The developmental processes of ventricular septal defects with outflow tract malalignment.

Ventricular septal defects (VSD) with outflow tract (OFT) malalignment are a common group of congenital heart diseases with varying severity. The developmental process of these defects is challenging to understand due to the complex nature of cardiac morphogenesis and the difficulties in visualizing the temporal and spatial changes that occur during pathogenesis. However, recent advancements in imaging techniques, such as high-resolution episcopic microscopy, have provided valuable insights into the normal septation of ventricular chambers and OFT alignment. Building upon this knowledge, we have utilized lightsheet microscopy, another innovative imaging method, to further investigate the developmental processes that lead to abnormal formation of the ventricular septum and the malalignment of arterial roots with the ventricular chambers. Our study highlights endocardial cushion hypoplasia and insufficient rotation of the outflow tract as two interrelated central factors contributing to the pathogenesis of these defects. This finding has the potential to enhance our understanding of the etiology of congenital heart diseases and may contribute to the development of improved diagnostic and therapeutic strategies in the future.

Beyond Synthetics: Promising Outcomes With the Invengenx® Bovine Pericardial Patch for Ventricular Septal Defect Repair in a Young Pediatric Population.

Ventricular septal defects (VSDs) are a prevalent congenital heart anomaly demanding safe and lasting interventions. This paper explores the application of Invengenx® bovine pericardial patch (Tisgenx, Irvine, California), a promising biomaterial, in VSD repair. We present two case studies: a seven-month-old infant and a three-year-old child undergoing VSD closure using autologous and bovine pericardial patches, respectively. Both patients tolerated the procedures well, experiencing no intra-operative complications and demonstrating excellent postoperative recovery. Echocardiography postoperatively showed no complications and improved clinical outcomes. Notably, the pericardial patches exhibited excellent integration and suture retention, highlighting their durability and compatibility with the growing heart. These cases establish the feasibility and effectiveness of the Invengenx® pericardial patch for VSD repair. The favorable outcomes in terms of safety and efficacy support the potential of this biomaterial as a valuable alternative in pediatric cardiac surgery, particularly for complex VSDs or patients with contraindications to synthetic patches. Further research is crucial to unlock the full potential of bovine pericardium as a durable and advantageous option for VSD repair in a broader range of pediatric patients.

Comparison Between Fast-Track and Conventional Anesthesia for Children Undergoing Closure of Ventricular Septal Defects: A Systematic Review and Meta-Analysis.

Ventricular septal defect (VSD) is common in pediatric patients. This study aimed to evaluate the safety and effectiveness of using fast-track anesthesia and comparing it to traditional anesthesia, among children undergoing a transthoracic device closure for VSD. A systematic review following the 2020 Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines was conducted. Relevant literature was identified through specific search terms in the Scopus, Medical Literature Analysis and Retrieval System Online (MEDLINE), Excerpta Medica database (Embase), Web of Science, The Cochrane Central Register of Controlled Trials (CENTRAL), and Google Scholar databases. The inclusion criteria focused on observational studies that compared fast-track anesthesia with conventional anesthesia in pediatric VSD closure cases. Data extraction, quality assessment, and meta-analysis were performed using standard differences in means. Initially, 6,535 papers were identified, and subsequent screening of titles and abstracts led to the inclusion of four retrospective studies from a total of 51 studies. The analysis encompassed 477 patients, with 235 in the fast-track anesthesia group and 242 in the conventional anesthesia group. No statistically significant disparities were observed between the two groups concerning the operative duration and hemodynamic variations post-intubation or post-procedure (P >0.05). Nevertheless, the fast-track anesthesia group demonstrated significantly reduced healthcare expenses as well as shorter periods of mechanical ventilation, ICU stay, and overall hospitalization compared to conventional anesthesia (P <0.05). The use of fast-track anesthesia in combination with transthoracic device closure for VSD demonstrates a safe and effective approach for pediatric patients. This approach results in reduced healthcare costs (10,000 Renminbi (RMB)) and shorter durations of mechanical ventilation, ICU admission, and hospitalization compared to conventional anesthesia. Further clinical trials are necessary to confirm these results and assess long-term outcomes.

Heart surgery in a pediatric patient with congenital heart disease and hemophilia B: A case report from Sudan.

Key Clinical Message: A multidisciplinary team approach, careful hemostasis, and factor replacement therapy are important in the perioperative management of hemophiliac patients undergoing pediatric cardiac surgery. Abstract: The combination of congenital heart diseases (CHD) and hemophilia is rare; furthermore, heart surgery and perioperative management of such cases is challenging. This report illustrates the challenges of pediatric cardiac surgery in an infant with both hemophilia B and CHD. Multidisciplinary team approach, careful hemostasis, and factor replacement therapy were key to success without hemorrhagic complications before, during and after surgery.

Long-term outcomes of percutaneous closure of ventricular septal defects in children using different devices: A single centre experience from Egypt.

BACKGROUND: The feasibility of percutaneous closure ventricular septal defects (VSD) in children has been previously proven. However, data on long-term outcomes are limited. We aim to evaluate the long-term outcome of our experience with percutaneous closure of VSD using various occluders. METHODS: Retrospective institutional analysis of children who underwent transcatheter closure of perimembranous and muscular VSDs between September 2012 and February 2020. Patient demographics, procedural, and long-term follow-up data were comprehensively analyzed. Patients who lost to follow-up within two years post-procedure were excluded. RESULTS: We identified 75 patients (54.7% males) with a median of 66 months (IQR, 46-96). The closure success rate at one year was 95.7%. Complete heart block was detected in two patients early post-procedure and resolved with steroids. The VSDs were perimembranous (52%), muscular (33.33%), and residual (14.67%). Implanted devices were Pfm Nit-Occlud LeˆVSD Coil (42.7%), HyperionTM VSD Muscular Occluder (28%), Amplatzer VSD muscular occluder (10.7%), Amplatzer Duct Occluder (14.7%), Occlutech Muscular VSD Occluder (2.7%), and Amplatzer Duct Occluder II (1.3%). No new arrhythmia or valve regurgitation was detected after two years post-procedure. Persisted complications on long-term follow-up included: residual shunting in 3(4%), mild tricuspid regurgitation in 2(2.7%), and aortic regurgitation in 2(2.7%), with one immediate post-catheterization mild aortic regurgitation worsened during follow-up, requiring surgical repair of VSD three years after device implantation. No deaths were reported. CONCLUSION: Long-term outcomes of pediatric transcatheter VSD closure using different devices are satisfactory. Post-procedural adverse events are limited, but long-term surveillance is necessary to monitor their progression.

Outlet Ventricular Septal Defect: Impact of Surgery on the Late Aortic and Pulmonary Valve Functions.

This study retrospectively evaluated long-term post-operative aortic and pulmonary valve functions in outlet ventricular septal defects. We evaluated aortic regurgitation and pulmonary regurgitation using pre- and post-operative echocardiograms. Overall, 158 patients who underwent intracardiac repair due to outlet ventricular septal defects with aortic valve deformity or congestive heart failure were included. The median follow-up period was 7 years (interquartile range: 0-17 years), without deaths or pacemaker implantations. The age, weight, ventricular septal defect size, and mild aortic regurgitation at surgery were factors associated with post-operative residual aortic regurgitation. Mild pulmonary regurgitation was observed in 12%, 30%, and 40% of patients 5, 10, and 15 years after surgery, respectively. There were no significant differences in age and weight at which surgery was performed between patients with mild pulmonary regurgitation and those with less than mild pulmonary regurgitation. However, the number of sutures across the pulmonary valve was associated with post-operative pulmonary regurgitation (P < 0.01). As some patients with mild pre-operative aortic regurgitation may not improve even after surgery, early surgical intervention is necessary when aortic regurgitation appears. Some patients may develop post-operative pulmonary regurgitation in the long term, suggesting the need for careful follow-up.

Fetal Cyclopia, Proboscis, Holoprosencephaly, and Polydactyly: A Case Report With Review of Literature.

We present a case of holoprosencephaly (HPE) with cyclopia and proboscis. The mother was a 35-year-old, G1P1 with no known comorbid conditions, not in a consanguineous marriage, and with no history of illicit drug use. On a routine antenatal ultrasound scan, features of alobar HPE, proboscis, and other anomalies were identified. The mother was counseled about the condition and as per their consent, the pregnancy was terminated. After labor induction, she gave birth to a female neonate weighing 1,000 g. The newborn's Apgar score could not be calculated. In the initial physical examination, an eye and a 3.5-cm proboscis were seen in the middle of the forehead. The newborn had no nose, and the outer ears were normal. On postmortem examination, alobar HPE, polydactyly, ventricular septal defect, and myelomeningocele were confirmed. This case report highlights the importance of attention to these details during antenatal scans for early detection in order to reduce the maternal and neonatal health burden. The pictures presented in this article were taken after obtaining parental consent.

Genetic studies in the Pakistani population reveal novel associations with ventricular septal defects (VSDs).

BACKGROUND: With prevalence up to 4%, Ventricular Septal Defect (VSD) is one of the leading causes of neonatal deaths. VSD is a common complex genetic disorder that has been associated with many genetic determinants. Variants from genes for the transcription factors including T-Box TBX5 and NFATc1 (nuclear factor of activated T cells, cytoplasmic 1), Vascular endothelial growth factor (VEGF), ISLET1 (encoded by the ISL1 gene) and enzyme MTHFR, a methylene tetrahydrofolate reductase were selected. Genetic risk score (GRS) is a widely accepted approach used to convert the genetic data into prediction and assessment tool for disease susceptibility. METHODS: A total of 200 participants were recruited for the current study, 100 VSD patients and 100 controls. Genotyping of the ISL1: rs1017, NFATc1: rs7240256, VEGF: rs36208048, TBX5: rs11067075, and MTHFR: rs1801133 variants was performed using tetra primer ARMS PCR and PCR-RFLP. For the statistical analysis, the software SPSS version 23 was used. Genotypic frequencies of cases and controls were calculated using chi-square (χ²) whereas allelic frequencies were calculated by using the SNPStats tool. The association of GRS quartiles with VSD was examined using binary logistic regression. Adjusted p-value 0.01 was used as significance threshold for all analyses. RESULTS: The ISL1 (OD: 0.242, CI: 0.158-0.37, p-value: 2.15 × 10- 4 :), NFATc1 (OD: 2.53, CI: 1.64-3.89, p-value: 2.11 × 10- 5), TBX5 (OD: 2.24, CI: 1.47-3.41, p-value:1.6 × 10- 4) and MTHFR (OD: 10.46, CI: 5.68-19.26, p-value: 2.09 × 10- 9:) variants were found to be in association with VSD. In contrast, the VEGF (OD: 0.952, CI: 0.56-1.62, p-value: 0.8921) variant did not show significance association with the VSD. For cases, the mean GRS score was 3.78 ± 1.285 while in controls it was 2.95 ± 1.290 (p-value: 0.479, CI: 0.474-1.190). Comparison of GRS between cases and control showed that mean GRS of cases was 1.90 ± 0.480 while in controls it was 1.68 ± 0.490 (p-value: 0.001, CI: 0.086-0.354). Higher quartiles were more prevalent in cases whereas lower quartiles were more prevalent in controls. CONCLUSION: GRS of these five loci was strongly associated with VSD. Moreover, genetic risk score can provide better information for the association between variants and disease as compared to a single SNP. We also illustrated that the cumulative power of GRS is greater over the single SNP effect. This is a pilot scale study with a relatively small sample size whose findings should be replicated in a larger sample size for the unique local Pakistani population.

Practice Patterns in the Management of Pressure Restrictive Perimembranous Ventricular Septal Defects: A Multinational Survey.

Indications for the closure of pressure restrictive perimembranous ventricular septal defects (pmVSD) are not well established in the pediatric population. We sought to assess practice variability among pediatric cardiologists in the United States (US), Canada, Australia, and New Zealand. A survey ascertaining practice patterns, including case vignettes with incremental progression of disease severity, was designed and administered through representative professional cardiac organizations and email listservs in the designated countries. Among the 299 respondents, 209 (70.0%) were from the US, 65 (21.7%) were from Canada and 25 (8.3%) were from Australia and New Zealand. Indications for pressure restrictive pmVSD closure included the presence of left ventricular (LV) dilation for 81.6% (244/299) (defined as z-score ≥ 2 for 59.0% (144/244) and ≥ 3 for 40.2% (98/244)) and significant pulmonary-systemic flow ratio (QP:QS) for 71.2% (213/299) [defined as ≥ 1.5:1 for 36.2% (77/213) and ≥ 2 for 62% (132/213)]. US pediatric cardiologists elected to close restrictive pmVSD at lower LV z-score and QP:QS ratio cut-offs (p-value 0.0002 and 0.013, respectively). In a case vignette, 63.6% (173/272) chose to intervene if there was right coronary cusp prolapse with stable mild aortic regurgitation. Of the remaining cardiologists, 93% (92/99) intervened if the aortic regurgitation was progressive (from trivial to mild). Commonly identified indications with variable thresholds for closure of pressure restrictive pmVSDs included the presence or progression of LV dilation, significant volume loading, and aortic valve prolapse with regurgitation. US pediatric cardiologists may have a lower threshold for pmVSD closure.

Peratrial device closure of perimembranous ventricular septal defects via a small right subaxillary incision: Midterm results in patients <12 months of age.

BACKGROUND: Both percutaneous and perventricular device closures of perimembranous ventricular septal defects (Pm-VSDs) are alternatives to surgical procedures，but they all present certain drawbacks. OBJECTIVE: To report our clinical experiences and midterm follow-up results of minimally invasive peratrial device closure of Pm-VSDs under the guidance of transesophageal echocardiography(TEE) in patients <12 months of age. METHODS: Between January 2015 and December 2020，268 patients <12 months of age with Pm-VSDs underwent peratrial device closure in our institute. The procedure was performed under TEE guidance via a small right subaxillary incision. The delivery pathways is established by manipulating the hollow probe, and then the device is installed. RESULTS: A total of 263 cases (98.1%) underwent successful closure, whereas five cases failed and were converted to cardiopulmonary bypass operation via the original incision during the procedure. The mean age was 9.5 ± 2.0 months and the mean body weight was 8.8 ± 1.4 kg. The mean diameter of the VSD was 4.4 ± 0.5 mm. One patient (0.4%) underwent a second thoracotomy for postoperative intercostal hemorrhage on the second day after surgery. The mean diameter of the occluder size was 5.5 ± 0.6 mm. During the follow-up (4.3 ± 1.4 y), there was no mortality, no new aortic valve regurgitation and atrioventricular block. CONCLUSION: Peratrial device closure of Pm-VSDs via the right subaxillary route under TEE guidance is safe and effective at midterm follow-up, confirming this is an valuable alternative method for patients <12 months of age.

4D Printing of Overall Radiopaque Customized Bionic Occlusion Devices.

Percutaneous closure of ventricular septal defect (VSD) can effectively occlude abnormal blood flow between ventricles. However, commonly used Nitinol occlusion devices have non-negligible limitations, such as nondegradability leading to life-threatening embolization; limited device size predisposing to displacement and wear; only a few radiopaque markers resulting in inaccurate positioning. Nevertheless, the exploration of customized, biodegradable, and overall radiopaque occluders is still vacant. Here, overall radiopaque, biodegradable, and dynamic reconfigurable 4D printed VSD occluders are developed. Based on wavy bionic structures, various VSD occluders are designed and manufactured to adapt to the position diversity of VSD. The customized configuration, biocompatibility, and biodegradability of the developed 4D printed bionic occluders can eliminate the series of complications caused by traditional occluders. The overall radiopacity of 4D printed VSD occluders is validated ex vivo and in vivo, whereby accurate positioning can be assured. Notably, the preparation strategies for 4D printed occluders are scalable, eliminating the barriers to mass production, and marking a meaningful step in bridging the gap between modeling and clinical application of 4D printed occlusion devices. This work opens attractive perspectives for the rapid manufacturing of customized intelligent medical devices for which overall radiopacity, dynamic reconfigurability, biocompatibility, and biodegradability are sought.

Congenital Heart Diseases: Genetic Risk Variants and Their Methylation Status.

(1) Background: The interaction between single nucleotide variants (SNVs) associated with congenital heart diseases (CHDs) and their gene methylation status has not been well researched. The aim of the present study was to determine if there is a relationship between the methy lation status (MS) of genes and the allelic variants associated with CHDs. (2) Methods: Seven SNVs of the genes AXIN1, TBX1, TBX20, and MTHFR were selected from the literature. DNA extraction, genotyping, and a methylation analysis were performed on healthy subjects and subjects with CHDs. (3) Results: Twenty-two subjects with CHDs were selected as the case group (15 with ventricular septal defects (VSDs) and 7 with atrial septal defects (ASDs)), and 44 healthy subjects comprised the control group. The MTHFR and AXIN1 genes were hypermethylated in the control group when compared to the case group. When analyzed separately, those with atrial septum defects exhibited greater methylation, except for the gene MTHFR where there were no differences. Only the alternate alleles of MTHFR showed a significantly different methylation status in those without cardiopathy. (4) Conclusions: The MTHFR and AXIN genes were hypermethylated in the control group; however, only the alternate alleles of MTHFR (rs1801133 and rs1801131) showed a significantly different methylation status.

Incidental Echocardiographic Finding of a Membranous Ventricular Septal Aneurysm in an 18-Year-Old Asymptomatic Athlete.

Membranous ventricular septal aneurysm (VSA) is an uncommon cardiac abnormality, potentially leading to several cardiac complications such as aortic valve prolapse, arrhythmias, and aneurysm rupture. A young competitive soccer player presented for a post-COVID sports cardiology assessment, denying any previous cardiological evaluations. On transthoracic echocardiography, a membranous VSA was incidentally found with no other cardiac abnormality nor hemodynamic impairment associated. A well-oriented anamnesis guided by echocardiographic findings revealed that a ventricular septal defect was diagnosed at birth with spontaneous closure at 4 years old. From that moment, no further follow-up was performed. Before granting cardiological approval to competitive sport, transesophageal echocardiography and Holter electrocardiogram were performed to confirm the absence of interventricular shunt and any other cardiac abnormality or arrhythmias associated with VSA. This case highlights the value of an accurate and comprehensive clinical and echocardiographic evaluation when performing a cardiological sports assessment, even in a young asymptomatic athlete.

Association and Interaction Effect of BHMT Gene Polymorphisms and Maternal Dietary Habits with Ventricular Septal Defect in Offspring.

This study attempted to learn the association between maternal betaine-homocysteine methyltransferase (BHMT) gene polymorphisms, maternal dietary habits, and their interactions with the risk of ventricular septal defects (VSD) in offspring. A total of 426 mothers of VSD children and 740 control mothers were included in the study. Logistic regression was used to evaluate the level of associations and interaction effects. Our study suggested that mothers reporting excessive intake of smoked foods (aOR = 2.44, 95%CI: 1.89-3.13), barbecued foods (aOR = 1.86, 95%CI: 1.39-2.48), fried foods (aOR = 1.93, 95%CI: 1.51-2.46), and pickled vegetables (aOR = 2.50, 95%CI: 1.92-3.25) were at a significantly higher risk of VSD in offspring, instead, mothers reporting regular intake of fresh fruits (aOR = 0.47, 95%CI: 0.36-0.62), fish and shrimp (aOR = 0.35, 95%CI: 0.28-0.44), fresh eggs, (aOR = 0.56, 95%CI: 0.45-0.71), beans (aOR = 0.68, 95%CI: 0.56-0.83), and milk products (aOR = 0.67, 95%CI: 0.56-0.80) were at a lower risk of VSD in offspring. In addition, maternal BHMT gene polymorphisms at rs1316753 (CG vs. CC: aOR = 2.01, 95%CI: 1.43-2.83) and rs1915706 (CT vs. TT: (aOR = 1.81, 95%CI: 1.33-2.46) were significantly associated with increased risk of VSD in offspring. Furthermore, a significant interaction between BHMT polymorphisms and maternal bean intake was identified in the study. In conclusion, Maternal BHMT polymorphisms at rs1316753 and rs1915706, dietary habits as well as their interaction were observed to be significantly associated with the risk of VSD in offspring.

Study of variants associated with ventricular septal defects (VSDs) highlights the unique genetic structure of the Pakistani population.

BACKGROUND: Ventricular septal defects (VSDs) are one of the leading causes of death due to cardiac anomalies during the first months of life. The prevalence of VSD in neonates is reported up to 4%. Despite the remarkable progress in medication, treatment and surgical procedure for VSDs, the genetic etiology of VSDs is still in infancy because of the complex genetic and environmental interactions. METHODS: Three hundred fifty subjects (200 VSD children and 150 healthy controls) were recruited from different pediatric cardiac units. Pediatric clinical and demographic data were collected. A total of six variants, rs1017 (ISL1), rs7240256 (NFATc1), rs36208048 (VEGF), variant of HEY2, rs11067075 (TBX5) and rs1801133 (MTHFR) genes were genotyped by tetra-ARMS PCR and PCR-RFLP methods. RESULTS: The results showed that in cases, the rs1017 (g.16138A > T) variant in the ISL1 gene has an allele frequency of 0.42 and 0.58 respectively for the T and A alleles, and 0.75 and 0.25 respectively in the controls. The frequencies of the AA, TA and TT genotypes were, 52%, 11% and 37% in cases versus 21%, 8% and 71% respectively in the controls. For the NFATc1 variant rs7240256, minor allele frequency (MAF) was 0.43 in cases while 0.23 in controls. For the variant in the VEGF gene, genotype frequencies were 0% (A), 32% (CA) and 68% (CC) in cases and 0.0%, 33% and 67% respectively in controls. The allele frequency of C and A were 0.84 and 0.16 in cases and 0.83 and 0.17 respectively in controls. The TBX5 polymorphism rs11067075 (g.51682G > T) had an allelic frequency of 0.44 and 0.56 respectively for T and G alleles in cases, versus 0.26 and 0.74 in the controls. We did not detect the presence of the HEY2 gene variant (g.126117350A > C) in our pediatric cohort. For the rs1801133 (g.14783C > T) variant in the MTHFR gene, the genotype frequencies were 25% (CC), 62% (CT) and 13% (TT) in cases, versus 88%, 10% and 2% in controls. The ISL1, NFATc1, TBX5 and MTHFR variants were found to be in association with VSD in the Pakistani pediatric cohort whilst the VEGF and HEY2 variants were completely absent in our cohort. CONCLUSION: We propose that a wider programme of genetic screening of the Pakistani population for genetic markers in heart development genes would be helpful in reducing the risk of VSDs.

Accuracy of fetal echocardiography diagnosis and anticipated perinatal and early postnatal care in congenital heart disease in mid-gestation.

INTRODUCTION: The aim of this study was to determine discrepancies in fetal congenital heart disease (CHD) diagnoses and anticipated early postnatal care and outcomes. MATERIAL AND METHODS: A retrospective review of 462 randomly selected cases (23% of all cases) referred to a fetal cardiac assessment during the second trimester (mean 26 weeks) at the Children's Hospital in Helsinki between October 2010 and December 2020. Discrepancy between prenatal and postnatal CHD case evaluations was assessed with independently provided cardiac severity and surgical complexity scores. RESULTS: In all, 250 cases, 181 CHD and 69 normal, with complete prenatal and postnatal live birth data as well as seven fetal autopsy reports available were included in the analysis. There were 12 false normal and seven false abnormal prenatal assessments. The prenatally anticipated level of early neonatal care was actualized in 62% and prostaglandin infusion in 95%. In total, 32.7% (84/257) cardiac severity scores were discrepant and in 12,4% (32/257) cases the discrepancies were considered significant (≥ +/- 2 scores). Among significant discrepancies, CHD severity score was overestimated in 13 and underestimated in 19 in fetal assessment. Progression of CHD severity after mid-gestation and during early neonatal phase explained eight of 19 underestimated fetal assessments. The most common discrepant diagnostic categories included ventricular septal defects (n = 7), borderline ventricles (n = 7; 5 left heart, 1 right heart and 1 double outlet right ventricle/transposition of the great arteries), arch anomalies including coarctations (n = 5) and tricuspid valve dysplasias (n = 4) with a significant change in postnatal diagnoses and treatment. CONCLUSIONS: Although fetal CHD diagnosis and counseling is accurate and reliable in general, the study elaborates specific areas of uncertainty in clinical fetal cardiology practice that may be important to consider in fetal CHD evaluation and counseling provided in mid-gestation.

Characterization of blood-mimicking fluids for echocardiography imaging of ventricular septal defects.

PURPOSE: Ventricular septal defects (VSD) are congenital heart malformations and, in severe cases, they require complex interventions under echocardiography guidance. Heart phantoms can be helpful to train and to understand the complex hemodynamics of VSD. The goal of this study was to characterize the best blood mimicking fluids in such heart phantoms for modelling the hemodynamics of VSD patients using echocardiography. METHODS: Four fluid compositions were considered. Distilled water was used as a baseline, while the other three fluids were developed based on physical properties of human blood, such as the viscosity and the refractive index. Three bi-ventricular heart phantoms of three different pediatric patients with complex VSD were designed from preoperative CT imaging. Custom molds were printed in 3-D and the anatomical structure was casted in polyvinyl alcohol cryogel. The VSD in each heart phantom were observed using echocardiography and color Doppler imaging was used for the hemodynamic study. RESULTS: Heart phantoms with blood mimicking fluids of 30% glycerol and 27% glycerol, 10% sodium iodide were found to be anatomically realistic under echocardiography imaging. Hemodynamic parameters such as the pressure gradient and the volume of the shunt were characterized using color Doppler imaging. CONCLUSION: Proper composition of blood mimicking fluids are important for improving the realism in echocardiographic heart phantoms and they contribute to better understand the complex hemodynamic of VSD under echocardiography.

Complete heart block in a young adult with non-isolated congenitally corrected transposition of the great arteries: Case report.

Introduction and importance: Congenitally corrected transposition of the great arteries (ccTGA) or L-looped transposition of the great arteries (L-TGA) is a very rare and complex form of congenital heart disease. The majority of patients with ccTGA have at least one or more associated congenital heart disorders, essentially ventricular septal defects. Patients with ccTGA can remain asymptomatic for a long time and the diagnosis can sometimes be made late in life at the stage of complications. Case presentation: Here, we report a rare case of a 19-year-old patient, with no medical or surgical history, presenting a complete heart block as initial presentation of a ''non-isolated'' ccTGA. The diagnosis is made essentially by echocardiography.This case aims to show diagnostic difficulties of this rare congenital heart disease and be aware of the risk of its relative complications.

A reassessment of the anatomical features of multiple ventricular septal defects.

BACKGROUND: Over the course of time, new developments associated with the embryogenesis of the murine heart have served to clarify the developmental processes observed in the human heart. This evidence allows for the creation of a developmental framework for many congenital cardiac defects. AIMS: We aim to solidify the framework related to the categorization of both solitary and multiple ventricular septal defects. MATERIALS AND METHODS: Mice having genetic perturbation of the Furin enzyme have demonstrated perimembranous and juxta-arterial ventricular septal defects, permitting the inference to be made that these defects can co-exist with defects occurring within the apical muscular septum. RESULTS: Basis of developmental evidence, furthermore, all interventricular communications can be placed into one of three groups, namely those which are perimembranous, juxta-arterial, and muscular. All of the defects are described based on their borders as seen from the morphologically right ventricle. Our focus here will be on those defects within the muscular ventricular septum, recognizing that such defects can co-exist with those that are perimembranous. We discuss the differentiation of multiple discrete defects from those referred to as the "Swiss cheese" variant. CONCLUSIONS: As we show, appropriate surgical management requires an understanding of the specific terminology, as the surgical approach may differ depending on the combination of the individual defects. Data from the Society for Thoracic Surgeons revealed that both mortality and morbidity were increased in the setting of multiple as opposed to solitary ventricular septal defects.

A review of the therapeutic management of multiple ventricular septal defects.

BACKGROUND AND AIM: We showed in our anatomical review, ventricular septal defects existing as multiple entities can be considered in terms of three major subsets. We address here the diagnostic challenges, associated anomalies, the role and techniques of surgical instead of interventional closure, and the outcomes, including reinterventions, for each subset. METHODS: We reviewed 80 published investigations, noting radiographic findings, and the results of clinical imaging elucidating the location, number, size of septal defects, associated anomalies, and the effect of severe pulmonary hypertension. RESULTS: Overall, perioperative mortality for treatment of residual multiple defects has been cited to be between 0% and 14.2%, with morbidity estimated between 6% and 13%. Perioperative mortality is twice as high for perimembranous compared to muscular defects, with the need for reoperation being over four times higher. Perventricular hybrid approaches are useful for the closure of high anterior or apical defects. Overall, the results have been unsatisfactory. Pooled data reveals incidences between 2.8% and 45% for device-related adverse events. Currently, however, outcomes cannot be assessed on the basis of the different anatomical subsets. CONCLUSIONS: We have addressed the approaches, and the results, of therapeutic treatment in terms of coexisting discrete defects, the Swiss-cheese septum, and the arrangement in which a solitary apical muscular defect gives the impression of multiple defects when viewed from the right ventricular aspect. Treatment should vary according to the specific combination of defects.