Impact of preoperative respiratory distress on outcomes of slide tracheoplasty.

OBJECTIVES: Children with congenital tracheal stenosis born in the developing world face a high risk of mortality due to limited access to proper treatment. Patients who required preoperative respiratory support were suspected to have poor survival after slide tracheoplasty; however, this was not clearly demonstrated in the previous studies. This study aims to investigate the impact of preoperative respiratory conditions on outcomes of slide tracheoplasty. METHODS: From 2016 to 2022, children who underwent slide tracheoplasty were retrospectively reviewed. Patients with respiratory distress requiring emergency operations (group A) were compared with patients in stable condition who were scheduled for surgery (group B). RESULTS: Perioperative results revealed that group A (n = 43) had a longer bypass time (P < 0.001), operation time (P = 0.01), postoperative ventilation time (P < 0.001) and length of intensive care unit stay (P = 0.00125) than group B (n = 60). The early mortality rate was 7.8%, and the actuarial 5-year survival rate was 85.3%. The cumulative incidence test revealed that group A was highly significant for overall mortality [sudistribution (SHR) 4.5; 95% confidence interval (CI) 1.23-16.4; P = 0.023]. Risk factors for overall mortality were prolonged postoperative ventilation time (hazard ratio 3.86; 95% CI 1.20-12.48; P = 0.024), bronchial stenosis (hazard ratio 5.77; 95% CI 1.72-19.31; P = 0.004), and preoperative tracheal mucositis (hazard ratio 5.67; 95% CI 1.51-21.31; P = 0.01). Four patients needed reintervention during a follow-up of 28.4 months (interquartile range 15.3-47.3). CONCLUSIONS: Preoperative respiratory distress negatively affected the outcomes of patients who required slide tracheoplasty. Therefore, early detection of congenital tracheal stenosis and aggressive slide tracheoplasty are crucial and obligatory to enhance long-term survival in this lethal congenital airway disease.

Right-Sided Aortic Arch: A Computed Tomography Angiography Investigation, A Systematic Review with Meta-Analysis.

Background/Objectives: The right-sided aortic arch (RAA) is an uncommon variation of the aortic arch (AA), characterized by the aorta crossing over the right main bronchus. In the RAA, the descending aorta can be found on either the right or left side of the spine. The current study comprises a comprehensive retrospective computed tomography angiography (CTA) investigation into the prevalence of the RAA within the Greek population. Additionally, we will conduct a systematic review and meta-analysis to elucidate both common and rare morphological variants of the RAA. This research is significant as it sheds light on the prevalence and characteristics of the RAA in a specific population, providing valuable insights for clinical practice. Methods: Two hundred CTAs were meticulously investigated for the presence of a RAA. In addition, the PubMed, Google Scholar, and Scopus online databases were thoroughly searched for studies referring to the AA morphology. The R programming language and RStudio were used for the pooled prevalence meta-analysis, while several subgroup analyses were conducted. Results: Original study: A unique case of 200 CTAs (0.5%) was identified with an uncommon morphology. The following branches emanated from the RAA under the sequence: the right subclavian artery (RSA), the right common carotid artery (RCCA), the left common carotid artery (LCCA), and the left vertebral artery (LVA) in common origin with the aberrant left subclavian artery (ALSA). The ALSA originated from a diverticulum (of Kommerell) and followed a retroesophageal course. Systematic Review and Meta-Analysis: Sixty-two studies (72,187 total cases) met the inclusion criteria. The pooled prevalence of the RAA with a mirror-image morphology was estimated at 0.07%, and the RAA with an ALSA was estimated at <0.01%. Conclusions: AA anomalies, specifically the RAA, raise clinical interest due to their coexistence with developmental heart anomalies and possible interventional complications. Congenital heart anomalies, such as the Tetralogy of Fallot and patent foramen ovale, coexisted with RAA mirror-image morphology.

A Case of Double Inlet Left Ventricle in a 63-Year-Old Female Patient.

This case report describes the medical history and presentation of an elderly patient who was born with single ventricle physiology, an anomaly that is both unique and complex. Patients with single ventricle cardiac anomalies may be susceptible to life-threatening complications. However, advances in medical treatment and understanding have allowed for clinicians to develop surgical and medical interventions to treat patients with univentricular cardiac defects. This case is unique in the sense that the patient has been able to demonstrate remarkable adaptability to this condition and have a sustained life with little intervention. This report serves to explore the pathophysiology of this condition as well as highlight the human body's astounding resilience to configure itself to abnormal conditions. The patient's presentation will be discussed as well as diagnostics and management utilized by the care providers. Despite its rare occurrence, understanding the manifestations of this complex cardiac abnormality can allow future providers to improve the prognosis and outcomes of patients born with a single ventricle.

Clinical implications of dextrocardia based on four visceroatrial situs studies.

BACKGROUND: Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. METHODS: We retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs. RESULTS: Dextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage. CONCLUSION: Our study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.

Surgical Repair of Atrial Septal Defect in a Patient with 3-Methylcrotonyl-CoA Carboxylase Deficiency.

3-Methylcrotonyl-CoA carboxylase (3-MCC) deficiency is a rare autosomal recessive disease of leucine catabolism. 3-MCC deficiency may lead to metabolic decompensation under stress; however, outcomes of elective surgery requiring cardiopulmonary bypass (CPB) are unknown. We report a 4-year-old girl with asymptomatic 3-MCC deficiency and atrial septal defect (ASD) who's undergone surgical ASD repair under CPB. She was otherwise normal developmentally and medically. Although patients with 3-MCC may face metabolic crises, the ASD repair under CPB was uneventful.

Art or Artifact: Evaluating the Accuracy, Appeal, and Educational Value of AI-Generated Imagery in DALL·E 3 for Illustrating Congenital Heart Diseases.

Artificial Intelligence (AI), particularly AI-Generated Imagery, has the potential to impact medical and patient education. This research explores the use of AI-generated imagery, from text-to-images, in medical education, focusing on congenital heart diseases (CHD). Utilizing ChatGPT's DALL·E 3, the research aims to assess the accuracy and educational value of AI-created images for 20 common CHDs. In this study, we utilized DALL·E 3 to generate a comprehensive set of 110 images, comprising ten images depicting the normal human heart and five images for each of the 20 common CHDs. The generated images were evaluated by a diverse group of 33 healthcare professionals. This cohort included cardiology experts, pediatricians, non-pediatric faculty members, trainees (medical students, interns, pediatric residents), and pediatric nurses. Utilizing a structured framework, these professionals assessed each image for anatomical accuracy, the usefulness of in-picture text, its appeal to medical professionals, and the image's potential applicability in medical presentations. Each item was assessed on a Likert scale of three. The assessments produced a total of 3630 images' assessments. Most AI-generated cardiac images were rated poorly as follows: 80.8% of images were rated as anatomically incorrect or fabricated, 85.2% rated to have incorrect text labels, 78.1% rated as not usable for medical education. The nurses and medical interns were found to have a more positive perception about the AI-generated cardiac images compared to the faculty members, pediatricians, and cardiology experts. Complex congenital anomalies were found to be significantly more predicted to anatomical fabrication compared to simple cardiac anomalies. There were significant challenges identified in image generation. Based on our findings, we recommend a vigilant approach towards the use of AI-generated imagery in medical education at present, underscoring the imperative for thorough validation and the importance of collaboration across disciplines. While we advise against its immediate integration until further validations are conducted, the study advocates for future AI-models to be fine-tuned with accurate medical data, enhancing their reliability and educational utility.

The effect of 3D modeling on family quality of life, surgical success, and patient outcomes in congenital heart diseases: objectives and design of a randomized controlled trial.

BACKGROUND: Understanding the severity of the disease from the parents' perspective can lead to better patient outcomes, improving both the child's health-related quality of life and the family's quality of life. The implementation of 3-dimensional (3D) modeling technology in care is critical from a translational science perspective. AIM: The purpose of this study is to determine the effect of 3D modeling on family quality of life, surgical success, and patient outcomes in congenital heart diseases. Additionally, we aim to identify challenges and potential solutions related to this innovative technology. METHODS: The study is a two-group pretest-posttest randomized controlled trial protocol. The sample size is 15 in the experimental group and 15 in the control group. The experimental group's heart models will be made from their own computed tomography (CT) images and printed using a 3D printer. The experimental group will receive surgical simulation and preoperative parent education with their 3D heart model. The control group will receive the same parent education using the standard anatomical model. Both groups will complete the Sociodemographic Information Form, the Surgical Simulation Evaluation Form - Part I-II, and the Pediatric Quality of Life Inventory (PedsQL) Family Impacts Module. The primary outcome of the research is the average PedsQL Family Impacts Module score. Secondary outcome measurement includes surgical success and patient outcomes. Separate analyses will be conducted for each outcome and compared between the intervention and control groups. CONCLUSIONS: Anomalies that can be clearly understood by parents according to the actual size and dimensions of the child's heart will affect the preoperative preparation of the surgical procedure and the recovery rate in the postoperative period.

2024 SIAC guidelines on cardiorespiratory rehabilitation in pediatric patients with congenital heart disease.

The 2024 Interamerican Society of Cardiology (SIAC) guidelines on cardiorespiratory rehabilitation (CRR) in pediatric patients with congenital heart disease aim to gather and evaluate all relevant evidence available on the topic to unify criteria and promote the implementation of CRR programs in this population in Latin America and other parts of the world. Currently, there is no unified CRR model for the pediatric population. Consequently, our goal was to create these CRR guidelines adapted to the characteristics of congenital heart disease and the physiology of this population, as well as to the realities of Latin America. These guidelines are designed to serve as a support for health care workers involved in the care of this patient group who wish to implement a CRR program in their workplace. The guidelines include an easily reproducible program model that can be implemented in any center. The members of this Task Force were selected by the SIAC on behalf of health care workers dedicated to the care of pediatric patients with congenital heart disease. To draft the document, the selected experts performed a thorough review of the published evidence.

Congenital heart diseases (CHDs) and forensic investigations: Searching for the cause of death.

Congenital Heart Diseases (CHDs) are a group of structural abnormalities or defects of the heart that are present at birth. CHDs could be connected to sudden death (SD), defined by the WHO (World Health Organization) as "death occurring within 24 h after the onset of the symptoms" in an apparently "healthy" subject. These conditions can range from relatively mild defects to severe, life-threatening anomalies. The prevalence of CHDs varies across populations, but they affect millions of individuals worldwide. This article aims to discuss the post-mortem investigation of death related to CHDs, exploring the forensic approach, current methodologies, challenges, and potential advancements in this challenging field. A further goal of this article is to provide a guide for understanding these complex diseases, highlighting the pivotal role of autopsy, histopathology, and genetic investigations in defining the cause of death, and providing evidence about the translational use of autopsy reports. Forensic investigations play a crucial role in understanding the complexities of CHDs and determining the cause of death accurately. Through collaboration between medical professionals and forensic experts, meticulous examinations, and analysis of evidence, valuable insights can be gained. These insights not only provide closure to the families affected but also contribute to the prevention of future tragedies.

Preventive Strategies In Paediatric Cardiovascular Surgery: Impact On Surgical Site Infections And Beyond.

BACKGROUND: Surgical management of congenital heart disease (CHD) has increased worldwide, but healthcare-associated infections (HAI) can impede these efforts. This study analysed the incidence of HAI, the impact of preventive interventions, and microbiological profiles in a paediatric cardiovascular surgery program. METHODS: Cohort study including children under 12 years of age with CHD who underwent cardiovascular surgery between 2010-2021 in Medellín, Colombia (a middle-income setting). Data were collected from medical and laboratory records and infection control program databases. Impact of various preventive interventions was assessed using a Poisson model. A p-value less than 0.05 was considered statistically significant. RESULTS: 2512 surgeries were analysed. Incidence of surgical site infection (SSI) was 5.9%, followed by central line-associated bloodstream infection (CLABSI; 4.7%), catheter-associated urinary tract infection (CAUTI; 2.2%) and ventilator-associated pneumonia (VAP; 1.4%). Most of the strategies focused on preventing SSI, resulting in a reduction from 9.5% in 2010 to 3.0% in 2021 (p=0.030). Antibiotic prophylaxis based on patient weight and continuous infusion had an impact on reducing SSI (RR:0.56; 95%CI:0.32-0.99). Vacuum-assisted closure (VAC) in clean wounds reduced 100% infections. No significant risk reduction was observed for other HAI with the implemented interventions. CONCLUSION: Preventive strategies effectively reduced SSI but no other infections, emphasizing the need for targeted approaches to address a broader spectrum of HAI successfully.

Children with Congenital Heart Diseases Exhibit Altered Deep Gray Matter Structures.

BACKGROUND AND OBJECTIVES: Children with congenital heart diseases (CHDs) have an increased risk of developing neurologic deficits, even in the absence of apparent brain pathology. The aim of this work was to compare quantitative macro- and microstructural properties of subcortical gray matter structures of pediatric CHD patients with normal appearing brain magnetic resonance imaging to healthy controls. METHODS: We retrospectively reviewed children with coarctation of the aorta (COA) and hypoplastic left heart syndrome (HLHS) admitted to our hospital. We identified 24 pediatric CHD patients (17 COA, 7 HLHS) with normal-appearing brain MRI. Using an atlas-based approach, the volume and apparent diffusion coefficient (ADC) were determined for the thalamus, caudate, putamen, pallidum, hippocampus, amygdala, nucleus accumbens, cerebral white matter, cerebral cortex, and brainstem. Multivariate statistics were used to compare the extracted values to reference values from 100 typically developing children without any known cardiac or neurological diseases. RESULTS: Multivariate analysis of covariance using the regional ADC and volume values as dependent variables and age and sex as co-variates revealed a significant difference between pediatric CHD patients and healthy controls (p < 0.001). Post-hoc comparisons demonstrated significantly reduced brain volumes in most subcortical brain regions investigated and elevated ADC values in the thalamus for children with CHD. No significant differences were found comparing children with COA and HLHS. CONCLUSIONS: Despite normal appearing brain MRI, children with CHD exhibit wide-spread macro-structural and regional micro-structural differences of subcortical brain structures compared to healthy controls, which could negatively impact neurodevelopment, leading to neurological deficits in childhood and beyond.

Efficiency of copy number variation sequencing combined with karyotyping in fetuses with congenital heart disease and the following outcomes.

BACKGROUND: Both copy number variant-sequencing (CNV-seq) and karyotype analysis have been used as powerful tools in the genetic aetiology of fetuses with congenital heart diseases (CHD). However, CNV-seq brings clinicians more confusions to interpret the detection results related to CHD with or without extracardiac abnormalities. Hence, we conducted this study to investigate the clinical value of CNV-seq in fetuses with CHD. RESULTS: A total of 167 patients with fetal CHD including 36 single CHD (sCHD), 41 compound CHD (cCHD) and 90 non-isolated CHD (niCHD) were recruited into the study. 28 cases (16.77%, 28/167) were revealed with chromosomal abnormalities at the level of karyotype. The pathogenic detection rate (DR) of CNV-seq (23.17%, 19/82) was higher than that of karyotyping (15.85%, 13/82) in 82 cases by CNV-seq and karyotyping simultaneously. The DR of pathogenic copy number variations (PCNVs) (31.43%) was higher in niCHD subgroup than that in sCHD and cCHD (9.52% and 23.08%). Conotruncal defect (CTD) was one of the most common heart malformations with the highest DR of PCNVs (50%) in 7 categories of CHD. In terms of all the pregnancy outcomes, 67 (40.12%) cases were terminated and 100 (59.88%) cases were live neonates. Only two among 34 cases with a pathogenic genetic result chose to continue the pregnancy. CONCLUSIONS: CNV-seq combined with karyotyping is a reliable and accurate prenatal technique for identifying pathogenic chromosomal abnormalities associated with fetal CHD with or without extracardiac abnormalities, which can assist clinicians to perform detailed genetic counselling with regard to the etiology and related outcomes of CHD.

The Outcomes of Cardiac Surgery in Children With DiGeorge Syndrome in a Single Center Experience: A Retrospective Cohort Study.

Background DiGeorge syndrome, a common genetic microdeletion syndrome, is associated with multiple congenital anomalies, including congenital cardiac diseases. This study aims to identify the short and midterm outcomes of cardiac surgery performed on children with DiGeorge syndrome. Methods A retrospective cohort study was conducted between the period of 2018-2022, which included children divided into two groups with a 1:2 ratio. Group one included DiGeorge syndrome patients who were diagnosed using fluorescence in situ hybridization (FISH). Group two included the control group of patients who were clear of genetic syndromes. The two groups were matched based on similar cardiac surgery, age of surgery, and Risk Adjustment in Congenital Heart Surgery (RACHS-1) score. The two groups were compared based on the demographical data and postoperative complications. Results The study consisted of 81 children; 27 were DiGeorge syndrome patients, and 54 were in the control group. DiGeorge syndrome patients showed an increase in mechanical ventilation duration (p=0.0047), intensive care unit (ICU) length of stay (p=0.0012), and hospital length of stay (p=0.0391). Moreover, they showed an increased risk for bacteremia (p=0.0414), ventilator-associated pneumonia (VAP; p=0.0036), urinary tract infections (UTI; p=0.0064), and surgical site infection (SSI; p≤0.0001). They were also more susceptible to postoperative seizures (p=0.0049). Furthermore, patients with DiGeorge syndrome had a higher prevalence of congenital renal anomalies. However, there was no mortality in either group.  Conclusion This study shows a variability in the postoperative outcomes between the two groups. The study demonstrates that patients with DiGeorge syndrome have higher risks of infections and longer hospital stay during the postoperative period. Further research with a larger sample is needed to confirm our findings.

Fetal left and right ventricular strain parameters using speckle tracking in congenital heart diseases.

Assessment of fetal ventricular function is mostly subjective, and currently, for the objective assessment left ventricular shortening fraction is obtained. However, this by itself is not very reliable. Hence, more tools that can provide an objective assessment are needed to increase the confidence of functional assessment. Speckle tracking imaging can provide one such tool. In this study we sought to establish the normative value of global longitudinal and circumferential strain for our fetal patients and for two major forms of congenital heart diseases, namely atrioventricular canal defects (AVC) and uncorrected dextro-transposition of the great arteries (dTGA) to act as a benchmark. The study was completed via a single center retrospective analysis on 72 fetal echocardiograms (26 normal, 15 dTGA, and 31 AVC). Tomtec Arena™ echocardiography analysis software was used for analysis. In normal fetuses, mean left ventricular (LV) global longitudinal strain (GLS) was - 22.6% (95% CI -24, -21.1) and mean right ventricular (RV) GLS was - 22.1% (95% CI -23.6, -20.6). In AVC patients LV GLS was-26.6% (95% CI -28,-25.3) and mean RV GLS was - 26.5% (95% CI -27.9,-25.2). In dTGA patients LV GLS was - 22.9% (95% CI of -24.8, -21) and RV GLS was - 21.3% (95% CI was - 23.4, -20.8). There was good intra-rater reliability though poor to fair inter-rater reliability. Notwithstanding its current limitations, strain imaging can provide useful information that can increase confidence of cardiac functional assessment in fetal patients. However, to be reliable across the board, further automation and standardization is required.

Exposure to Endocrine-Disrupting Chemicals and Congenital Heart Diseases: The Pooled Results Based on the Current Evidence.

The relationships between maternal exposure to endocrine-disrupting chemicals (EDCs) and congenital heart diseases (CHD) are not elucidated yet. The exposure levels of EDCs are generally estimated based on self-reported questionnaires or occupational exposure evaluations in the literature. Therefore, a study based on epidemiological data from human biospecimens is required to provide stronger evidence between maternal exposure to EDC and CHD. Embase, Pubmed, Scopus, and the Cochrane Library databases were searched for related research which provided risk estimates regarding the relationships between maternal EDC exposure and CHD in human offspring. Baseline characteristics and outcomes of CHD were extracted from each included study. Odds ratios (ORs) with 95% confidence intervals (CIs) were pooled to calculate the overall estimates of CHD. Subgroup and meta-regression analyses were performed to identify the sources of heterogeneity. Bootstrapping techniques were used in analyses where several studies originated from a similar population. A total of seventeen studies were involved in the meta-analyses. Maternal EDC exposure was significantly related to CHD in offspring (OR 2.15; 95%CI 1.64 to 2.83). EDC exposure was significantly associated with septal defects (OR 2.34; 95%CI 1.77 to 3.10), conotruncal defects (OR 2.54; 95%CI 1.89 to 3.43), right ventricular outflow tract obstruction (OR 2.65; 95%CI 1.73 to 4.07), left ventricular outflow tract obstruction (OR 3.58; 95%CI 2.67 to 4.79), anomalous pulmonary venous return (OR 2.31; 95%CI 1.34 to 4.00), and other heart defects (OR 2.49; 95%CI 1.75 to 3.54). In addition, maternal exposure to heavy metals, which included lead (OR 2.19; 95%CI 1.29 to 3.71), cadmium (OR 1.81; 95%CI 1.28 to 2.56), mercury (OR 2.23; 95%CI 1.13 to 4.44), and manganese (OR 2.65; 95%CI 1.48 to 4.74), increased risks for CHD significantly. In conclusion, based on the latest evidence, maternal EDC exposure may increase CHD risks in human offspring, especially in heavy metal exposure conditions.

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

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

Engineered platforms for mimicking cardiac development and drug screening.

Congenital heart defects are associated with significant health challenges, demanding a deep understanding of the underlying biological mechanisms and, thus, better devices or platforms that can recapitulate human cardiac development. The discovery of human pluripotent stem cells has substantially reduced the dependence on animal models. Recent advances in stem cell biology, genetic editing, omics, microfluidics, and sensor technologies have further enabled remarkable progress in the development of in vitro platforms with increased fidelity and efficiency. In this review, we provide an overview of advancements in in vitro cardiac development platforms, with a particular focus on technological innovation. We categorize these platforms into four areas: two-dimensional solid substrate cultures, engineered substrate architectures that enhance cellular functions, cardiac organoids, and embryos/explants-on-chip models. We conclude by addressing current limitations and presenting future perspectives.

Management and outcomes of pregnant women with cardiovascular diseases in a cardio-obstetric team.

BACKGROUND: Cardiovascular diseases (CVDs) are currently the leading cause of maternal death in Western countries. Although multidisciplinary cardio-obstetric teams are recommended to improve the management of pregnant women with CVD, data supporting this approach are scarce. AIMS: To describe the characteristics and outcomes of pregnant patients with CVD managed within the cardio-obstetric programme of a tertiary centre. METHODS: We included every pregnant patient with history of CVD managed by our cardio-obstetric team between June 2017 and December 2019, and collected all major cardiovascular events (death, heart failure, acute coronary syndromes, stroke, endocarditis and aortic dissection) that occurred during pregnancy, peripartum and the following year. RESULTS: We included 209 consecutive pregnancies in 202 patients. CVDs were predominantly valvular heart diseases (37.8%), rhythm disorders (26.8%), and adult congenital heart diseases (22.5%). Altogether, 47.4% were classified modified World Health Organization (mWHO)>II, 66.5% had CARdiac disease in PREGnancy score (CARPREG II)≥2 and 80 pregnancies (38.3%) were delivered by caesarean section. Major cardiovascular events occurred in 16 pregnancies (7.7%, 95% confidence interval [CI] 4.5-12.2) during pregnancy and in three others (1.5%, 95% CI 0.3-4.1) during 1-year follow-up. Most events (63.1%) occurred in the 16.3% of patients with unknown CVD before pregnancy. CONCLUSIONS: The management of pregnant patients with CVD within a cardio-obstetric team seems encouraging as we found a relatively low rate of cardiovascular events compared to the high-risk profile of our population. However, most of the remaining events occurred in patients without cardiac monitoring before pregnancy.

Maternal diabetes and risk of offspring congenital heart diseases: the Japan Environment and Children's Study.

BACKGROUND: Few prospective cohort studies have examined the association between maternal diabetes, including pre-pregnancy and gestational diabetes, and the risk of congenital heart disease (CHD) in Asian offspring. METHODS: We examined the association between maternal diabetes and offspring CHD among 97,094 mother-singleton infant pairs in the Japan Environment and Children's Study (JECS) between January 2011 and March 2014. Odds ratios (OR) and 95% confidence intervals (CI) of offspring CHD based on maternal diabetes (pre-pregnancy diabetes and gestational diabetes) were estimated using logistic regression after adjusting for maternal age at delivery, pre-pregnancy body mass index (BMI), maternal smoking habits, alcohol consumption, annual household income, and maternal education. The diagnosis of CHD in the offspring was ascertained from the transcript of medical records. RESULTS: The incidence of CHD in the offspring was 1,132. Maternal diabetes, including both pre-pregnancy diabetes and gestational diabetes, was associated with a higher risk of offspring CHD: multivariable OR (95%CI) = 1.81 (1.40-2.33) for maternal diabetes, 2.39 (1.05-5.42) for pre-pregnancy diabetes and 1.77 (1.36-2.30) for gestational diabetes. A higher risk of offspring CHD was observed in pre-pregnancy BMI ≥25.0 kg/m2 (OR = 2.55, 95% CI: 1.74-3.75) than in pre-pregnancy BMI <25.0 kg/m2 (OR = 1.49, 95% CI: 1.05-2.10, p for interaction = 0.04). CONCLUSIONS: Maternal diabetes, including both pre-pregnancy and gestational, was associated with an increased risk of CHD in offspring.

Seguimiento a muy largo plazo tras implante de stent en la coartación de aorta / Very long-term follow-up after aortic stenting for coarctation of the aorta

Introducción y objetivos: El implante de stent es el tratamiento de elección en niños mayores y adultos con coartación aórtica (CoA). El objetivo fue determinar la incidencia de eventos tardíos después del tratamiento con stent. Métodos: Se analizó una cohorte de pacientes con CoA tratados en el centro entre 1993 y 2018 a los que se siguió periódicamente, incluyendo evaluación mediante tomografía computarizada (TC) y fluoroscopia. Resultados: Se incluyó a 167 pacientes con TC y fluoroscopia: 83 (49,7%) ≤ 12 años y 46 (28%) mujeres. El tiempo medio de seguimiento clínico fue de 17±8 años (rango 4-30) y de 11±7 años hasta la TC/fluoroscopia. Se detectó aneurisma en un 13% y se asoció al stent PALMAZ (OR=3,09; IC95%, 1,11-9,49; p=0,036) y a la longitud del stent (OR=0,94; IC95%, 0,89-0,99; p=0,039). La fractura del stent fue frecuente (34%) pero no asociada a la presencia de aneurisma. Se asoció con edad joven (OR=3,57; IC95%, 1,54-8,33; p=0,003), sexo masculino (OR=4,00; IC95%, 1,51-12,50; p=0,008) e inversamente con el stent PALMAZ (OR=0,29; IC95%, 0,12-0,67; p=0,005). La reintervención fue menor en adultos (10%), principalmente por aneurismas. Los pacientes que recibieron tratamiento cuando tenían 12 años o menos presentaron tasas de reintervención más altas (43%) debido al crecimiento somático. Conclusiones: Se observó una incidencia notable de eventos tardíos a largo plazo en pacientes con CoA tratados mediante stent. La reintervención fue más frecuente en pacientes tratados a edades más jóvenes. Parece aconsejable una vigilancia periódica mediante pruebas de imagen. (AU)

Introduction and objectives: Stent implantation is the preferred treatment in older children and adults with aortic coarctation (CoA). We aimed to determine the incidence of very late events after CoA stenting. Methods: We analyzed a cohort of CoA patients who underwent stent implantation at our center between 1993 and 2018. Patients were periodically followed up in outpatient clinics, including computed tomography (CT) and fluoroscopy assessment. Results: A total of 167 patients with CT and fluoroscopy data were included: 83 (49.7%) were aged ≤ 12 years and 46 (28%) were female. The mean clinical follow-up time was 17±8 (range 4-30) years and the mean time to CT/fluoroscopy was 11±7 years. Aortic aneurysm was present in 13% and was associated with the PALMAZ stent (OR, 3.09; 95%CI, 1.11-9.49; P=.036) and the stented length (OR, 0.94; 95%CI, 0.89-0.99; P=.039). Stent fracture was frequent (34%), but was not related to the presence of aneurysm. Stent fracture was associated with young age (OR, 3.57; 95%CI, 1.54-8.33; P=.003), male sex (OR, 4.00; 95%CI, 1.51-12.5, P=.008) and inversely with the PALMAZ stent (OR, 0.29; 95%CI, 0.12-0.67, P=.005). Reintervention was lower in adults (10%), mainly related to aneurysms. Those treated when aged ≤ 12 years had higher reintervention rates (43%) due to recoarctation somatic growth. Conclusions: This long-term follow-up study of CoA patients treated with stenting revealed a significant incidence of late events. Reintervention rates were higher in patients treated at younger ages. Periodic imaging surveillance appears to be advisable. (AU)