TAPVR

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...

Heterotaxy syndrome (HS) occurs in developing embryos due to an inability to establish the normal anatomy, which manifests as abnormal symmetry and malposition of the thoracoabdominal viscera and vasculature, including cardiac and extracardiac anomalies. It is classified as right or left atrial isomerism. This classification depends on the atrial appendage morphology and the extracardiac defect associated with it. Right isomerism usually presents with right atrial appendages (RAA), asplenia, total anomalous pulmonary venous return, and severe pulmonary stenosis...

• Multimodality imaging is important in patients with ACHD and challenging anatomy. • Complex congenital anatomy and physiology requires multidisciplinary discussions. • Image-guided percutaneous intervention may be warranted after TAPVR repair.

We report an uncommon case report of total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein's ostium without a sinus venosus defect, in situs solitus, without vertical vein or a posterior pulmonary venous confluence.

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) A new index for prenatal diagnosis of total anomalous pulmonary venous return, (2) Outcomes of patients with Tetralogy of Fallot after pulmonary valve replacement (PVR), and (3) Short-term outcomes of the self expanding Harmony valve for transcatheter PVR...

OBJECTIVE: Challenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. METHODS: A prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Based on the preoperative admission location in our hospital, patients were classified into those being admitted to cardiac intensive care unit versus neonatal intensive care unit or general pediatric intensive care unit...

OBJECTIVE: Image quality of fetal echocardiography (FE) has improved in the recent era, but few recent studies have reported the accuracy of FE specifically in single ventricle congenital heart disease (SV). Our study aims to assess the ability of FE to correctly predict postnatal anatomy and physiology in SV in a contemporary cohort. METHODS: The contemporary, clinical reports of FE with SV performed from 7/2017 to 7/2021 were compared with postnatal echocardiograms in a formal quality assurance program...

Vertical vein (VV) ligation during total anomalous pulmonary venous return (TAPVR) repair is controversial. While some surgeons prefer ligation of the VV to prevent adverse sequelae of shunting across it and to promote flow through the newly created anastomosis, others leave it to serve as a "pop off valve" to the left heart structures, which are believed to be hypoplastic and noncompliant, presumably contributing to a more favorable post-operative outcome. We report two patients post-Fontan procedure, who underwent cardiac catheterization to explore the etiology of hypoxia and were found to have a persistent VV responsible for right to left shunting...

INTRODUCTION: Three-dimensional (3D) printed cardiac models are increasingly being used for medical education, simulation and training, communication, surgical planning and research. Given the complexities of congenital cardiac anatomy, 3D printing is well suited as an adjunct to traditional teaching methods. This study aims to explore the influence of 3D printed cardiac models as a teaching aid for nurses and paediatric trainees. We hypothesise that using 3D models as an adjunct to didactic teaching methods improves knowledge and confidence levels of participants, regardless of their cardiology experience...

No abstract text is available yet for this article.

INTRODUCTION AND IMPORTANCE: Pentaloy of fallot (POF) is a congenital cardiac anomaly that includes ventricular septal defect (VSD), pulmonary stenosis (PS), overriding of the aorta, and right ventricular hypertrophy. Dextrocardia, on the other hand, is a congenital condition in which the heart is right-sided. Rarely, both of these conditions can coexist. In this case, we report the 20-year follow-up results for the successful management of POF coexisting with Dextrocardia and other anomalies, which is the first described case in the literature...

Cor triatriatum sinister is rare congenital heart disease. It is defined as the presence of an abnormal septum dividing the left atrium into two chambers, and in some cases may be associated with a total abnormal pulmonary venous connection. Prenatal diagnosis of cor triatriatum sinister may be possible with fetal echocardiography and the diagnosis can be made earlier than postnatal imaging techniques. In the fetal echocardiography performed in our patient, cor triatriatum sinister was present and the main pathology was the flow of right and left pulmonary vein return from the accessory atrium to the right atrium...

Total anomalous pulmonary venous return due to septum primum malposition is a poorly understood condition despite being very common in left atrial isomerism or polysplenia syndrome. Due to the leftward displacement of the septum primum, either the two right pulmonary veins or all four pulmonary veins can drain abnormally into the right atrium, despite their correct position. In other words, the four pulmonary veins (or the two right pulmonary veins), looking from outside the heart, return at the back of the atrium in the normal position...

RAC1 at 7p22.1 encodes a RAC family small GTPase that regulates actin cytoskeleton organization and intracellular signaling pathways. Pathogenic RAC1 variants result in developmental delay and multiple anomalies. Here, exome sequencing identified a rare de novo RAC1 variant [NM_018890.4:c.118T > C p.(Tyr40His)] in a male patient. Fetal ultrasonography indicated the patient to have multiple anomalies, including persistent left superior vena cava, total anomalous pulmonary venous return, esophageal atresia, scoliosis, and right-hand polydactyly...

BACKGROUND: Atrioventricular valve regurgitation (AVVR) is present in up to 75% of Fontan patients, and it is associated with an increased risk of Fontan circulation failure, morbidity, and mortality. Traditional treatment options include surgical repair vs. surgical replacement. We present, to the best of our knowledge, one of the first cases of successful trans-catheter repair of severe common AVVR using the MitraClip device. CASE SUMMARY: A 20-year-old male with a history of double-outlet right ventricle (DORV) with unbalanced common atrioventricular canal to the right ventricle, severely hypoplastic left ventricle, and total anomalous pulmonary venous return status post-Fontan procedure presented with progressively worsening dyspnoea on exertion...

Turner syndrome (TS) is a genetic disorder presenting in phenotypic females with total or partial monosomy of the X chromosome. Cardiovascular abnormalities are common, including congenital heart defects (CHD) and aortic dilation. Although mosaic TS is suspected to have less severe phenotype as compared to non-mosaic TS, differences in cardiovascular manifestations between karyotypes are not well studied. This is a single-center retrospective cohort study including patients with TS seen from 2000 to 2022. Demographic data, chromosomal analysis, and imaging were reviewed...

No abstract text is available yet for this article.

OBJECTIVES: The study objectives were to analyze the outcomes of pediatric patients with heterotaxy syndrome undergoing cardiovascular surgery and to determine the predictors of mortality. METHODS: A retrospective analysis of 82 patients diagnosed with heterotaxy syndrome who underwent cardiovascular surgery between January 2008 and December 2017 was performed. Univariate and multivariable Cox regression analyses to determine risk factors for mortality and Kaplan-Meier analysis for survival were performed...

BACKGROUND: The superior sinus venosus atrial septal defect is a congenital communication between the left and right atria. Open surgical approach by patch closure has historically been the only treatment option. Recently, a transcatheter approach has been developed. This study aims to compare the efficacy and safety of surgical and transcatheter approach in treatment of sinus venosus atrial septal defect. METHODS: Between March 2010 and December 2020, 58 patients (median age: 45...

BACKGROUND: Pulmonary vein stenosis (PVS) is an uncommon but known cause of morbidity and mortality in adults and children and can be managed with percutaneous re-vascularization strategies of pulmonary vein balloon angioplasty (PBA) or pulmonary vein stent implantation (PSI). AIM: To study the safety and efficacy outcomes of PBA vs PSI in all patient categories with PVS. METHODS: We performed a literature search of all studies comparing outcomes of patients evaluated by PBA vs PSI for PVS...