ductus arteriosus. These findings were confirmed by CT angiography. This association has rarely been found in past studies. Due to PH and pulmonary infection, the patient was treated with intravenous prostaglandin and antibiotics. However, in cases of timely diagnosis and treatment of UAPA, fatal complications such as pulmonary hypertension, morbidity, and mortality are reduced. This case emphasizes the importance of awareness of this abnormality and its associated anomalies to enable early diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Objectives: We proposed a strategy for the creation of a 6-mm transjugular intrahepatic portosystemic shunt (TIPS) and to assess its effectiveness compared to a conventional 8-mm shunt for TIPS-induced hepatic encephalopathy (HE). Methods: Patients were reviewed retrospectively using propensity score matching (1:1) and divided into 6-mm and 8-mm shunt groups based on shunt diameter. The stent patency, HE incidence, and rebleeding rate between the two groups were then compared. Results: From January 2018 to June 2021, both 6-mm shunt group and 8-mm shunt group included 58 patients. The 6-mm shunt group had significantly smaller liver volumes (879.3 ± 237.1 vs. 1008.8 ± 293.0; p = 0.010), and the median stent patency times were 30.7 and 33.8 months in the 6-mm and 8-mm groups, respectively (p = 0.124). No statistically significant difference was found between the two groups in the 1-year (8.6% vs. 3.4%; p = 0.242) and 2-year (17.2% vs. 12.1%; p = 0.242) rebleeding rates. The 1-year cumulative incidences of overt HE were 12.1% and 27.6% in the 6-mm and 8-mm groups, respectively (p = 0.040), and the 2-year cumulative overt HE incidences in these groups were 19.0% and 36.2%, respectively (p = 0.038). Notably, patients with a 6-mm shunt also experienced less hepatic impairment. Conclusions: For patients with variceal bleeding and a small liver volume, the 6-mm shunt significantly reduced the incidence of overt HE, protected perioperative liver function, and did not affect stent patency or rebleeding rate. Clinical relevance statement: For patients with variceal bleeding with small liver volume, the 6-mm transjugular intrahepatic portosystemic shunt (TIPS) significantly reduced the incidence of overt hepatic encephalopathy after TIPS, protected perioperative liver function, and did not affect stent patency and rebleeding rate. Key Points.: • A strategy for the creation of a 6-mm transjugular intrahepatic portosystemic shunt for patients with variceal bleeding and a small liver volume was proposed. • The 6-mm transjugular intrahepatic portosystemic shunt significantly reduced the incidence of overt hepatic encephalopathy. • The 6-mm transjugular intrahepatic portosystemic shunt did not affect stent patency or rebleeding rate. [ABSTRACT FROM AUTHOR]

We report a 5‐year‐old girl with transient complete atrioventricular (AV) block following surgical closure of a symptomatic conoventricular ventricular septal defect (VSD) which recovered on post‐operative day 9. She later presented with exertional dizziness and fatigue. While congenital cardiac defect repairs are occasionally complicated by complete heart block, this patient was found to have intra‐Hisian Wenckebach which is rare in the pediatric population and can be very difficult to discern from surface electrocardiograms and by Holter monitoring. Mechanisms of post‐surgical AV block, including intra‐Hisian Wenckebach, are not well characterized in the pediatric population. [ABSTRACT FROM AUTHOR]

Congenital heart diseases (CHDs) represent a heterogeneous group of congenital defects, with high prevalence worldwide. Non-invasive imaging is essential to guide medical and surgical planning, to follow the patient over time in the evolution of the disease, and to reveal potential complications of the chosen treatment. The application of cardiac magnetic resonance imaging (CMRI) in this population allows for obtaining detailed information on the defects without the necessity of ionizing radiations. This review emphasizes the central role of CMR in the overall assessment of CHDs, considering also the limitations and challenges of this imaging technique. CMR, with the application of two-dimensional (2D) and tri-dimensional (3D) steady-state free precession (SSFP), permits the obtaining of very detailed and accurate images about the cardiac anatomy, global function, and volumes' chambers, giving essential information in the intervention planning and optimal awareness of the postoperative anatomy. Nevertheless, CMR supplies tissue characterization, identifying the presence of fat, fibrosis, or oedema in the myocardial tissue. Using a contrast agent for angiography sequences or 2D/four-dimensional (4D) flows offers information about the vascular, valvular blood flow, and, in general, the cardiovascular system hemodynamics. Furthermore, 3D SSFP CMR acquisitions allow the identification of coronary artery abnormalities as an alternative to invasive angiography and cardiovascular computed tomography (CCT). However, CMR requires expertise in CHDs, and it can be contraindicated in patients with non-conditional devices. Furthermore, its relatively longer acquisition time and the necessity of breath-holding may limit its use, particularly in children under eight years old, sometimes requiring anesthesia. The purpose of this review is to elucidate the application of CMR during the pediatric age. [ABSTRACT FROM AUTHOR]

Copyright of Türkiye Klinikleri Journal of Ophthalmology is the property of Turkiye Klinikleri and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) worldwide. It accounts for 7% of CHD cases in Uganda and leads to fatal outcomes in the long term without surgery. Surgery is often delayed in developing countries like Uganda due to limited resources. Objective: This study aimed to determine the early surgical outcomes of patients with TOF who underwent primary intracardiac repair at the Uganda Heart Institute (UHI) and to identify factors associated. Methodology: This retrospective chart review evaluated outcomes of primary TOF repair patients at UHI from February 2012 to October 2022. Patient outcomes were assessed from surgery until 30 days post-operation. Results: Out of the 104 patients who underwent primary TOF repair at UHI, records of 88 patients (84.6%) were available for review. Males accounted for 48.9% (n = 43). The median age at the time of operation was 4 years (with an interquartile range of 2.5-8.0 years), ranging from 9 months to 16 years. Genetic syndromes were present in 5/88 (5.7%). These included 2 patients with trisomy 21, 2 with Noonan's, and 1 with 22q11.2 deletion syndrome. Early postoperative outcomes for patients included: residual ventricular septal defects in 35/88 (39.8%), right ventricular dysfunction in 33/88 (37.5%), residual pulmonary regurgitation in 27/88 (30.7%), residual right ventricular outflow tract obstruction in 27/88 (30.0%), pleural effusion in 24/88 (27.3%), arrhythmias in 24/88(27.3%), post-operative infections in 23/88(26.1%) and left ventricular systolic dysfunction in 9/88 (10.2%). Out of the children who underwent surgery after one year of age, 8% (7 children) died within the first 30 days. There was a correlation between mortality and post-operative ventilation time, cardiopulmonary bypass (CPB) time, aortic cross-clamp time, preoperative oxygen saturations, RV and LV dysfunction and the operating team. Conclusion: The most frequent outcomes after surgery were residual ventricular septal defects and right ventricular failure. In our study, the 30-day mortality rate following TOF repair was 8%. Deceased patients had lower pre-operative oxygen levels, longer CPB and cross-clamp times, longer post-operative ventilation, RV/LV dysfunction, and were more likely operated by the local team. [ABSTRACT FROM AUTHOR]

We describe the echocardiographic features of a 22-year-old female with a giant aneurysm of membranous ventricular septum (AMVS). Both transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) demonstrated significant dilatation of the aortic annulus and severe aortic regurgitation. A giant aneurysm was detected extending from a large membranous ventricular septal defect (MVSD) to the anterior surface of the aortic root. Contrast-enhanced CT and three-dimensional CT revealed a giant aneurysm located below the aortic root and connected to the left ventricular outflow tract (LVOT). The diagnosis was confirmed by surgery and postoperative pathological examination. [ABSTRACT FROM AUTHOR]

Simple Summary: In a groundbreaking procedure, our team successfully performed surgery on a young Ragdoll cat with a rare type of heart defect known as an infundibular muscular ventricular septal defect, which is a hole between the two lower heart chambers. This condition, if left untreated, can lead to severe heart problems. Therefore, the surgery was performed using a method called cardiopulmonary bypass, which is quite unusual in cats. This approach involved stopping the heart temporarily to repair the defect safely. Despite being a challenging situation, in which an adjacent structure was unintentionally damaged intraoperatively, resulting in a temporary complication that was treated non-surgically, the cat recovered well. By 490 days postoperatively, the cat was healthy with only a minor residual defect, showing that the heart had nearly returned to its normal size. This case highlights a promising surgical option for treating similar heart defects in cats, which can potentially improve their quality of life significantly. Therefore, with proper surgical treatment, even in cases of severe congenital heart disease in cats, owners will be able to live with their cats without the need for numerous medications. Ventricular septal defects (VSDs) can lead to congestive heart failure and pulmonary hypertension, particularly in patients with large shunts. However, no surgical treatment for feline VSD has been reported. Here, we elucidated the first surgical correction of an infundibular muscular VSD in a one-year-old Ragdoll cat, atypically located and classified under the Soto classification rather than the standard Kirklin classification, through cardiac arrest using cardiopulmonary bypass—a method rarely used in feline cardiac surgery. Detailed echocardiography revealed that the defect required intervention owing to left heart and main pulmonary artery enlargement. Despite the VSD being located on the contralateral side, as anticipated in the preoperative examinations, the choice of median sternotomy allowed for the successful closure of the defect. Conversely, the insertion of two cannulas into the ascending aorta resulted in damage to the adjacent thoracic duct, causing transient chylothorax, which was resolved with conservative treatment. Cardiac arrest induced by a cardioplegic solution facilitated the surgical procedure, although it leads to anemia in cats. However, on postoperative day 490, the patient exhibited only minor residual shunting, with normalized heart size, and remained healthy. This technique appears to be a viable treatment option for congenital heart disease in cats. [ABSTRACT FROM AUTHOR]

Patients with previous interatrial shunt device (IASD) implantation may face greater challenges during future left atrial interventional procedures. Herein, we report the first case of left atrial appendage closure (LAAC) in a patient with previous IASD implantation. The patient successfully underwent LAAC using the LAmbre device without complications. [ABSTRACT FROM AUTHOR]

Background: Persistent truncus arteriosus is a rare congenital cyanotic heart defect characterized by a single ventricular outflow tract. Without surgical intervention, it has a poor prognosis in infancy. Here, we report an adult female patient with uncorrected truncus arteriosus type I, who presented with acute-onset abdominal pain due to torsion of a small bowel gastrointestinal stromal tumor (GIST). Case Report: A 41-year-old woman came to our Emergency Department with acute-onset lower abdominal pain for 2 days. Congenital heart disease, truncus arteriosus, had been diagnosed at birth, and there had been no surgical intervention. Abdominal computed tomography revealed a 10×9×12-cm mixed-density mass in the pelvic capacity. Transthoracic echocardiography revealed a 33-mm ventricular septal defect. The ascending aorta originated mainly from the right ventricle, and the pulmonary artery originated from the beginning of the aorta (type I truncus arteriosus, according to Collett and Edwards classification). After a quick and detailed preoperative workup, the patient underwent tumor resection by open surgery with general anesthesia. Conclusions: This is the first case to report emergency surgery for a patient with uncorrected persistent truncus arteriosus due to torsion of a small bowel GIST. A multidisciplinary team with deep understanding of the disease entity was crucial. By considering the fixed hemodynamic and respiratory physiology, overtreatment and unrealistic goals were avoided. Eventually, the patient was discharged after being hospitalized for 2 weeks. [ABSTRACT FROM AUTHOR]

Introduction: The left ventricular summit (LVS) is the highest point on the epicardial surface of the left ventricle. A part of the LVS that is located between the left coronary arteries (lateral‐LVS) is one of the major sites of idiopathic ventricular arrhythmia (VA) origins. Some idiopathic epicardial VAs can be ablated at endocardial sites adjacent to the epicardial area septal to the lateral‐LVS (septal‐LVS). This study examined the prevalence and electrocardiographic and electrophysiological characteristics of septal‐LVS VAs. Methods: We studied consecutive patients with idiopathic VAs originating from the LVS (67 patients) and aortic root (93 patients). Results: Based on the ablation results, among 67 LVS VAs, 54 were classified as lateral and 13 as septal‐LVS VAs. As compared with the lateral‐LVS VAs, the septal‐LVS VAs were characterized by a greater prevalence of left bundle branch block with left inferior‐axis QRS pattern, later precordial transition, lower R‐wave amplitude ratio in leads III to II, lower Q‐wave amplitude ratio in leads aVL to aVR, and later local ventricular activation time relative to the QRS onset during VAs (V‐QRS) in the great cardiac vein. The electrocardiographic and electrophysiological characteristics of the septal‐LVS VAs were similar to those of the aortic root VAs. However, the V‐QRS at the successful ablation site was significantly later during the septal‐LVS VAs than aortic root VAs (p <.0001). The precordial transition was significantly later during the septal‐LVS VAs than aortic root VAs (p <.05). Conclusions: Septal‐LVS VAs are considered a distinct subgroup of idiopathic VAs originating from the left ventricular outflow tract. [ABSTRACT FROM AUTHOR]

Lin, Tian, Huaping Jia, Yunming Li, Yongxing Xu, Bei Zhao, Dong Zheng, Hongfeng Yan, Meihui Zhao, Yanlei Li, Liping Xia, Fengxia Zhou, Cuiping Liu, Ke Ma, Ma Mi, and Jianwen Gu. Epidemiological survey of congenital heart disease among children aged from 2 to 18 in Suo County, Nagqu, Tibet. High Alt Med Biol. 00:000–000, 2024. Background: Studies have reported the prevalence of congenital heart disease (CHD) in parts of Tibet, but relative epidemiological surveys are rare. We aimed to explore the prevalence of CHD in children and its relationship with family history in Suo County, Nagqu, Tibet, an altitude of 3,980 meters. Methods: We recruited 4,002 children aged 2–18 years. Subjects underwent a family history investigation, cardiac auscultation, and clinical manifestation examination and then received echocardiographic screening. Results: The prevalence of CHD among children in Suo County was 0.97% (39 cases), much higher than the prevalence at sea level. The most common subtype was atrial septal defect, accounting for 53.9% of CHD, followed by patent ductus arteriosus (33.3%) and ventricular septal defect (12.8%). We also found that children whose mothers had previously borne children with CHD had a higher risk of CHD than those without (p = 0.002); other factors related to CHD during pregnancy, such as smoking, drinking, drug use, and viral infection, showed no statistical differences between children with and without CHD. Conclusions: The prevalence of CHD in children in Suo County is much higher than at low altitude, consisting mostly of simple forms with left-to-right shunt, with rare complex CHD. These results support implementing diagnostic and treatment plans to prevent CHD in Suo County. [ABSTRACT FROM AUTHOR]

Left ventricular outflow tract obstruction (LVOTO) remains a significant complication after primary repair of interrupted aortic arch with ventricular septal defect (IAA-VSD). Clinical and echocardiographic predictors for LVOTO reoperation are controversial and procedures to prophylactically prevent future LVOTO are not reliable. However, it is important to identify the patients at risk for future LVOTO intervention after repair of IAA-VSD. Patients who underwent single-stage IAA-VSD repair at our center 2006–2021 were retrospectively reviewed, excluding patients with associated cardiac lesions. Two-dimensional measurements, LVOT gradients, and 4-chamber (4C) and short-axis (SAXM) strain were obtained from preoperative and predischarge echocardiograms. Univariate risk analysis for LVOTO reoperation was performed using unpaired t-test. Thirty patients were included with 21 (70%) IAA subtype B and mean weight at surgery 3.0 kg. Repair included aortic arch patch augmentation in 20 patients and subaortic obstruction intervention in three patients. Seven (23%) required reoperations for LVOTO. Patient characteristics were similar between patients who required LVOT reoperation and those who did not. Patch augmentation was not associated with LVOTO reintervention. Patients requiring reintervention had significantly smaller LVOT AP diameter preoperatively and at discharge, and higher LVOT velocity, smaller AV annular diameter, and ascending aortic diameter at discharge. There was an association between LVOT-indexed cross-sectional area (CSAcm2/BSAm2) ≤ 0.7 and reintervention. There was no significant difference in 4C or SAXM strain in patients requiring reintervention. LVOTO reoperation was not associated with preoperative clinical or surgical variables but was associated with smaller LVOT on preoperative echo and smaller LVOT, smaller AV annular diameter, and increased LVOT velocity at discharge. [ABSTRACT FROM AUTHOR]