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Abstract Objective To analyze the clinical characteristics and types of congenital heart defect (CHD) in mild congenital anorectal malformation (CARM), namely the rectoperineal and rectovestibular fistulas. Methods The retrospective study of 183 patients with mild CARM was conducted with assessments of demographic information, color Doppler echocardiography results, and follow-up data. We performed an analysis of the clinical characteristics of CHD, grouping them based on sex and type of mild CARM. Results Of the 183 patients, rectoperineal fistula occurred in 133 patients (72.7%), while the frequency of CHD was 79.8% (146/183). Ventricular septal defects (VSDs) occur more frequently in patients with rectoperineal fistula compared to those with rectovestibular fistula (1.5% vs. 10%), while the opposite trend was observed for patent ductus arteriosus (PDAs) (39.8% vs. 22.0%). Additionally, males presented higher frequency of PDA (42.7% vs. 26.4%) and self-healing (6 months: 87.2% vs. 42.6%; 12 months: 91.0% vs. 63.2%) than females. However, males had a lower rate of undergoing cardiac surgery (6.4% vs. 17.6%) and a younger median diagnosis age (1 day vs. 9 days). Conclusion Our study indicates that there is a necessity for meticulous cardiac assessment and follow-up in neonates diagnosed with mild CARM.

Xuning Lu,1,2 Guoju Li,1 Qin Wu,1 Wei Ni,1 Silin Pan,1 Quansheng Xing1 1Heart Center, Qingdao Women and Children’s Hospital, Shandong University, Qingdao, Shandong Province, People’s Republic of China; 2Heart Center, Dalian Municipal Women and Children’s Medical Center (Group), Dalian, Liaoning Province, People’s Republic of ChinaCorrespondence: Quansheng Xing, Heart Center, Qingdao Women and Children’s Hospital, Shandong University, 217 Liaoyang West Road, Qingdao, Shandong Province, 266034, People’s Republic of China, Tel +86 136 0642 6893, Fax +86 532 8285 7650, Email xqs_qdfnet@126.comObjective: The outcomes of fetuses with isolated congenital heart disease (CHD) diagnosed prenatally have not been investigated in a population-based study in China. This population-based study aimed to evaluate the rate of voluntary termination of pregnancy after the prenatal diagnosis of isolated CHD in Qingdao, China.Methods: This was a population-based retrospective study in which data were collected from all pregnant women in Qingdao (eastern China) from August 2018 to July 2020; fetal data, maternal data and data on pregnancy outcomes were extracted from medical records regarding prenatal diagnosis of CHD. The inclusion criteria were as follows: pregnant women or their husbands who had a household registration in Qingdao and who underwent regular prenatal screening in Qingdao. The exclusion criterion was the failure to sign an informed consent form. Counseling for all parents of fetuses with CHD was provided by a multidisciplinary team of experienced pediatric cardiologists, obstetricians, geneticists, etc. According to the type and severity of CHD, the pregnancy termination rate was analyzed.Results: Among the 126,843 pregnant women, 1299 fetuses with a prenatal diagnosis of CHD were included in the study. Among the included fetuses, 1075 were diagnosed with isolated CHD, and the overall pregnancy termination rate was 22.8%. Termination rates varied according to the complexity of CHD (low complexity vs moderate complexity, P=0.000; low complexity vs high complexity, P=0.000; moderate complexity vs high complexity, P=0.000), with rates of 6.0% for low complexity, 54.2% for moderate complexity, and 99.1% for high complexity. The decision to terminate the pregnancy in cases of isolated CHD was unrelated to maternal age (P=0.091) but was related to gestational age (p=0.000).Conclusion: In Qingdao, 99.1% of parents whose fetuses were diagnosed with isolated high-complexity CHD chose to voluntarily terminate the pregnancy. The pregnancy termination rate increased with increasing complexity of prenatally diagnosed CHD.Plain Language Summary: In our study population, with the popularity of prenatal diagnosis, complex congenital heart disease will disappear, because 99.1% of parents whose fetuses were diagnosed with isolated high-complexity congenital heart disease chose voluntary pregnancy termination.Keywords: congenital heart defect, fetal echocardiography, prenatal diagnosis, termination of pregnancy

Persistent left superior vena cava drain into the left atrium is frequently overlooked since it rarely has a major influence on haemodynamic in children. Therefore, it is frequently asymptomatic and accidentally found during catheterization and imaging modalities. It has the potential to mimic pulmonary disease, especially in individuals who do not have a congenital cardiac anomaly. We report the case of a five-year-old boy who had recurrent pneumonia with persistent hypoxaemia and was discovered to have persistent left superior vena cava into the left atrium by computer tomography pulmonary angiography. Due to the lifetime risk of emboli and bacteraemia from right to left shunting, the left superior vena cava must be terminated either percutaneously or surgically. [ABSTRACT FROM AUTHOR]

Background: A peculiar subgroup of patients with partial or complete atrioventricular canal defect exhibits a spectrum of left‐sided obstructions including right ventricular dominance and aortic coarctation. The association of atrioventricular canal defect with left‐sided obstructions is found in several genetic syndromes; however, the molecular basis of nonsyndromic atrioventricular canal defect with aortic coarctation is still poorly understood. Although some candidate genes for nonsyndromic atrioventricular canal defect are known, a complex oligogenic inheritance determined in some cases by the co‐occurrence of multiple variants has also been hypothesized. Case Report: We describe a nonsyndromic infant with mesocardia with viscero‐atrial situs solitus, partial atrioventricular canal defect, mild right ventricular dominance, and coarctation of the aorta. Next generation sequencing genetic testing revealed variants in two genes, GDF1 and NOTCH1, previously reported in association with atrioventricular canal defect and left‐sided obstructive lesions, respectively. Conclusion: The present report could support the hypothesis that the co‐occurrence of cumulative variants may be considered as genetic predisposing risk factor for specific congenital heart defects. [ABSTRACT FROM AUTHOR]

Sotos syndrome is an autosomal dominant condition characterized by overgrowth with advanced bone age, macrodolicocephaly, motor developmental delays and learning difficulties, and characteristic facial features caused by heterozygous pathogenetic variants in the NSD1 gene located on chromosome 5q35. The prevalence of heart defects (HDs) in individuals with Sotos syndrome is estimated to be around 15–40%. Septal defects and patent ductus arteriosus are the most commonly diagnosed malformations, but complex defects have also been reported. The aim of our study was to analyze the prevalence of HD, the anatomic types, and the genetic characteristics of 45 patients with Sotos syndrome carrying pathogenetic variants of NSD1 or a 5q35 deletion encompassing NSD1, who were followed at Bambino Gesù Children's Hospital in Rome. Thirty-nine of the forty-five patients (86.7%) had a mutation in NSD1, while six of the forty-five (13.3%) had a deletion. Most of the patients (62.2%, 28/45) were male, with a mean age of 14 ± 7 years (range 0.2–37 years). A total of 27/45 (60.0%) of the patients had heart defects, isolated or combined with other defects, including septal defects (12 patients), aortic anomalies (9 patients), mitral valve and/or tricuspid valve dysplasia/insufficiency (1 patient), patent ductus arteriosus (3 patients), left ventricular non-compaction/hypertrabeculated left ventricle (LV) (4 patients), aortic coarctation (1 patient), aortopulmonary window (1 patient), and pulmonary valve anomalies (3 patients). The prevalences of HD in the two subgroups (deletion versus intragenic mutation) were similar (66.7% (4/6) in the deletion group versus 58.91% (23/39) in the intragenic variant group). Our results showed a higher prevalence of HD in patients with Sotos syndrome in comparison to that described in the literature, with similar distributions of patients with mutated and deleted genes. An accurate and detailed echocardiogram should be performed in patients with Sotos syndrome at diagnosis, and a specific cardiological follow-up program is needed. [ABSTRACT FROM AUTHOR]

Purpose: Our aim in this study was to investigate the prenatal and postnatal prognosis of double outlet right ventricle (DORV) cases diagnosed prenatally by analyzing the outcomes based on the subtype. Methods: This study is a retrospective chart review. Cases diagnosed with fetal DORV by prenatal ultrasound in the maternal–fetal medicine department of our hospital between 2014 and 2022 were included. Data on maternal characteristics, fetal echocardiographic features (type of DORV), pregnancy and neonatal outcomes (termination of pregnancy [TOP], intrauterine fetal death [IUD], neonatal death [NND], death in infancy (IND), survival) were collected and analyzed. Results: Ninety‐nine cases of prenatally diagnosed cases of DORV were included. The prenatal diagnosis was right in 97% of the liveborn fetuses. The cases were classified into subtypes, including transposition of great arteries (TGA), Fallot, ventricular septal defect (VSD), remote, and heterotaxy types. The cohort consisted of 32.3% TGA type, 19.1% fallot type, 11.1% VSD type, 2% remote type, and 35.3% heterotaxy type of DORV. An additional cardiac anomaly was observed in 87% and an extra‐cardiac anomaly was observed in 54% of the cases. When we excluded the cases with heterotaxy type but without any chromosomal abnormality, additional genetic abnormalities were detected in 42% of the remaining cases. Outcome of pregnancy was livebirth in 68/99 (68.7%), IUFD in 5/99 (5.1%), and TOP in 26/99 (26.3%). Postnatal cardiac surgical repair was performed in 48 cases. Survival among livebirths was 39/68 (57.3%). Twenty‐nine neonates or infants who had additional cardiac anomalies and/or genetic abnormalities died before any surgical intervention. The postoperative survival rate was 39/48 (81.2%). Conclusion: The prognosis in DORV depends on the anatomical subtype, the presence, and severity of associated anomalies. Survival increases in isolated cases without any additional structural or genetic anomalies. [ABSTRACT FROM AUTHOR]

Heart failure results in significant morbidity and mortality for young children with hypoplastic left heart syndrome (HLHS) following the Norwood procedure. The trajectory in later childhood is not well described. We studied the outcome into adolescence of participants enrolled in the Single Ventricle Reconstruction trial who underwent the Fontan procedure or survived to 6 years without having undergone Fontan procedure. The primary outcome was heart failure events, defined as heart transplant listing or death attributable to heart failure. Symptomatic heart failure for participants surviving 10 or more years was also assessed utilizing the Pediatric Quality of Life Inventory (PedsQL). Of the 345 participants who underwent a Fontan operation or survived to 6 years without Fontan, 25 (7.2%) had a heart failure event before the age of 12 years. Among these, 21 were listed for heart transplant, and 4 died from heart failure. Nineteen participants underwent heart transplant, all of whom survived to age 12 years. Factors associated with a heart failure event included longer Norwood hospital length of stay, aortic atresia, and no Fontan operation by age 6 years. Assessment of heart failure symptoms at 12 years of age revealed that 24 (12.2%) of 196 PedsQL respondents "often" or "almost always" had difficulty walking more than one block. Heart failure events occur in over 5% of children with palliated HLHS between preschool age and adolescence. Outcomes for children listed for transplant are excellent. However, a substantial portion of palliated HLHS children have significant symptoms of heart failure at 12 years of age. [ABSTRACT FROM AUTHOR]

Aim: This study aimed to develop a model to help parents cope with decisional conflict. Background: Parents of children with congenital heart defect experience decisional conflict when they are uncertain about treatment decisions for their child, which may lead to delay in seeking care or distress over the decision made. Design: Correlational design with model building and data triangulation was used. Methods: Data were collected through surveys and interviews with a consecutive sample of 221 parent respondents from June to December 2018. Structural equation modelling and qualitative data analysis were used. Results: Lower decisional conflict was seen in parents with higher income, more nurse support and physician risk communication. Time delay for surgery was correlated with the child's age, social service coverage, and social support. Decisional conflict mediated the influence of income, nurse support and physician risk communication on satisfaction with decision. Based on model fit parameters, the emerging model is a good and parsimonious model of decisional conflict. The overall theme, 'Deciding for Surgery: What Matters Most', described the processes parents went through in making treatment decisions. Conclusion: Nurses may help parents feel more certain, less conflicted, and more satisfied with their decision by addressing factors including knowledge gaps, personal values, available support, and resource access. Summary statement: What is already known about this topic? Decisional conflict occurs when parents are uncertain of the best action for their child because treatment options entail risks for undesirable outcomes, value compromise, unclear prognosis, or anticipated regret over the decision.Professional support, information, and communication are vital to parents' decision‐making process.Child, parent, and support factors may influence parent treatment decisions in varied paediatric conditions. What this paper adds? Child's age at the time of decision‐making had an effect on the delay in surgery. However, assisting parents to weigh their options and focus on personal values allowed them to make their treatment decision.Low income, costly health services, and bureaucratic processes impeded the timely availment of surgery. Healthcare reforms that provide a system of government funding, streamlined health structure, and social insurance may be looked into.Family support weighed in on the parents' decision to delay surgery for fear of blame or guilt. Providing relevant information enabled parents to make a choice and stand by their decision regardless of the outcome. The implications of this paper: Modelling provides a framework to identify which factors are more important and how they interact to affect decision‐making. It may be used as an approach to find solutions to clinical problems for groups with different diagnoses.Though nursing support, information, and communication are essential, a more holistic family nursing care approach may be considered to assist parents to make appropriate treatment decisions for their child.Familiarity with the healthcare system may equip nurses to aid parents in processing available healthcare funding and dealing with financial uncertainty affecting decisions for their child's treatment. [ABSTRACT FROM AUTHOR]

To assess the safety and effectiveness of utilizing eccentric occlusion for the treatment of sub-arterial ventricular septal defects, we performed a retrospective study on the classification and analysis of relevant cases. A total of 105 patients with a minimally invasive incision were enrolled in this study, from April 2018 to September 2022. All patients underwent treatment of transthoracic sub-arterial ventricular septal defect occlusion. We analyzed the causes of closure failure, indication, and complication. Briefly, the closure device was successfully implanted in 78 cases (74.2%) with a mean age of 31.4 ± 31.8 months, VSD size of 4.3 ± 0.9 mm, and device size of 6.0 ± 1.1 mm. However, 27 cases (25.8%) required cardiopulmonary bypass due to failure of occlusion. The reasons for failure included 13 cases with worsened aortic regurgitation, two cases with worsened aortic valve prolapse, one case with worsened mitral regurgitation, eight cases with significant residual shunt, and three cases with deviated occlusion morphology. During the 1–36-month follow-up visit, no cases experienced displacement of the eccentric umbrella, shedding, or arrhythmia. All residual shunts resolved during the visit. We concluded that occlusion for sub-arterial VSD has sufficient security and feasibility, under the strict control of surgical indications, appropriate choice of occluder and precise perioperative management. [ABSTRACT FROM AUTHOR]

Objectives: To ascertain whether abnormalities in neonatal head circumference and/or body weight are associated with levels of angiogenic/antiangiogenic factors in the maternal and cord blood of pregnancies with a congenital heart defect (CHD) and to assess whether the specific type of CHD influences this association. Methods: This was a multicenter case–control study of women carrying a fetus with major CHD. Recruitment was carried out between June 2010 and July 2018 at four tertiary care hospitals in Spain. Maternal venous blood was drawn at study inclusion and at delivery. Cord blood samples were obtained at birth when possible. Placental growth factor (PlGF), soluble fms‐like tyrosine kinase‐1 (sFlt‐1) and soluble endoglin (sEng) were measured in maternal and cord blood. Biomarker concentrations in the maternal blood were expressed as multiples of the median (MoM). Results: PlGF, sFlt‐1 and sEng levels were measured in the maternal blood in 237 cases with CHD and 260 healthy controls, and in the cord blood in 150 cases and 56 controls. Compared with controls, median PlGF MoM in maternal blood was significantly lower in the CHD group (0.959 vs 1.022; P < 0.0001), while median sFlt‐1/PlGF ratio MoM was significantly higher (1.032 vs 0.974; P = 0.0085) and no difference was observed in sEng MoM (0.981 vs 1.011; P = 0.4673). Levels of sFlt‐1 and sEng were significantly higher in cord blood obtained from fetuses with CHD compared to controls (mean ± standard error of the mean, 447 ± 51 vs 264 ± 20 pg/mL; P = 0.0470 and 8.30 ± 0.92 vs 5.69 ± 0.34 ng/mL; P = 0.0430, respectively). Concentrations of sFlt‐1 and the sFlt‐1/PlGF ratio in the maternal blood at study inclusion were associated negatively with birth weight and head circumference in the CHD group. The type of CHD anomaly (valvular, conotruncal or left ventricular outflow tract obstruction) did not appear to alter these findings. Conclusions: Pregnancies with fetal CHD have an antiangiogenic profile in maternal and cord blood. This imbalance is adversely associated with neonatal head circumference and birth weight. © 2023 International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Transposition of the great arteries (TGA) is a conotruncal abnormality characterized by discordant ventriculoarterial alignments, with the aorta arising from the right ventricle and the pulmonary artery arising from the left ventricle and resultant parallel circulation of the systemic and pulmonary circuits. TGA can be dextro-TGA or levo-TGA based on the position of the aortic valve, right or left, regarding the pulmonary valve, and simple or complex TGA based on associated cardiac anomalies. With an incidence of 0.2-0.3/1,000 live births, TGA is the secondmost common cyanotic heart disease following the tetralogy of Fallot. Around 98% of TGA cases have a normal four-chamber view (4CV). The International Society of Ultrasound in Obstetrics and Gynecology and the American Institute of Ultrasound in Medicine added evaluation of the outflow tracts, three-vessel view (3VV), and three-vessel trachea view (3VTV) which improved the sensitivity of ultrasound screening for congenital heart disease (CHD). However, the prenatal detection rate of TGA, especially simple TGA, is generally low internationally, ranging from 25 to 50%. Without treatment, 50% of these infants die within the 1st month and 90% within the 1st year of life. The prenatal diagnosis of TGA improves postnatal outcomes through planned delivery at a tertiary care center and timely perinatal management. Immediately after birth, prostaglandin-E1 is given to keep the ductus arteriosus open to allow the mixing of oxygen-rich and oxygen-poor blood. If necessary, an atrial septostomy can be performed for short-term survival. Arterial switch operation (ASO) restores normal pulmonary and systemic circulation, with a 20-year survival rate of 90-97%. [ABSTRACT FROM AUTHOR]

bnormalities in different regions of Iran, most of which were conducted on live births. This study was conducted to determine fetal abnormalities in pregnancies leading to legal abortion in Golestan province. Methods: This descriptive-analytical study was conducted on 199 pregnant women with a gestational age of less than 20 weeks with abortion licenses from the Forensic Medicine Center of Golestan province over 9 months during 2018-19. Demographic characteristics, including parents’ age, ethnicity, and family relationship, and the type of fetal abnormalities were recorded. Results: Given 29,460 births in Golestan province over a 9-month period, the prevalence of fetal abnormalities was determined to be 6.75 per thousand births. The incidence rates of fetal abnormalities were determined to be 6.78, 6.68, 7.69, and 5.65 per thousand births in native Fars (80 cases), Turkmen (65 cases), Sistani (34 cases), and other (20 cases) ethnic groups, respectively. Since the gender of 80 fetuses was unknown, the incidence rates of abnormality were determined to be 4.36 and 3.72 per thousand births in female (63 cases) and male (56 cases) fetuses, respectively. The most common fetal abnormalities included central nervous system (n=49, 24.62%), chromosomal abnormalities (n=47, 23.61%), and cardiovascular impairments (n=26, 13.06%). The incidence of fetal abnormalities was not found to have a statistically significant relationship with parents' age, ethnicity, and family relationship. Conclusion: The most prevalent fetal abnormality was related to central nervous system disorders. The incidence of fetal abnormalities had no relationship with the parents’ age, ethnicity, and family relationship. [ABSTRACT FROM AUTHOR]

Aim: Our aim was to assess undiagnosed congenital heart defects (CHD) after newborns' hospital discharge in patients with a murmur or CHD suspicion, to find out the signs that predict CHDs and to estimate the costs of the examinations. Methods: We reviewed retrospective medical records of patients (n = 490) referred for the evaluation of CHD suspicion during 2017–2018. Results: The median age of the patients was 2.5 (IQR 0.5–7.4) years. Sixty‐three (13%) patients had an abnormal echocardiography. Neither ductal‐dependent nor cyanotic CHDs were found. Cardiac interventions were performed for 14 out of 63 (22%) patients. Clinical signs indicating CHDs were murmur grade ≥3 (10/11 [91%] vs. 53/479 [11%], p < 0.001) and harsh murmur (15/44 [34%] vs. 48/446 [11%], p < 0.001). Abnormal electrocardiography did not indicate CHD (8/40 [20%] vs. 55/447 [12%], p = 0.165). The total cost of the examinations was 259 700€. The share of the cost of studies assessed as benign was 59%. Conclusion: Only a few CHDs were found after newborn hospital discharge among patients who received foetal and newborn screening and were examined due to CHD suspicion. The high number of benign murmurs in children leads to many referrals, resulting in unnecessary healthcare costs. [ABSTRACT FROM AUTHOR]

Background: Neonates with critical congenital heart defects (CCHD neonates) experience high rates of feeding intolerance, necrotizing enterocolitis (NEC), and malnutrition. The benefits of human milk and direct chest/breastfeeding are well known, but research is limited in CCHD neonates. Therefore, the purpose of this study is to examine the impact of neonatal diet and feeding modality on the incidence of feeding intolerance, NEC, and malnutrition among a cohort of CCHD neonates. Methods: A single-center retrospective study was conducted using electronic health record data of CCHD neonates admitted to a cardiac intensive care unit between April 2016 and April 2020. Regression models were fit to analyze associations between neonatal diet, feed modality, and adverse feeding outcomes. Results: Seventy-four CCHD neonates were included. Increased days of direct chest/breastfeeding were associated with fewer signs of gastrointestinal distress (P = .047) and bloody stools (P = .021). Enteral feeding days of "all human milk" were associated with higher growth trajectory (P < .001). Conclusions: Human milk and direct chest/breastfeeding may be protective against some adverse feeding outcomes for CCHD neonates. Larger, multicenter cohort studies are needed to continue investigating the effects of neonatal diet type and feeding modality on the development of adverse feeding outcomes in this unique population. [ABSTRACT FROM AUTHOR]

Copyright of Turkiye Klinikleri Journal of Nursing Sciences 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.)

Copyright of Gyermekgyógyászat is the property of Semmelweis Kiado 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.)

Copyright of Gyermekgyógyászat is the property of Semmelweis Kiado 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: Interrupted aortic arch (IAA) is a rare congenital heart disease characterized by loss of continuity between the ascending and the descending aorta. Prenatal diagnosis of IAA by echocardiography is challenging but nonetheless can be accomplished via a systematization of cardiac fetal evaluation. Case Presentation: We report a case of fetal IAA type A prenatally diagnosed through two‐dimensional echocardiography using both a three vessel‐trachea view and a sagittal view. Conclusion: Prenatal counseling regarding the diagnosis and prognosis about this anomaly is still challenging nowadays due to associated anomalies/chromosomal abnormalities who may impact the prognosis. Fetal autopsy in all cases of pregnancy termination after abnormal ultrasound findings is important in order to make a full diagnosis and characterize the anomaly. [ABSTRACT FROM AUTHOR]

Objectives: Early extubation applications after pediatric congenital heart surgery have increased in recent years because of the positive results of these studies. It remains controversial whether early extubation should be performed in the operating room or in the intensive care unit. In addition, there are differences between hospitals in early extubation practices after pediatric cardiac surgery. In this study, we aimed to contribute to the literature by presenting our first 12 cases of infants extubated in the operating room after congenital heart surgery. Materials and Methods: Between May 2022 and December 2022, this study included the first 12 infant patients aged ≤1 year who underwent congenital heart surgery and were early extubate in the operating room. Results: The cohort comprised 6 girls and 6 boys, with an 11-month-old male patient presenting with pulmonary hypertension. Seven patients underwent cardiopulmonary bypass, with temperatures set at 32 °C for 4 patients and 34 °C for 3 patients. No respiratory problems occurred in the patients. None of the infants required non-invasive ventilation. There was no need for reintubation in the early or late period. Conclusion: Our findings suggest that a significant proportion of infant patients can be safely extubate in the operating room following congenital heart surgery. Early extubation offers the potential to decrease the need for sedation and inotropes, thereby averting complications associated with mechanical ventilation. [ABSTRACT FROM AUTHOR]

Background Socioeconomic factors may lead to a disproportionate impact on health care usage and death among individuals with congenital heart defects (CHD) by race, ethnicity, and socioeconomic factors. How neighborhood poverty affects racial and ethnic disparities in health care usage and death among individuals with CHD across the life span is not well described. Methods and Results Individuals aged 1 to 64 years, with at least 1 CHD‐related International Classification of Diseases, Ninth Revision, Clinical Modification (ICD‐9‐CM) code were identified from health care encounters between January 1, 2011, and December 31, 2013, from 4 US sites. Residence was classified into lower‐ or higher‐poverty neighborhoods on the basis of zip code tabulation area from the 2014 American Community Survey 5‐year estimates. Multivariable logistic regression models, adjusting for site, sex, CHD anatomic severity, and insurance‐evaluated associations between race and ethnicity, and health care usage and death, stratified by neighborhood poverty. Of 31 542 individuals, 22.2% were non‐Hispanic Black and 17.0% Hispanic. In high‐poverty neighborhoods, non‐Hispanic Black (44.4%) and Hispanic (47.7%) individuals, respectively, were more likely to be hospitalized (adjusted odds ratio [aOR], 1.2 [95% CI, 1.1–1.3]; and aOR, 1.3 [95% CI, 1.2–1.5]) and have emergency department visits (aOR, 1.3 [95% CI, 1.2–1.5] and aOR, 1.8 [95% CI, 1.5–2.0]) compared with non‐Hispanic White individuals. In high poverty neighborhoods, non‐Hispanic Black individuals with CHD had 1.7 times the odds of death compared with non‐Hispanic White individuals in high‐poverty neighborhoods (95% CI, 1.1–2.7). Racial and ethnic disparities in health care usage were similar in low‐poverty neighborhoods, but disparities in death were attenuated (aOR for non‐Hispanic Black, 1.2 [95% CI=0.9–1.7]). Conclusions Racial and ethnic disparities in health care usage were found among individuals with CHD in low‐ and high‐poverty neighborhoods, but mortality disparities were larger in high‐poverty neighborhoods. Understanding individual‐ and community‐level social determinants of health, including access to health care, may help address racial and ethnic inequities in health care usage and death among individuals with CHD.

AimPulmonary artery banding serves as an important palliative procedure used for the management of several congenital heart lesions. This study aims to describe a 20-year experience of pulmonary artery banding at a tertiary care center in a developing country.MethodsThis is a retrospective chart review of patients who underwent pulmonary artery banding over a 20-year period between January 2000 and July 2020 in a tertiary care center in a developing country. Data regarding demographics, indications, diagnosis, echocardiographic findings, postoperative complications, hospital stay, and outcomes were recorded.ResultsA total of 143 patients underwent pulmonary artery banding between 2000 and 2020, with a decrease from approximately 15 surgeries per year in 2012 to 1–2 surgeries a year in 2020. At the time of banding, the median age of patients was approximately 90 days [interquartile range, IQR, 30–150 days]. Four patients (2.8%) died during the band placement. No significant association was observed between baseline characteristics or type of heart defect at presentation and postoperative morbidity and mortality.ConclusionPulmonary artery banding remains useful in a subset of congenital heart lesions and as a surgical palliation, with relatively low mortality, allowing postponement of total correction to a higher weight. This technique continues to be valuable in developing countries or for heart surgical programs with limited resources.

Right aortic arch with isolation of left brachiocephalic artery is a rare congenital aortic arch anomaly. Herein, we reported a case of this rare anomaly with ventricular septal defect in a 9-month-old infant. We successfully reconstructed the islolated left brachiocephalic artery and repaired the ventricular septal defect in one stage.

OBJECTIVE: Hypoplastic left heart syndrome (HLHS) is a congenital disease characterized by an underdevelopment of the anatomical components inside the left heart. Approximately 30% of HLHS newborns will develop tricuspid regurgitation (TR), and it is currently unknown how the valve annulus mechanics and geometry are associated with regurgitation. Thus, we present an engineering mechanics-based analysis approach to quantify the mechanics and geometry of the HLHS-afflicted tricuspid valve (TV), using 4-dimensional echocardiograms. METHODS: Infants born with hypoplastic left heart syndrome (n=8) and healthy newborns (n=4) had their tricuspid valves imaged, and the data was imported to the 3D Slicer. The annular curves were defined at five points in the cardiac cycle. The geometry and deformation (strain) of the TV annulus were calculated to elucidate the mechanics of this critical structure, and compare them between HLHS and normal neonates. RESULTS: For the annular geometry, HLHS-afflicted newborns had significantly larger annular circumferences (20-30%) and anterior-posterior diameters (35-45%) than the healthy patients. From a biomechanics perspective, the HLHS patients had significantly smaller strains in the anterior segments (-0.1±2.6%) during end diastolic and end isovolumetric relaxation (1.7±3.0%) compared to the healthy counterparts (-13.3±2.9% and 6.8±0.9%, respectively). CONCLUSIONS: The image-based analysis in this study may provide novel insights into the geometric and mechanistic differences in the TV annulus between healthy and HLHS newborns. Future longitudinal studies of the biomechanics of TV annulus and other subvalvular structures may inform our understanding of the initiation and development of TR and the design of optimal repairs in this challenging population.

Background and Objective: In recent years, studies have been conducted to determine the prevalence rate of congenital abnormalities in different regions of Iran, most of which were conducted on live births. This study was conducted to determine fetal abnormalities in pregnancies leading to legal abortion in Golestan province. Methods: This descriptive-analytical study was conducted on 199 pregnant women with a gestational age of less than 20 weeks with abortion licenses from the Forensic Medicine Center of Golestan province over 9 months during 2018-19. Demographic characteristics, including parents’ age, ethnicity, and family relationship, and the type of fetal abnormalities were recorded. Results: Given 29,460 births in Golestan province over a 9-month period, the prevalence of fetal abnormalities was determined to be 6.75 per thousand births. The incidence rates of fetal abnormalities were determined to be 6.78, 6.68, 7.69, and 5.65 per thousand births in native Fars (80 cases), Turkmen (65 cases), Sistani (34 cases), and other (20 cases) ethnic groups, respectively. Since the gender of 80 fetuses was unknown, the incidence rates of abnormality were determined to be 4.36 and 3.72 per thousand births in female (63 cases) and male (56 cases) fetuses, respectively. The most common fetal abnormalities included central nervous system (n=49, 24.62%), chromosomal abnormalities (n=47, 23.61%), and cardiovascular impairments (n=26, 13.06%). The incidence of fetal abnormalities was not found to have a statistically significant relationship with parents' age, ethnicity, and family relationship. Conclusion: The most prevalent fetal abnormality was related to central nervous system disorders. The incidence of fetal abnormalities had no relationship with the parents’ age, ethnicity, and family relationship.

Abstract Background Pulmonary regurgitation is the most common complication after the complete repair of tetralogy of Fallot, and severe pulmonary regurgitation after surgery requires pulmonary valve replacement. In this retrospective observational, cross-sectional study, we included a total of 56 children aged 6 years or younger who underwent complete repair of TOF at Shahid Rajaei Cardiovascular Medical and Research Center in Tehran, Iran. Preoperative dual-source computed tomography was used to measure the McGoon ratio and Nakata index. The patients were divided into two groups based on the severity of postoperative pulmonary regurgitation, as estimated by trans-thoracic echocardiography: the severe pulmonary regurgitation group and the non-severe pulmonary regurgitation group. The McGoon ratio and Nakata index were then compared between the two groups. Results When comparing the two groups, we found that the corrected right pulmonary artery diameter, main pulmonary artery diameter, and McGoon ratio in the non-severe pulmonary regurgitation group were higher than in the severe pulmonary regurgitation group. However, none of these differences were statistically significant. Additionally, other variables, including the corrected left pulmonary artery diameter and Nakata index, showed higher measurements in children with severe pulmonary regurgitation, but again, the differences were not statistically significant. Conclusions This study indicates that pulmonary arteries diameter, Nakata index, and McGoon ratio were not significantly correlated with the severity of pulmonary regurgitation after the complete repair of tetralogy of Fallot.

Introduction: The objectives of this study were to assess the role of neuromarkers like glial fibrillary acidic protein (GFAP), brain-derived neurotrophic factor (BDNF), protein S100 (pS100), and neuron-specific enolase (NSE) as diagnostic markers of acute brain injury and also as prognostic markers for short-term neurodevelopmental impairment. Methods: Pediatric patients with congenital heart defects (CHDs) undergoing elective cardiac surgery were included. Neurodevelopmental functioning was assessed preoperatively and 4–6 months postoperatively using the Denver Developmental Screening Test II. Blood samples were collected preoperatively and postoperatively. During surgery, regional cerebral tissue oxygen saturation was monitored using near-infrared spectroscopy (NIRS). Results: Forty-two patients were enrolled and dichotomized into cyanotic and non-cyanotic groups based on peripheric oxygen saturation. Nineteen patients (65.5%) had abnormal developmental scores in the non-cyanotic group and eleven (84.6%) in the cyanotic group. A good diagnostic model was observed between NIRS values and GFAP in the cyanotic CHD group (AUC = 0.7). A good predicting model was observed with GFAP and developmental scores in the cyanotic CHD group (AUC = 0.667). A correlation was found between NSE and developmental quotient scores (r = 0.09, p = 0.046). Conclusions: From all four neuromarkers studied, only GFAP was demonstrated to be a good diagnostic and prognostic factor in cyanotic CHD patients. NSE had only prognostic value.

Abstract Background Congenital heart defects (CHDs) affect approximately half of individuals with Down syndrome (DS), but the molecular reasons for incomplete penetrance are unknown. Previous studies have largely focused on identifying genetic risk factors associated with CHDs in individuals with DS, but comprehensive studies of the contribution of epigenetic marks are lacking. We aimed to identify and characterize DNA methylation differences from newborn dried blood spots (NDBS) of DS individuals with major CHDs compared to DS individuals without CHDs. Methods We used the Illumina EPIC array and whole-genome bisulfite sequencing (WGBS) to quantitate DNA methylation for 86 NDBS samples from the California Biobank Program: (1) 45 DS-CHD (27 female, 18 male) and (2) 41 DS non-CHD (27 female, 14 male). We analyzed global CpG methylation and identified differentially methylated regions (DMRs) in DS-CHD versus DS non-CHD comparisons (both sex-combined and sex-stratified) corrected for sex, age of blood collection, and cell-type proportions. CHD DMRs were analyzed for enrichment in CpG and genic contexts, chromatin states, and histone modifications by genomic coordinates and for gene ontology enrichment by gene mapping. DMRs were also tested in a replication dataset and compared to methylation levels in DS versus typical development (TD) WGBS NDBS samples. Results We found global CpG hypomethylation in DS-CHD males compared to DS non-CHD males, which was attributable to elevated levels of nucleated red blood cells and not seen in females. At a regional level, we identified 58, 341, and 3938 CHD-associated DMRs in the Sex Combined, Females Only, and Males Only groups, respectively, and used machine learning algorithms to select 19 Males Only loci that could distinguish CHD from non-CHD. DMRs in all comparisons were enriched for gene exons, CpG islands, and bivalent chromatin and mapped to genes enriched for terms related to cardiac and immune functions. Lastly, a greater percentage of CHD-associated DMRs than background regions were differentially methylated in DS versus TD samples. Conclusions A sex-specific signature of DNA methylation was detected in NDBS of DS-CHD compared to DS non-CHD individuals. This supports the hypothesis that epigenetics can reflect the variability of phenotypes in DS, particularly CHDs.

Objective: The aim of this study was to assess glial fibrillary acidic protein (GFAP) as a marker of short-term neurodevelopmental delay in pediatric patients with congenital defects (CHD) after cardiovascular surgical intervention.

Background: Transposition of the great arteries (TGA) is a common critical neonatal congenital heart defect. After birth, physiological shunts close rapidly, necessitating early treatment with prostaglandin infusion and balloon-atrial septostomy. Timing of delivery is challenging, balancing the risks and advantages of early-term delivery and specialized care. The aim of this study is to assess the safety of a full-term delivery policy in fetuses diagnosed with TGA. Methods: A retrospective chart review was conducted of 17 women with a prenatal diagnosis of fetal TGA at Virgen del Rocío University Hospital between 2015 and 2021. Primary outcomes included: incidence of preterm, early-term, full-term, and late-term delivery, and rate of cesarean section. Secondary outcomes included: Saturday to Sunday admission and birth, and delivery between 0:00 a.m. and 8:00 a.m. Results: Full-term birth was achieved in 94.1%, reaching a low cesarean delivery rate (17.6%). A total of 82.4% of infants were born on weekdays, and only in three of the cases (17.6%) did delivery occur between 0 a.m. and 8 a.m. The median birth weight was 3300 g. Intravenous prostaglandins were administered in all cases, and 94.1% required balloon-atrial septostomy. Conclusions: In our study favoring full-term delivery, we reduce early-term deliveries and the cesarean section rate in prenatally diagnosed TGA.

Objective: This study investigated the safety of performing surgical repair for doubly committed ventricular septal defects by right vertical infra-axillary minithoracotomy (RVIAT). Methods: A retrospective comparative study was performed to evaluate the outcomes of patients who underwent doubly committed ventricular septal defects closure from January 2019 to May 2022. Seventy-four patients were enrolled in the study and treated with either the median sternotomy approach (MSA: n = 37) or the RVIAT approach (RVIAT: n = 37). Results: The median weight and age in the MSA group were significantly lower than those in the RVIAT group (MSA: 6.0 kg [interquartile range] (IQR), 5.2 to 8.7 kg] vs RVIAT: 7.5 kg [IQR, 5.6-14 kg]; P = .034 and MSA: 4.9 months [IQR, 3.6-9.4 month] vs 9.6 months [IQR, 5.0-60.4 months]; P = .0084). No patients died, and no patients in the RVIAT group required conversion to the MSA approach. The mean prebypass surgical time was longer in the RVIAT group (36.1 ± 8.2 minutes vs 31.8 ± 5.6 minutes; P = .03). There were no significant differences between the 2 groups in cardiopulmonary bypass time, aortic crossclamp time, or operation time. Significantly shorter ventilation times were observed in the RVIAT group (11.9 ± 8.2 hours vs 15.4 ± 6.3 hours; P = .006). Conclusions: Closure of doubly committed ventricular septal defects through the pulmonary trunk by the RVIAT approach is feasible and safe, and does not increase the risk of bypass-related complications.

We present a case of a full-term newborn with complex congenital heart defects, including single-ventricle physiology and discontinuous pulmonary arteries. Prompt surgical intervention was performed, which involved pulmonary neoconfluence with autologous pericardium graft and systemic-to-pulmonary shunt placement. However, postoperative complications required stenting to address pulmonary artery stenosis.

Coronary artery fistulas (CAFs) are rare coronary anomalies involving the communication of an epicardial coronary artery and another cardiovascular structure. CAFs are usually easily distinguished from nearby coronary arteries. Here, we report a unique case of CAF that mimics the size, branching pattern, and appearance of a native epicardial left anterior descending artery.

A very large fetal pericardial teratoma was diagnosed at 28 weeks’ gestation, prompting urgent multidisciplinary expert consultations to weigh the risks and benefits of various prenatal invasive procedures and preterm delivery for postnatal surgical management. Ultimately, the infant was born by planned cesarean section and underwent immediate cardiopulmonary bypass and surgical resection.

Left ventricular outflow tract obstruction (LVOTO), a major form of outflow tract malformation, accounts for a substantial portion of congenital heart defects (CHDs). Unlike its prevalence, the genetic architecture of LVOTOremains largely unknown. To unveil the genetic mutations and risk genes potentially associated with LVOTO, we enrolled a cohort of 106 LVOTO patients and 100 healthy controls and performed a whole-exome sequencing (WES). 71,430 rare deleterious mutations were found in LVOTO patients. By using gene-based burden testing, we further found 32 candidate genes enriched in LVOTO patient including known pathological genes such as GATA5 and GATA6. Most variants of 32 risk genes occur simultaneously rather exclusively suggesting polygenic inherence of LVOTO and 14 genes out of 32 risk genes interact with previously discovered CHD genes. Single cell RNA-seq further revealed dynamic expressions of GATA5, GATA6, FOXD3 and MYO6 in endocardium and neural crest lineage indicating the mutations of these genes lead to LVOTO possibly through different lineages. These findings uncover the genetic architecture of LVOTO which advances the current understanding of LVOTO genetics. [ABSTRACT FROM AUTHOR]

To explore the effect of a daily goal checklist on pediatric cardiac intensive care unit (PCICU) length of stay (LOS) after congenital heart surgery. This study is a prospective randomized single-center study. Group characteristics were as follows: STANDARD group: n = 30, 36.7% female, median age 0.9 years; control group: n = 33, 36.4% female, median age 1.1 years. Invasive ventilation time, STAT categories, mean vasoactive-inotropic score (VIS)24h, maximal (max.) VIS24h, mean VIS24–48h, max. VIS24–48h, VIS category, number of sedatives, analgesics, diuretics, number of deployed diagnostic modalities, morbidities, and mortality did not differ between both groups. Median PCICU LOS was 96.0 h (STANDARD group) versus 101.5 h (control group) (p = 0.63). In the overall cohort, univariate regression analysis identified age at surgery (b = −0.02), STAT category (b = 18.3), severity of CHD (b = 40.6), mean VIS24h (b = 3.5), max. VIS24h (b = 2.2), mean VIS24–48h (b = 6.5), and VIS category (b = 13.8) as significant parameters for prolonged PCICU LOS. In multivariate regression analysis, age at surgery (b = −0.2), severity of CHD (b = 44.0), and mean VIS24h (b = 6.7) were of significance. Within the STANDARD sub-group, univariate regression analysis determined STAT category (b = 32.3), severity of CHD (b = 70.0), mean VIS24h (b = 5.0), mean VIS24–48h (b = 5.9), number of defined goals (b = 2.6), number of achieved goals (b = 3.3), number of not achieved goals (b = 10.8), and number of unevaluated goals (b = 7.0) as significant parameters for prolonged PCICU LOS. Multivariate regression analysis identified the number of defined goals (b = 2.5) and the number of unevaluated goals (b = −3.0) to be significant parameters. Conclusion: The structured realization and recording of daily goals is of advantage in patients following pediatric cardiac surgery by reducing PCICU LOS. What is known: • Communication errors are the most frequent reasons for adverse events in intensive care unit patients. • Improved communication can be achieved by discussion and documentation of the patients' goals during daily rounds. What is new: • In the overall cohort age at surgery, severity of congenital heart defect and mean vasoactive inotropic score within the first 24 hours had significant impact on pediatric cardiac intensive care unit (PCICU) length of stay (LOS). • In the intervention group, the number of defined goals and the number of unevaluated goals were significant parameters for prolonged PCICU LOS. [ABSTRACT FROM AUTHOR]

Background: Pulmonary regurgitation is the most common complication after the complete repair of tetralogy of Fallot, and severe pulmonary regurgitation after surgery requires pulmonary valve replacement. In this retrospective observational, cross-sectional study, we included a total of 56 children aged 6 years or younger who underwent complete repair of TOF at Shahid Rajaei Cardiovascular Medical and Research Center in Tehran, Iran. Preoperative dual-source computed tomography was used to measure the McGoon ratio and Nakata index. The patients were divided into two groups based on the severity of postoperative pulmonary regurgitation, as estimated by trans-thoracic echocardiography: the severe pulmonary regurgitation group and the non-severe pulmonary regurgitation group. The McGoon ratio and Nakata index were then compared between the two groups. Results: When comparing the two groups, we found that the corrected right pulmonary artery diameter, main pulmonary artery diameter, and McGoon ratio in the non-severe pulmonary regurgitation group were higher than in the severe pulmonary regurgitation group. However, none of these differences were statistically significant. Additionally, other variables, including the corrected left pulmonary artery diameter and Nakata index, showed higher measurements in children with severe pulmonary regurgitation, but again, the differences were not statistically significant. Conclusions: This study indicates that pulmonary arteries diameter, Nakata index, and McGoon ratio were not significantly correlated with the severity of pulmonary regurgitation after the complete repair of tetralogy of Fallot. [ABSTRACT FROM AUTHOR]

An anomalous right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital anomaly that does not have the typical presentation of the more common anomalous left coronary artery. We present an infant with tetralogy of Fallot with atypical findings on the preoperative echocardiogram. A cardiac computerized tomographic (CT) scan showed ARCAPA. This was confirmed intraoperatively and repaired successfully. Close attention to coronaries on echocardiography and a low threshold for additional imaging can successfully diagnose ARCAPA in the presence of additional congenital heart defects. [ABSTRACT FROM AUTHOR]

Ventricular septal defects (VSD) represent the most common congenital heart defect in newborns. We assessed the electrocardiographic characteristics of newborns with VSDs in a general population sample. The Copenhagen Baby Heart Study is a prospective population–based cohort study offering cardiac evaluation of newborns. Echocardiograms and electrocardiograms were obtained within 30 days after birth and systematically analysed. A VSD was identified in 530 newborns (mean age 11 ± 7 days, 42% boys). Newborns with VSDs had a more left-shifted QRS axis (116 ± 34 vs. 120 ± 3°, p = 0.02), and a higher S-wave amplitude in V1 (721 ± 584 vs. 636 ± 549 µV, p = 0.001) than controls. The largest differences were found in newborns with large or perimembraneous VSDs with a higher frequency of left axis deviation, higher S-wave amplitudes in V1, and higher R- and S-wave amplitudes in V6 compared with controls. R-waves in V1 and V6 were significantly associated to left ventricular mass, whereas S-waves in V1 and V6 were dependent on left ventricular end-diastolic diameter on echocardiography. Conclusion: Newborns with VSDs showed significant differences in QRS axis, and R- and S-wave precordial amplitudes compared to matched controls. Perimembranous and large VSDs had the greatest effect on the neonatal ECG. What is Known: • Ventricular septal defects in newborns are prevalent and may affect cardiac function and structure. What is New: • The Copenhagen Baby Heart Study is the largest study including a cohort of unselected newborns undergoing postnatal cardiac examination. • We found that newborns with VSD showed significant electrocardiographic differences depending on size and type of VSD compared with healthy newborns. [ABSTRACT FROM AUTHOR]

Congenital heart defects (CHDs) are the most common neonatal malformations and are a leading cause of infant death in developed countries. Finding safe and effective diagnostic methods to screen for CHDs is important. The aim of this study was to evaluate the effectiveness of pulse oximetry (PO) and perfusion index (PI) in screening CHD. We conducted a systematic review of studies in PubMed, Embase, and the Cochrane Library published on or before October 1, 2021. Studies based on PICOS were included in this systematic review. The flow chart is made by PRISMA software. The quality of included studies was assessed by RevMan5 software (QUADAS-2: Quality Assessment of Diagnostic Accuracy Studies-2). The sensitivity, specificity, and other measurements of accuracy were pooled using Stata/SE 12.0 software. Five studies containing 46,965 neonates were included in this study. A randomized-effects model was used for the meta-analysis because of significant heterogeneity. The combined sensitivity and specificity were 0.82 (95% confidence interval [CI], 0.53–0.95) and 0.97 (95% CI, 0.57–1.00), respectively. The area under the curve was 0.92 (95% CI, 0.89–0.94). The combination PO and PI was significant in CHD screening. Once diagnosed by the combined method, it means that the neonate is most likely to have a CHD. Key Points Pulse oximetry and PI screening. Congenital heart defects. A systematic review and meta-analysis. [ABSTRACT FROM AUTHOR]

Objective: To investigate the national prevalence and prenatal detection rate (DR) of major congenital heart disease (mCHD) in twin pregnancies without twin‐to‐twin transfusion syndrome (TTTS)‐associated CHD in a Danish population following a standardized prenatal screening program. Methods: This was a national registry‐based study of data collected prospectively over a 10‐year period. In Denmark, all women with a twin pregnancy are offered standardized screening and surveillance programs in addition to first‐ and second‐trimester screening for aneuploidies and malformation, respectively: monochorionic (MC) twins every 2 weeks from gestational week 15 and dichorionic (DC) twins every 4 weeks from week 18. The data were retrieved from the Danish Fetal Medicine Database and included all twin pregnancies from 2009–2018, in which at least one fetus had a pre‐ and/or postnatal mCHD diagnosis. mCHD was defined as CHD requiring surgery within the first year of life, excluding ventricular septal defects. All pregnancy data were pre‐ and postnatally validated in the local patient files at the four tertiary centers covering the entire country. Results: A total of 60 cases from 59 twin pregnancies were included. The prevalence of mCHD was 4.6 (95% CI, 3.5–6.0) per 1000 twin pregnancies (1.9 (95% CI, 1.3–2.5) per 1000 live births). The prevalences for DC and MC were 3.6 (95% CI, 2.6–5.0) and 9.2 (95% CI, 5.8–13.7) per 1000 twin pregnancies, respectively. The national prenatal DR of mCHD in twin pregnancies for the entire period was 68.3%. The highest DRs were in cases with univentricular hearts (100%) and the lowest with aortopulmonary window, total anomalous pulmonary venous return, Ebstein's anomaly, aortic valve stenosis and coarctation of the aorta (0–25%). Mothers of children with prenatally undetected mCHD had a significantly higher body mass index (BMI) compared to mothers of children with a prenatally detected mCHD (median, 27 kg/m2 and 23 kg/m2, respectively; P = 0.02). Conclusions: The prevalence of mCHD in twins was 4.6 per 1000 pregnancies and was higher in MC than DC pregnancies. The prenatal DR of mCHD in twin pregnancies was 68.3%. Maternal BMI was higher in cases of prenatally undetected mCHD. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Objectives: Blood‐flow pattern is an essential factor in cardiovascular development. Recently, blood speckle‐tracking echocardiography (BST) based on high‐frame‐rate ultrasound has emerged as a promising technique for the assessment of blood‐flow patterns and properties. The objectives of this study were to determine the feasibility of BST in the fetus and to assess intracardiac blood‐flow patterns of fetuses with a congenital heart defect (CHD) using this technique. Methods: This was a prospective study consisting of 35 normal fetuses, 35 fetuses with left‐sided obstructive lesion (LSOL) and 35 fetuses with right‐sided obstructive lesion (RSOL). BST images of fetal intracardiac regions of interest (ROIs), including the left ventricle (LV), right ventricle (RV), ascending aorta (AAo), aortic arch (AA), descending aorta (DAo) and pulmonary artery (PA), were obtained and analyzed. The feasibility of BST was assessed, and blood‐flow pattern and number of vortices in the ROIs were recorded. Results: The median gestational age of the fetuses was 24.7 weeks (range, 19.6–34.3 weeks). BST was feasible in 81.6% of cases, and the cut‐off value of depth for an adequate BST image was ≤ 7.9 cm. There were no differences in the presence of vortex/turbulent blood flow in the LV or RV among the three groups. Vortex/turbulent blood flow in the AAo was detected in 0% (0/35), 14.3% (5/35) and 57.1% (20/35) of cases in the control, LSOL and RSOL groups, respectively. The respective values were 5.7% (2/35), 14.3% (5/35) and 51.4% (18/35) for the AA; 0% (0/35), 48.6% (17/35) and 0% (0/35) for the DAo; and 0% (0/35), 40.0% (14/35) and 51.4% (18/35) for the PA. With the exception of the DAo in the RSOL group, vortex/turbulent flow in the great artery ROIs was significantly more common in the LSOL and RSOL groups than in controls (P < 0.01). In the LSOL group, the number of vortices in the AAo, AA, DAo and PA was significantly greater compared with that in controls (P < 0.01). In the RSOL group, the number of vortices in the LV, AAo, AA and PA was significantly greater compared with that in controls (P < 0.01). Conclusions: Fetuses with CHD were more likely to exhibit vortex/turbulent blood flow and increased number of vortices in the great arteries compared with healthy controls. Further research is needed to determine the biomechanical effect of blood‐flow patterns, especially vortex flow, on fetal cardiovascular structure and function. © 2023 International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Interrupted aortic arch (IAA) is a rare congenital heart condition where there is a complete discontinuation between the ascending and descending aorta. The association with a patent ductus arteriosus or developed arterial collateral allows survival until adulthood in extremely rare cases. We report a case series of adult forms of IAA. Even if the incidence is very rare, IAA should be could excluded in the setting of resistant asymmetrical hypertension. The first case is singular regarding its association with a bicuspid aortic valve and aortic aneurysm, while the second case is characterized by massive left ventricular hypertrophy. The diagnosis was suspected on echocardiographic findings and confirmed by computed tomography angiography. Both patients refused surgical repair given the high operative risk. -The evaluation of severe resistant high blood pressure should include complementary investigation for adult form interrupted aortic arch. -To understand the major impact of multimodality imaging for the recognition, localization, and stratification of interrupted aortic arch. [ABSTRACT FROM AUTHOR]

Background: Research on the association between neighborhood social deprivation and health among adults with congenital heart defects (CHD) is sparse. Methods: We evaluated the associations between neighborhood social deprivation and health care utilization, disability, and comorbidities using the population‐based 2016–2019 Congenital Heart Survey To Recognize Outcomes, Needs, and well‐beinG (CH STRONG) of young adults. Participants were identified from active birth defect surveillance systems in three U.S. sites and born with CHD between 1980 and 1997. We linked census tract‐level 2017 American Community Survey information on median household income, percent of ≥25‐year‐old with greater than a high school degree, percent of ≥16‐year‐olds who are unemployed, and percent of families with children <18 years old living in poverty to survey data and used these variables to calculate a summary neighborhood social deprivation z‐score, divided into tertiles. Adjusted prevalence ratios (aPR) and 95% confidence intervals (CI) derived from a log‐linear regression model with a Poisson distribution estimated the association between tertile of neighborhood social deprivation and healthcare utilization in previous year (no encounters, 1 and ≥2 emergency room [ER] visits, and hospital admission), ≥1 disability, and ≥1 comorbidities. We accounted for age, place of birth, sex at birth, presence of chromosomal anomalies, and CHD severity in all models, and, additionally educational attainment and work status in all models except disability. Results: Of the 1435 adults with CHD, 43.8% were 19–24 years old, 54.4% were female, 69.8% were non‐Hispanic White, and 33.7% had a severe CHD. Compared to the least deprived tertile, respondents in the most deprived tertile were more likely to have no healthcare visit (aPR: 1.5 [95% CI: 1.1, 2.1]), ≥2 ER visits (1.6 [1.1, 2.3]), or hospitalization (1.6 [1.1, 2.3]) in the previous 12 months, a disability (1.2 [1.0, 1.5]), and ≥1 cardiac comorbidities (1.8 [1.2, 2.7]). Conclusions: Neighborhood social deprivation may be a useful metric to identify patients needing additional resources and referrals. [ABSTRACT FROM AUTHOR]

Introduction: The objectives of this study were to assess the role of neuromarkers like glial fibrillary acidic protein (GFAP), brain-derived neurotrophic factor (BDNF), protein S100 (pS100), and neuron-specific enolase (NSE) as diagnostic markers of acute brain injury and also as prognostic markers for short-term neurodevelopmental impairment. Methods: Pediatric patients with congenital heart defects (CHDs) undergoing elective cardiac surgery were included. Neurodevelopmental functioning was assessed preoperatively and 4–6 months postoperatively using the Denver Developmental Screening Test II. Blood samples were collected preoperatively and postoperatively. During surgery, regional cerebral tissue oxygen saturation was monitored using near-infrared spectroscopy (NIRS). Results: Forty-two patients were enrolled and dichotomized into cyanotic and non-cyanotic groups based on peripheric oxygen saturation. Nineteen patients (65.5%) had abnormal developmental scores in the non-cyanotic group and eleven (84.6%) in the cyanotic group. A good diagnostic model was observed between NIRS values and GFAP in the cyanotic CHD group (AUC = 0.7). A good predicting model was observed with GFAP and developmental scores in the cyanotic CHD group (AUC = 0.667). A correlation was found between NSE and developmental quotient scores (r = 0.09, p = 0.046). Conclusions: From all four neuromarkers studied, only GFAP was demonstrated to be a good diagnostic and prognostic factor in cyanotic CHD patients. NSE had only prognostic value. [ABSTRACT FROM AUTHOR]

Congenital heart defects (CHDs) are the most common birth defects. Recently, artificial intelligence (AI) was used to assist in CHD diagnosis. No comparison has been made among the various types of algorithms that can assist in the prenatal diagnosis. Normal and abnormal fetal ultrasound heart images, including five standard views, were collected according to the International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) Practice guidelines. You Only Look Once version 5 (YOLOv5) models were trained and tested. An excellent model was screened out after comparing YOLOv5 with other classic detection methods. On the training set, YOLOv5n performed slightly better than the others. On the validation set, YOLOv5n attained the highest overall accuracy (90.67 %). On the CHD test set, YOLOv5n, which only needed 0.007 s to recognize each image, had the highest overall accuracy (82.93 %), and YOLOv5l achieved the best accuracy on the abnormal dataset (71.93 %). On the VSD test set, YOLOv5l had the best performance, with a 92.79 % overall accuracy rate and 92.59 % accuracy on the abnormal dataset. The YOLOv5 models achieved better performance than the Fast region-based convolutional neural network (RCNN) & ResNet50 model and the Fast RCNN & MobileNetv2 model on the CHD test set (p<0.05) and VSD test set (p<0.01). YOLOv5 models are able to accurately distinguish normal and abnormal fetal heart ultrasound images, especially with respect to the identification of VSD, which have the potential to assist ultrasound in prenatal diagnosis. [ABSTRACT FROM AUTHOR]