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Background: As right heart catheterization (RHC) is invasive and not always accessible, this study developed a noninvasive model (P-echo) to predict shunt closure feasibility in adult congenital heart disease (ACHD) patients with post-tricuspid valve shunt defects (PTD), specifically isolated ventricular septal defects (VSD) and patent ductus arteriosus (PDA)., Methods: A retrospective analysis of 1474 VSD or PDA patients from 2012 to 2022 was conducted. Echocardiographic parameters were assessed, and key variables identified via LASSO regression. The P-echo model incorporated left to right velocity (LRv), right to left velocity (RLv), tricuspid regurgitation (TR), pulmonary artery diameter (PA), and RV/LV ratio. Its predictive performance was evaluated using ROC curve analysis., Results: The P-echo model demonstrated excellent predictive performance with AUC values of 0.975 (95% CI: 0.965-0.984) in the derivation set, 0.963 (95% CI: 0.937-0.989) in the validation set, and high accuracy in both PDA (AUC 0.975, 95% CI: 0.965-0.984) and VSD (AUC 0.958, 95% CI: 0.936-0.980) subsets. In the derivation set, the model categorized patients into low (9.1% closure rate), medium (70.9% closure rate), and high-risk groups (99.7% closure rate) for shunt closure feasibility. Calibration plots confirmed the model's accuracy. Decision curve analysis showed a higher net benefit across a range of threshold probabilities, indicating the clinical usefulness of the model., Conclusions: The P-echo model is a robust and reliable tool for predicting the feasibility of shunt closure in patients with PTD, offering a noninvasive alternative to RHC. This model can guide clinical decision-making and support individualized treatment strategies in ACHD management., (© 2024 Wiley Periodicals LLC.)

Tetralogy of Fallot is the most common «blue type» heart defect. The goals of surgical correction are closure of ventricular septal defect and reconstruction of right ventricular outflow tract. The results of reconstructions depend on several factors: age, material, management and size of conduit. Some patients may require redo surgery due to dysfunction after primary correction.

The development of a fully functional four-chambered heart is critically dependent on the correct formation of the structures that separate the atrial and ventricular chambers. Perturbation of this process typically results in defects that allow mixing of oxygenated and deoxygenated blood. Atrioventricular septal defects (AVSD) form a class of congenital heart malformations that are characterized by the presence of a primary atrial septal defect (pASD), a common atrioventricular valve (cAVV), and frequently also a ventricular septal defect (VSD). While AVSD were historically considered to result from failure of the endocardial atrioventricular cushions to properly develop and fuse, more recent studies have determined that inhibition of the development of other components of the atrioventricular mesenchymal complex can lead to AVSDs as well. The role of the dorsal mesenchymal protrusion (DMP) in AVSD pathogenesis has been well-documented in studies using animal models for AVSDs, and in addition, preliminary data suggest that the mesenchymal cap situated on the leading edge of the primary atrial septum may be involved in certain situations as well. In this chapter, we review what is currently known about the molecular mechanisms and animal models that are associated with the pathogenesis of AVSD., (© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.)

Objective: The aim of this study was to explore the efficacy and safety of minimally invasive transthoracic closure (MITC) in treating ventricular septal defect (VSD)., Methods: A total of 252 children with VSD were investigated between August 2013 and March 2015. Their electrocardiographic indices were monitored at different time points (before surgery [T0], immediately after surgery [T1], and 3 days/7 days/1 month/3 months/6 months after surgery [T2-6]) and followed up every year. Related adverse events also were recorded., Results: All children with VSD successfully underwent MITC. The average follow-up time was 5 years, during which no changes in the heart rate or corrected QT interval were found. The PR and QRS intervals were prolonged in the early postoperative period (T3); the SV1+RV5 amplitude decreased significantly at T6, and the left axis deviation significantly recovered at T6 (P < 0.05). A total of 32 patients developed arrhythmia within 3 days after surgery; however, no severe arrhythmia, including ventricular tachycardia, ventricular fibrillation, second-and third-degree atrioventricular block, complete left or right bundle branch block, and delayed arrhythmia, occurred during the follow-up period. Binary multivariate logistic regression revealed that the longer the surgery time and the larger the VSD diameter, the higher the risk of arrhythmia., Conclusions: The incidence of long-term arrhythmia after MITC is low, and the outcomes are good.

Untreated ventricular septal defects (VSDs) can lead to pulmonary arterial hypertension (PAH) characterized by elevated pulmonary artery (PA) pressure and vascular remodeling, known as PAH associated with congenital heart disease (PAH-CHD). Though previous studies have investigated hemodynamic effects on vascular mechanobiology in late-stage PAH, hemodynamics leading to PAH-CHD initiation have not been fully quantified. We hypothesize that abnormal hemodynamics from left-to-right shunting in early stage VSDs affects PA biomechanical properties leading to PAH initiation. To model PA hemodynamics in healthy, small, moderate, and large VSD conditions prior to the onset of vascular remodeling, computational fluid dynamics simulations were performed using a 3D finite element model of a healthy 1-year-old's proximal PAs and a body-surface-area-scaled 0D distal PA tree. VSD conditions were modeled with increased pulmonary blood flow to represent degrees of left-to-right shunting. In the proximal PAs, pressure, flow, strain, and wall shear stress (WSS) increased with increasing VSD size; oscillatory shear index decreased with increasing VSD size in the larger PA vessels. WSS was higher in smaller diameter vessels and increased with VSD size, with the large VSD condition exhibiting WSS >100 dyn/cm[Formula: see text], well above values typically used to study dysfunctional mechanotransduction pathways in PAH. This study is the first to estimate hemodynamic and biomechanical metrics in the entire pediatric PA tree with VSD severity at the stage leading to PAH initiation and has implications for future studies assessing effects of abnormal mechanical stimuli on endothelial cells and vascular wall mechanics that occur during PAH-CHD initiation and progression., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To investigate the midterm effect of exercise capacity and quality of life (QoL) of adult patients who underwent transthoracic device closure of ventricular septal defects (VSDs) and explore the gap in the quality of life and cardiopulmonary function between those patients and healthy people., Methods: From January 2010 to January 2015, 58 adult patients who underwent transthoracic device closure of VSD and 60 healthy people matched for age and sex were selected and analyzed. Echocardiography and exercise capacity tests were performed, and the MOS 36-item short-form health survey (SF-36) was used to investigate the changes in QoL., Results: Fifty-five patients completed the study. At the 1-year and 5-year follow-ups, the patients' left ventricular end-systolic and end-diastolic diameters were smaller than those preoperatively, but the difference was not statistically significant. In the QoL survey, the patients' scores after treatment showed a trend of improvement, and the improvement effect was not transient. After VSD closure, the difference in QoL between the patients and the control group was significantly reduced. However, in the exercise capacity test, the patients' results were still worse than those of the controls., Conclusion: Transthoracic device closure of VSDs is significant in improving adult patients' QoL at the midterm follow-up, reflected in their physical and psychological fields. However, they are still unable to achieve normal levels of peak exercise ability. Therefore, further exploration and interventions are worth considering., (© 2021. The Author(s).)

Transcatheter closure of ventricular septal defect (VSD) is an alternative treatment of surgery. However, because of the potential risk of the complete atrioventricular block (AVB) and the lack of available dedicated devices for VSD, this procedure rarely used most centers. Recently in Thailand, with distinct device design that may cause less clamp force and radial force, the Amplatzer TM Duct Occluder (ADO) II has become available for the closure of small VSDs. This is a retrospective review of 49 patients who underwent transcatheter VSD closure using ADO II at Songklanagarind hospital and Queen Sirikit National Institute of Child Health between January 2014 and December 2016 with an intention to report procedural success rate and 12-months-outcomes. Twenty-six cases were female. The median age and weight at treatment were 7.8 year (ranged from 1.9 to 25 year) and 25.1 kg (ranged from 11 and 57 kg). The majority (65.3%) of the VSD was a perimembranous defect. The mean diameter of LV entry was 5.2 ± 2.5 mm and the mean RV exit was 3.2 ± 0.8 mm. Four patients had more than 1 exit at RV orifice. The mean length of the VSD was 5.3 ± 1.8 mm. The mean aortic valve-VSD distance was 3.9 ± 2.0 mm. The mean tricuspid valve-VSD distance was 3.8 ± 2.5 mm. The procedural success achieved in all patients (100%), most of the implantations were performed in a retrograde approach (53.1%). The ADO II was successfully implanted in 2 cases (5%) who failed other devices in the first attempts. There were no major complications. There were no changes in the severity of tricuspid and aortic valve regurgitation immediate after implantation and at 12 months' follow-up. Three patients (6%) had tiny to small residual shunt at 12 months' follow-up. None of the patients had a complete heart block. However, new-onset of left bundle branch block and right bundle branch block occurred in 2 (5%) cases and 5 (10%) cases, respectively. With symmetrical double-disc design and softness of the device, the ADO II can be safely and effectively used for closure of the VSDs with the defect size less than 6 mm.

Transthoracic echocardiography (TTE) is noninvasive but can only be performed intermittently during fluoroscopy. In a prior study, we created a transducer holder device to allow for hemodynamic monitoring in the intensive care unit. The current study is the first instance of the use of a three-dimensional (3D)-printed TTE transducer holder, which is easily customized and personalized to a previous transducer holder at relatively low cost and short production time, to enable continuous TTE monitoring during device closure of an atrial septal defect (ASD) and ventricular septal defect (VSD). There were 14 ASD patients and 9 VSD patients scheduled to undergo device closure. The study's real-time TTE monitoring was performed by using a 3D-printed transducer holder over the course of the entire implantation procedure. There were 23 patients who successfully underwent septal closures using the 3D-printed holder that enabled real-time images over the entire procedure. The median duration for real-time TTE guidance was 15 minutes for the ASD and 36 minutes for the VSD and the median fluoroscopy time was 11 minutes for the ASD and 30 minutes for the VSD. One migrating VSD occluder and one case of aortic regurgitation after occluder deployment were noted by real-time TTE monitoring during the procedure. Our novel 3D-printed transducer holder can provide transesophageal echocardiography-like real-time imaging during device closure of an ASD and a VSD and may become a new alternative method in ASD and VSD closures. It can also prevent radiation exposure for the intervention team who would otherwise need to perform TTE during live fluoroscopy., Competing Interests: Conflicts of interest: Dr. Shen-Kou Tsai, an editorial board member at Journal of the Chinese Medical Association, had no role in the peer review process of or decision to publish this article. The other authors declare that they have no conflicts of interest related to the subject matter or materials discussed in this article., (Copyright © 2021, the Chinese Medical Association.)

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether the tricuspid valve detachment (TVD) approach to ventricular septal defect repair provides superior outcomes compared with the non-TVD approach. Altogether more than 54 papers were found using the reported search, of which 10 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. A total of 2059 participants were enrolled in the 10 studies, including 2 prospective studies and 8 retrospective studies. Six studies demonstrated a longer cardiopulmonary bypass time or aortic clamp time in the TVD group, whereas 4 studies showed no difference. Only 1 study reported a lower incidence of trivial tricuspid regurgitation in the TVD group, whereas the other 9 studies showed no significant difference. One study showed that a higher incidence of residual shunting occurred in those patients who had indications for TVD but did not perform detachment during surgery. No difference in postoperative residual shunting was demonstrated in the other 9 studies. We conclude that surgeons should be reassured that if TVD is required to repair the ventricular septal defect, although it may lead to longer cardiopulmonary bypass time and cross-clamp times, outcomes are equivalent in terms of the degree of tricuspid regurgitation and incidence of the residual ventricular septal defect., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The aim of this study was to determine factors associated with physical fitness (PF) in children who underwent surgery for congenital heart disease (CHD). Sixty-six children (7-14 years) who underwent surgery for ventricular septal defect (n = 19), transposition of great arteries (n = 22), coarctation of aorta (n = 10), and tetralogy of Fallot (n = 15) were included. All children performed PF tests: cardiorespiratory fitness, upper- and lower-limb muscle strength, speed, balance, and flexibility. Cardiac evaluation was done via echocardiography and cardiopulmonary exercise test. Factors related to child's characteristics, child's lifestyle, physical activity motivators/barriers, and parental factors were assessed. Linear regression analyses were conducted. The results showed no significant differences in physical activity (PA) level by CHD type. Boys had better cardiorespiratory fitness (difference = 1.86 ml/kg/min [0.51;3.22]) and were more physically active (difference = 19.40 min/day [8.14;30.66]), while girls had better flexibility (difference = - 3.60 cm [- 7.07;- 0.14]). Physical activity motivators showed an association with four out of six PF components: cardiorespiratory fitness, coefficient = 0.063 [0.01;0.11]; upper-limb muscle strength, coefficient = 0.076 [0.01;0.14]; lower-limb muscle strength, coefficient = 0.598 [0.07;1.13]; and speed, coefficient = 0.03 [0.01;0.05]. Age, sex, and motivators together reached a maximum adjusted R 2  = 0.707 for upper-limb strength. Adding other possible determinants did not significantly increase the explained variance. Apart from age and sex as non-modifiable determinants, the main target which might improve fitness would be the introduction of an intervention which increases the motivation to be active.

Objective: This study aimed to show the long-term results in patients who underwent unidirectional valve patch repair of ventricular septal defect with pulmonary artery hypertension., Methods: Thirty-five acyanotic patients aged 2 to 26 years (mean 9.3 years) with a large ventricular septal defect and elevated pulmonary vascular resistance (mean 9.5 Wood units) underwent surgery in Madani Heart Hospital. The medical records and clinical outcomes were reviewed from March 1998 to March 2017., Results: Five patients died in the first postoperative week. In the long-term follow-up (mean 11 years), two patients were lost to follow-up. Pulmonary artery hypertension gradually decreased in 17 patients within 6-12 months with significant improvement in right ventricular end-diastolic diameter, New York Heart Association functional class, and tricuspid regurgitation. Eleven patients with persistent pulmonary artery hypertension were divided into tolerable and non-tolerable groups. Six patients in the tolerable group had satisfactory conditions compared to before the operation, and gained weight with improved functional class despite echocardiographic findings of persistent elevated pulmonary artery pressure. One had a full-term delivery by caesarean section in the fifth postoperative year. Five patients in the non-tolerable group gradually developed right heart failure and complications such as extremity edema, ascites, pleural effusions, and died after 10-30 months., Conclusion: Although relatively high mortality occurred during long-term follow-up, surviving patients were in a better condition and functional class despite persistent pulmonary artery hypertension. Therefore, fear of persistent pulmonary artery hypertension should not prohibit surgery in this group of patients.

Reports thus far suggest a mild course for acute COVID-19 infection in children; however, its effects in vulnerable paediatric populations, including children with haemodynamically significant congenital heart disease, have rarely been reported. We therefore report on a 4-month-old Hispanic male with a moderate sized conoventricular ventricular septal defect and pulmonary overcirculation who presented with COVID-19-associated pneumonia.

Background: Most of congenital ventricular septal defects evolve towards spontaneous closure of different mechanisms depending on their location., Aim: To determine the prevalence and factors associated with spontaneous closure of congenital ventricular septal defects., Methods: We conducted a retrospective study of 1000 patients diagnosed with congenital ventricular septal defects in our department from January 2000 to December 2017., Results: After an average follow-up of 52.65 months (± 76.93 months), 183 (18.88%) of ventricular septal defects closed spontaneously. The average time for spontaneous closure was 45.78 months (76.34 months). 30.77% of trabecular ventricular septal defects (p<0.05) and 16.93% of perimembranous defects closed spontaneously (p=0.17). 28.5% of perimembranous defects associated with aneurysm formation versus 17.4% of those without associated aneurysm evolved to spontaneous closure (p<0.05). 65.6% of spontaneous closure occured during the first 3 years of life. In multivariate analysis, trabecular site [OR=2.85; CI (2.05-3.97)] and aneurysms of membranous septum [OR=1.9; CI (1.41-2.8)] were independent factors associated with spontaneous closure of defects., Conclusion: The highest VSD closure rate was observed during the first three years of life. Trabecular site and aneurysms tissue of membranous septum were found as independent factors associated with spontaneous closure.

This study aims to compare and analyze the health-related quality of life (HRQoL) of adult patients with ventricular septal defects (VSDs) who underwent transthoracic or transcatheter device closure.The HRQoL data of 30 patients who underwent transthoracic device closure for VSDs and 30 who underwent transcatheter device closure for VSDs were retrospectively evaluated before and one year after the procedure. The Medical Outcomes Study 36-Item Short-Form (SF-36), the Hospital Anxiety and Depression Scale (HADS), and a self-designed questionnaire were used as evaluation tools.After treatment, both groups showed significant improvements in SF-36 and HADS scores. After comparing the two groups regarding the SF-36, there was a significant difference in the two dimensions of vitality and mental health. There were no statistically significant differences in the HADS-A and HADS-D scores between these two groups. The results of the self-designed questionnaire also showed that the subjective feedback of the two groups was roughly the same. In the process of exploring the influential factors, we found that the scores of patients on most dimensions of the SF-36 in the two groups showed a significantly negative trend with increasing age. In terms of HADS scores, patients in both groups showed a tendency toward increasing scores with age.The HRQoL of adult patients undergoing transthoracic and transcatheter device closure for VSDs was similar, and the HRQoL was affected by the patient's own condition, so it is necessary to pay more attention to patients after device closure.

Aim & Objective: To report mid-term follow-up result of transcatheter closure of perimembranous Ventricular septal defect (VSD) in children weighing less than 10 kg using Amplatzer Duct Occlude-I (ADO-I) by left ventricular (LV) mid-cavity approach., Material & Method: This is retrospective review of 35 children weighing less than 10 kg with moderate to large perimembranous VSD who were selected for transcatheter closure of VSD using ADO-I in between October 2016 to September 2018. Mean age was 2.08 ± 0.67 years (mean ± SD) and mean weight was 7.2 ± 1.2 kg (mean ± SD). Procedure was done by crossing the VSD from right ventricular side instead of using the standard approach by forming arterio-venous loop. Average fluoroscopic time was 9.2 ± 2.9 min (mean ± SD) and mean procedure time was 34.1 ± 13.1 min (mean ± SD). Mean follow-up period was 8.7 months (3-12 months) RESULT: Device closure was successfully done in 32 patients. There was device induced aortic regurgitation (AR) in one case who was sent for surgery. One child was found to have complete heart block on first post-procedure day requiring permanent pace-maker implantation. During follow up none of them had any residual VSD, rhythm disturbance, AR and left or right ventricular outflow obstruction., Conclusion: Device closure can be successfully done in moderate to large perimembranous VSD using left ventricular mid cavity approach in small children. LV mid-cavity approach has advantages in terms of lesser hemodynamic instability, lesser fluoroscopy and lesser chance of device induced AR than standard technique particularly in smaller children., Competing Interests: Conflicts of interest All authors have none to declare., (Copyright © 2020 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.)

Background Ventricular septal defects (VSD), when treated correctly in childhood, are considered to have great prognoses, and the majority of patients are discharged from follow-up when entering their teens. Young adults were previously found to have poorer functional capacity than healthy peers, but the question remains whether functional capacity degenerates further with age. Methods and Results A group of 30 patients with surgically closed VSDs (51±8 years) with 30 matched, healthy control participants (52±9 years) and a group of 30 patients with small unrepaired VSDs (55±12 years) and 30 matched control participants (55±10 years) underwent cardiopulmonary exercise testing using an incremental workload protocol and noninvasive gas measurement. Peak oxygen uptake was lower in participants with closed VSDs than matched controls (24±7 versus 34±9 mL/min per kg, P <0.01) and with unrepaired VSDs than matched controls (26±5 versus 32±8 mL/min per kg, P <0.01). Patients demonstrated lower oxygen uptake from exercise levels at 20% of maximal workload compared with respective control groups ( P <0.01). Peak ventilation was lower in patients with surgically closed VSDs than control participants (1.0±0.3 versus 1.4±0.4 L/min per kg, P <0.01) but similar in patients with unrepaired VSDs and control participants ( P =0.14). Exercise capacity was 29% lower in older patients with surgically closed VSDs than healthy peers, whereas younger patients with surgically closed VSDs previously demonstrated 18% lower capacity compared with peers. Older patients with unrepaired VSDs reached 21% lower exercise capacity, whereas younger patients with unrepaired VSDs previously demonstrated 17% lower oxygen uptake than healthy peers. Conclusions Patients with VSDs demonstrate poorer exercise capacity than healthy peers. The difference between patients and control participants increased with advancing age-and increased most in patients with operated VSDs-compared with previous findings in younger patients. Results warrant continuous follow-up for these simple defects.

Surgery for D-transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction has continuously evolved to achieve optimal hemodynamic performance across the right and left ventricular outflow tracts, include predominantly native tissues, and preserve pulmonary valve function. Classically, three types of repair are applied: Rastelli, REV, and translocation procedures. The concept of translocation remains more radical and exposed to many modifications. Its extensive reconstructive nature extends its application to similar lesions with discordant ventriculo-arterial connection. We tried to compare the values and limitations of these surgical options, emphasizing how a more anatomical repair could impact the functional outcome.

Ventricular septal defects (VSDs) have recently demonstrated poorer functional outcome with disrupted ventricular contractility during exercise in young patients. It is not known whether this will change with age. Therefore, echocardiography was performed in older patients with congenital VSDs, either surgically closed or unrepaired and all without Eisenmenger physiology, to compare functional outcomes with healthy peers. Transthoracic echocardiography was performed at rest and during supine bicycle exercise, with tissue velocity Doppler for assessment of primary end points: isovolumetric acceleration and systolic velocities. In total, 30 surgically closed VSDs (51 ± 8 years) with 30 healthy controls (52 ± 9 years) and 30 unrepaired VSDs (55 ± 12 years) with 30 matched controls (55 ± 10 years) were included. Surgical patients displayed lower right ventricular (RV) systolic and early diastolic-filling velocities compared with controls, p <0.01, lower tricuspid annular plane systolic excursion (17.8 ± 5 vs 22.7 ± 3mm, p <0.01) and lower fractional area change (37.8 ± 6 vs 46.4% ± 7%, p <0.01). Unrepaired VSDs also had lower RV fractional area change than matched controls (39.9 ± 7 vs 48.4% ± 7%, p <0.01). Both patient groups had more tricuspid regurgitation and larger RV outflow tract dimensions than controls, p <0.01. During exercise, isovolumetric acceleration and systolic velocities were lower in both patient groups compared with controls, with the difference increasing with workload level. In conclusion, adults in their mid-50s with surgically closed or unrepaired VSDs have abnormal RV function at rest, with even more pronounced differences during exercise. These results suggest that a VSD, whether repaired early or considered hemodynamically insignificant, is not a benign lesion and continuous follow-up of adults is warranted., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Pulmonary hypertension (PH) presents unusual hemodynamic states characterized by abnormal high blood pressure in pulmonary artery. The objective of this study is to simulate how the hemodynamics develops in typical PH cases without treatment. A lumped-parameter circuit platform of human circulation system is set up to simulate hemodynamic abnormalities of PH in different etiologies and pathogenesis. Four typical cases are considered, which are distal pulmonary artery stenosis, left ventricular diastolic dysfunction, ventricular septal defect, and mitral stenosis. The authors propose regulation laws for chambers and vessels to adapt the abnormal hemodynamic conditions for each PH case. The occurrence and development of each PH case are simulated over time using the lumped-parameter circuit platform. The blood pressure, blood flow, pressure-volume relations for chambers and vessels are numerically calculated for each case of PH progression. The model results could be a quite helpful to understand the hemodynamic mechanism of typical PHs. Graphical Abstract.

An infant with transposition of the great arteries was paced for postoperative heart block (single-site, right ventricular [RV] epicardial). She developed severe left ventricular (LV) dysfunction and septal dyskinesis. Resynchronization was performed at the age of 4 with an LV epicardial lead and an RV septal endocardial lead. The endocardial lead was affixed to the interventricular septum, then tunneled through the RV free wall and attached to an abdominal pulse generator. QRS duration decreased (176 to 122 ms) and LV ejection fraction improved (26 to 61%) and remained stable for 8 years. We present a case of successful resynchronization in congenital heart disease using a transmural RV septal lead., (© 2020 Wiley Periodicals LLC.)

Objectives: To evaluate the safety, efficiency, and midterm outcomes of transcatheter perimembranous ventricular septal defect (pmVSD) closure using the new KONAR-MF™ VSD occluder., Background: Off-label percutaneous pmVSD closure is a well-established procedure with promising results. Yet, interventionists are still searching for the ideal device., Methods: Between June 2018 and March 2019, 20 patients with hemodynamically significant but restrictive-type pmVSD underwent an attempted transcatheter closure using the new KONAR-MF™. All implantations were performed retrogradely under general anesthesia, transoesophageal echocardiography, and fluoroscopic guidance. Prospective follow-up using transthoracic echocardiography and electrocardiogram was done until August 2019., Results: The median age was 6.4 years (8 months to 43.4 years), and the median body weight was 17.3 (9-74) kg. The mean defect size on the left ventricular aspect was 11.7 ± 2.8 mm. All devices were successfully and rapidly implanted. One device embolized in the pulmonary artery, 24 hr after implantation and was percutaneously retrieved with no complication. Over a mean follow-up period of 8.2 ± 3.0 months, complete closure was achieved in 84.2% of patients. One new onset of mild aortic regurgitation was detected before discharge and remained stable. Tricuspid valve regurgitation, complete heart block, major complication, and death were not observed., Conclusions: Transcatheter pmVSD closure using the KONAR-MF™ can be successfully performed in adult and pediatric patients. It is a safe and promising device, designed to provide high conformability to septal defects with a lower risk of heart block. Retrograde implantation allows procedural flexibility, efficient deliverability, and control of valvular interferences., (© 2019 Wiley Periodicals, Inc.)

Objective: To visualize and quantify the atrioventricular conduction axis in the setting of ventricular septal defect using phase contrast computed tomography., Methods: We used the SPring-8 synchrotron radiation facility in Hyogo prefecture in Japan, processing and reconstructing the data with 3-dimensional software., Results: We studied 8 hearts obtained from patients known to have had ventricular septal defects, aged from 6 to 150 days, with a median of 24.5 days. None of the individuals, however, had undergone corrective surgery. The penetrating bundle was found at a median of 1.43 mm from the septal crest, with a range of 0.99 to 1.54 mm. The distance to the nonbranching bundle to the right ventricular endocardium was 1.10 mm, with a range from 0.49 to 2.49 mm, to the origin of the left bundle branch was 2.46 mm, with a range from 1.7 to 3.18 mm, and to the origin of the right bundle branch was 2.34 mm, with a range from 0.50 to 2.59 mm. The median distance from the edge of the caudal limb of the septomarginal trabeculation to the right bundle branch was 1.04 mm, with a range from 0.81 to 1.16 mm., Conclusions: We were able to show the precise location of the axis, with our findings suggesting that longitudinal sutures placed along the posteroinferior rim should be effective in avoiding iatrogenic injury, but sutures should not be placed in the valley between the limbs of the septomarginal trabeculation., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Pulmonary vascular disease resulting from CHDs may be the most preventable cause of pulmonary artery hypertension worldwide. Many children in developing countries still do not have access to early closure of clinically significant defects, and the long-term outcomes after corrective surgery remain unclear. Focused on long-term results after isolated ventricular septal defect repair, our review sought to determine the most effective medical therapy for the pre-operative management of elevated left-to-right shunts in patients with an isolated ventricular septal defect., Methods: We identified articles specific to the surgical repair of isolated ventricular septal defects. Specific parameters included the pathophysiology and pre-operative medical management of pulmonary over-circulation and outcomes., Results: Studies most commonly focused on histologic changes to the pulmonary vasculature and levels of thromboxanes, prostaglandins, nitric oxide, endothelin, and matrix metalloproteinases. Only 2/44 studies mentioned targeted pharmacologic management to any of these systems related to ventricular septal defect repair; no study offered evidence-based guidelines to manage pulmonary over-circulation with ventricular septal defects. Most studies with long-term data indicated a measurable frequency of pulmonary artery hypertension or diminished exercise capacity late after ventricular septal defect repair., Conclusion: Long-term pulmonary vascular and respiratory changes can occur in children after ventricular septal defect repair. Research should be directed at providing an evidenced-based approach to the medical management of infants and children with ventricular septal defects (and naturally all CHDs) to minimise consequences of pulmonary artery hypertension, particularly as defect repair may occur late in underprivileged societies.

To better understand the evolvement of the pulmonary dysfunction in the aging ventricular septal defect (VSD) patient, we invited adult patients with a congenital VSD and healthy age- and gender-matched controls for static and dynamic spirometry, impulse oscillometry, multiple breath washout, and diffusion capacity for carbon monoxide testing. Primary outcome was forced expiratory volume in 1 second. In total, 30 patients with a surgically corrected VSD (mean age 51 ± 8 years), 30 patients with a small, unrepaired VSD (mean age 55 ± 1 years), and 2 groups of 30 age- and gender-matched, healthy controls (mean age 52 ± 9 years and 55 ± 10 years, respectively) were included. Median age of radical surgery was 6.3 (total range: 1.4 to 54) years and median follow-up after surgery was 40 years (total range: 3.4 to 54). Compared with healthy matched controls, surgically corrected VSD patients had lower forced expiratory volume in 1 second, 87 ± 19% versus 105 ± 14% of predicted, p <0.01, lower forced vital capacity, 92 ± 16% versus 107 ± 13% of predicted, p <0.01, and lower peak expiratory flow, 100 ± 21% versus 122 ± 16% of predicted, p <0.01. Furthermore, corrected VSD patients revealed increased airway resistance in the small airways, 30 ± 22% versus 15 ± 14% of total resistance, p <0.01, and reduced diffusion capacity, 84 ± 12% versus 101 ± 11%, p <0.01. Patients with a small, unrepaired VSD had comparable pulmonary function to their matched controls. In conclusion, VSD patients 40 years of age or older demonstrated significant impaired pulmonary function in terms of reduced dynamic pulmonary function, increased airway resistance in the small airways, and reduced diffusion capacity up to 54 years after defect closure compared with healthy age- and gender-matched controls., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: Biventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect is preferred, but previously developed surgical procedures are complicated and associated with high mortality and morbidity. We developed a technique using an intraventricular conduit to connect the ventricular septal defect and the aorta in this anomaly in patients aged more than 2 years., Methods: Thirty-one patients (age 2-23 years; median, 5.4) with double-outlet right ventricle with noncommitted ventricular septal defect underwent biventricular repair with intraventricular conduit. A 16-mm or 19-mm polytetrafluoroethylene (Gore-Tex; WL Gore & Associates, Flagstaff, Ariz) vascular prosthesis was used to construct the intraventricular conduit rerouting the ventricular septal defect to the aorta, with enlargement of the ventricular septal defect and resecting the hypertrophic muscular bands in the bilateral conus when necessary. Follow-up was made in all patients with a median duration of 93 months (range, 8-140 months)., Results: One patient died during hospitalization and 1 patient died at 8 months after operation, making the mortality 6.5%. The peak pressure gradient across the left ventricular outflow tract was less than 30 mm Hg in all patients but 1 (3.3%). In the last patient, it increased from 16 mm Hg early after operation to 50 mm Hg at 7 years follow-up. The peak pressure gradient across the right ventricular outflow tract ranged from 6 to 30 mm Hg in all patients. One patient had moderate mitral regurgitation with New York Heart Association class II. One patient had preoperative severe pulmonary arterial hypertension (mean pressure, 50 mm Hg) and was treated with bosentan. Other patients were in New York Heart Association class I., Conclusions: Biventricular repair with intraventricular conduit is a relatively simple and safe procedure for patients aged more than 2 years with double-outlet right ventricle with noncommitted ventricular septal defect, with excellent early and midterm outcomes., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Transcatheter ventricular septal defect (VSD) closure is a safe and efficacious alternative to surgery. However, its benefits in asymptomatic or minimally symptomatic patients remain unknown., Methods: Sixty patients with VSD aged 12 to 60 years underwent cardiopulmonary exercise test and echocardiography 1 day before transcatheter VSD closure and 6 months after intervention (closure group). Thirty patients who did not receive the intervention underwent the same evaluations over 6 months (observation group)., Results: No significant change in exercise function was observed after VSD closure, except for increased peak oxygen (O 2 ) pulse (absolute increase: 0.4±1.4 mL/beat). Left ventricular end-diastolic dimension and mitral peak early filling velocity-to-early diastolic annular velocity ratio decreased (absolute decrease: 0.3±0.6 cm and 0.7±1.9, respectively). Despite unchanged overall peak O 2 consumption, 33% of closure group patients exhibited clinically significant improvements in peak O 2 consumption (>10% increase relative to baseline). This was not related to the pulmonary flow-to-systemic flow ratio or baseline exercise capacity. By contrast, all exercise function parameters deteriorated significantly in the observation group. Subgroup analysis revealed that patients with a baseline left ventricular end-diastolic dimension Z score of >2 exhibited a significantly greater improvement in peak O 2 consumption, peak O 2 pulse, and oxygen uptake efficiency slope than did the observation group., Conclusions: Compared with conservative management, transcatheter VSD closure prevents deterioration in exercise capacity and promotes left ventricular reverse remodeling in asymptomatic or minimally symptomatic patients. These benefits are most prominent in patients whose left ventricular end-diastolic dimension Z score before intervention is >2, irrespective of baseline peak O 2 consumption and pulmonary flow/systemic flow ratio. Registration: URL: http://www.clinicaltrials.gov. Unique identifier: NCT03127748.

Objectives: Clinical studies have revealed decreased exercise capacity in adults with small, unrepaired ventricular septal defects. Increasing shunt ratio and growing incompetence of the aortic and pulmonary valve with retrograde flow during exercise have been proposed as reasons for the previously found reduced exercise parameters. With MRI, haemodynamic shunt properties were measured during exercise in ventricular septal defects., Methods: Patients with small, unrepaired ventricular septal defects and healthy peers were examined with MRI during exercise. Quantitative flow scans measured blood flow through ascending aorta and pulmonary artery. Scans were analysed post hoc where cardiac index, retrograde flows, and shunt ratio were determined., Results: In total, 32 patients (26 ± 6 years) and 28 controls (27 ± 5 years) were included. The shunt ratio was 1.2 ± 0.2 at rest and decreased to 1.0 ± 0.2 at peak exercise, p < 0.01. Aortic cardiac index was lower at peak exercise in patients (7.5 ± 2 L/minute/m2) compared with controls (9.0±2L l/minute/m2), p<0.01. Aortic and pulmonary retrograde flow was larger in patients during exercise, p < 0.01. Positive correlation was demonstrated between aortic cardiac index at peak exercise and previously established exercise capacity for all patients (r = 0.5, p < 0.01)., Conclusions: Small, unrepaired ventricular septal defects revealed declining shunt ratio with increasing exercise and lower aortic cardiac index. Patients demonstrated larger retrograde flow both through the pulmonary artery and the aorta during exercise compared with controls. In conclusion, adults with unrepaired ventricular septal defects redistribute blood flow during exercise probably secondary to a more fixed pulmonary vascular resistance compared with age-matched peers.

Objectives: To demonstrate safety and efficacy of using different generations of softer Amplatzer™ devices for ventricular septal defect (VSD) closure to avoid serious complications at follow-up., Background: Transcatheter closure of perimembranous ventricular septal defects (PmVSD) is a well-established procedure; however, it is associated with unacceptable incidence of complete heart block. Great advantages have been achieved by using softer devices for VSD transcatheter closure. The first and second generation of Amplatzer™ occluders (AVP II, ADO, and ADO II) seem to offer a safe and attractive alternative for this procedure. These devices can be delivered using either an arterial (retrograde) or venous (prograde) approach., Methods and Results: Patients with congenital PmVSD who underwent transcatheter closure using ADO, ADO II, and AVP II devices were included. Primary end point was to determine efficacy and safety of these generations of devices and to determine the incidence of complications at follow-up (complete AV block and aortic/tricuspid/mitral regurgitation). One hundred and nineteen patients underwent VSD closure at a median age of 5 years (8 months-54 years). During the catheterization, there were only minor complications and at follow-up of 36 ± 25.7 months (up to 99 months), the closure rate was high of 98.3% and freedom from AV block was 100%., Conclusions: The use of softer Amplatzer™ devices is a good alternative to achieve PmVSD closure safely with no risk of AVB during the procedure or at midterm follow-up., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2020 Roberto Mijangos-Vázquez et al.)

Competing Interests: Competing interests: None declared.

Background: Transcatheter perimembranous ventricular septal defect (pmVSD) closure remains challenging and is seldom used in France given the risk of atrioventricular block (AVB). pmVSD closure with the Nit-Occlud Lê VSD coil was recently introduced in France as an alternative to occluder devices., Aims: To study the safety and feasibility of pmVSD closure with the Nit-Occlud Lê VSD coil., Methods: All consecutives cases of pmVSD closure with the Nit-Occlud Lê VSD coil in 20 tertiary French centres were included between January 2015 and December 2018., Results: Among 46 procedures in five centres, indications for pmVSD closure were left ventricle overload (76.1%), exertional dyspnoea (17.4%), history of infective endocarditis (4.3%) and mild pulmonary hypertension (2.2%). The median (interquartile [IQR]) age of the patients was 13.9 (5.7-31.8) years. Aneurismal tissue was identified in 91.3% of patients. VSD median (IQR) size was 8 (7-10) mm on the left ventricle side and 5 (4-6) mm on the right ventricle side. Implantation was successful in 40 patients (87.0%; 95% confidence interval [CI] 73.7-95.1%). Severe complications occurred in six patients (13.0%, 95% CI 4.9-26.3%), mainly severe haemolysis (8.7%, 95% CI 2.4-20.8%). One aortic valve lesion required surgical aortic valvuloplasty. Occurrence of severe complications was significantly related to the presence of haemolysis (P=0.001), residual shunt (P=0.007) and multi-exit VSD (P=0.005). Residual shunt was observed in 40% of cases with the implanted device shortly after closure and 15% after a median follow-up of 27 months. No immediate or delayed device embolization or complete AVB was recorded., Conclusion: pmVSD closure with the Nit-Occlud Lê VSD Coil is feasible in older children and adults. However, residual shunting (leading to haemolysis) is a dreaded complication that should not be tolerated. pmVSD closure with the Nit-Occlud Lê VSD as a therapeutic strategy remains controversial and is limited to selected patients., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Ventricular performance and its loading condition change drastically after surgical correction of congenital heart defect. Pressure-volume loops analysis can provide quantitative information about ventriculo-arterial coupling (VAC) indicating the interaction between ventricular contractility and loading condition. Therefore, we investigated changes in VAC after corrective surgery for ventricular septal defect (VSD)/tetralogy of Fallot (TOF), and implication of ventriculo-arterial decoupling as a prognostic factor of post-operative outcomes. In children with VSD/TOF, pre- and post-operative arterial elastance (Ea), end-systolic ventricular elastance (Ees) and VAC (Ea/Ees) were non-invasively estimated using echocardiographic parameters. Post-operative outcomes included maximum vasoactive-inotropic score, the duration of mechanical ventilation and hospital stay. Preoperatively, patients with VSD had significantly lower Ea and Ees than patients with TOF; however, VAC were preserved in both. In patients with VSD, post-operative Ea increased disproportionately to change in Ees, resulting in increased VAC. Post-operative higher VAC in patients with VSD was independently associated with maximum vasoactive-inotropic score (odds ratio [OR] 63.9; 95% Confidence Interval [CI] 4.0-553.0; P = 0.003), prolonged mechanical ventilation (OR 6.3; 95% CI 1.1-37.8; P = 0.044) and longer hospitalization (OR 17.6; 95% CI 1.6-187.0; P = 0.018). In patients with TOF, Ea and Ees reduced post-operatively; however, VAC remained unchanged and was not associated with post-operative outcomes. Despite of different loading condition, preoperative VAC maintained in both of VSD and TOF. However, particularly in VSD patients, abrupt increase in post-operative loading state induces contractility-load decoupling, which leads to worse post-operative outcomes.

Management strategy for patients of ventricular septal defect and pulmonary atresia (VSD/PA) with hypoplastic pulmonary arteries presenting in late childhood or adolescence is still controversial. We present our experience with the use of right ventricle-pulmonary artery shunt (RV-PA) in management of this entity. Between January 2014 and April 2018, 25 patients of VSD/PA underwent valveless RV-PA shunt at our center. The size of the RV to PA shunt was calculated as half the expected diameter of the main pulmonary artery. We retrospectively reviewed the data from hospital records. Follow-up data were recorded from outpatient records or via telephone. Mean age of the cohort was 12.25 ± 3.18 years. There was 1 early and 1 interstage mortality. None of the patient developed acute renal failure, ventricular dysfunction, and arrhythmias. At interstage follow-up of 8.28 ± 3.7 months, both Nakata index (from 66.23 ± 24.12 to 185.8 ± 58 mm 2 /m 2 ) and McGoon ratio (0.9 ± 0.22 vs 49 1.84 ± 0.4) increased significantly compared to preoperative value, whereas RPA-LPA ratio was not significantly changed (1.095 ± 0.39 vs 1.01 ± 0.56, P = 0.63). Prerepair pulmonary vascular resistance in 17 patients, who underwent complete repair, was 2.9 ± 0.69 woods unit/m 2 . Postrepair right ventricle-left ventricle pressure ratio was 0.5 ± 0.14. There was no early or late mortality and none of the patient required conduit revision or VSD fenestration. On follow-up of 25.75 ± 17.94 months, 16 patients were in NYHA I and 1 patient was in NYHA II. Appropriate-sized RV-PA shunt is an effective strategy for achieving balanced pulmonary artery growth in VSD/PA with hypoplastic pulmonary arteries presenting late without the risk of pulmonary over circulation or systemic malperfusion., (Copyright © 2019. Published by Elsevier Inc.)

Background: Transcatheter closure of perimembranous ventricular septal defects is one of the greatest challenges in interventional cardiology. Short- and midium-term follow-up data for large samples are limited. This report presents our experience with transcatheter closure of perimembranous ventricular septal defects using an occluder., Methods: Two hundred fifty-three patients included in the database of the Second Affiliated Hospital and Yuying Children's Hospital from January 2011- December 2015 with transcatheter closure of perimembranous ventricular septal defects and discharged from follow-up. All patients were invited for clinical and transthoracic echocardiography, electrocardiogram, and thoracic radiography check-up., Results: Device implantation was successful in 252 of 253 patients (99.6%). The median age was 42 months (range 27-216 months). The median follow-up duration was 36 months (range 6-60 months). The mean defect diameter was 3.5 ± 1.4 mm and the mean size of the ventricular septal defect rim below the aortic valve was 3.7 ± 1.8 mm. The mean diameter of the devices used was 4 mm. Thirty-seven patients developed arrhythmia after the procedure and recovered within 24 months; four patients had hemolysis and four had moderate tricuspid valve regurgitation. No other serious adverse event occurred during the follow-up period., Conclusion: Transcatheter closure of perimembranous ventricular septal defects using an occluder is safe and effective in most patients.

Objectives: D-transposition of the great arteries and l-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction are complex biventricular congenital heart diseases for which decision-making regarding surgical strategy remains challenging. We investigated the intermediate-term outcomes of Fontan versus biventricular procedures in these patients., Methods: We analyzed 129 patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 85) or l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 44) and 2 functional ventricles from Australia who had primary surgical management (29 Fontan, 100 biventricular repair) undertaken between 1990 and 2015., Results: Median operative age of patients was 2.9 years (range, 0.2-26.8 years). During a median follow-up of 6.2 years (range, 2 days to 25.8 years), 9 patients died after biventricular repair (3 early and 6 late deaths). One patient received a transplant 1.2 years after Fontan completion. Overall transplant-free survivals at 1, 5, 10, and 15 years were 95%, 93%, 92%, and 90%, respectively. Overall reintervention-free survivals at 1, 5, 10, and 15 years were 79%, 64%, 45%, and 29% respectively. Biventricular repair tended to be associated with a higher rate of death, transplantation, or reintervention than the Fontan pathway (hazard ratio, 1.83; 95% confidence interval, 0.90-3.71; P = .10). Some 73% of transplant-free survivors had New York Heart Association class I. Functional status was similar between the Fontan and biventricular groups., Conclusions: Intermediate-term outcomes were comparable between patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction and patients with l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction. Both Fontan and biventricular pathways are associated with excellent mortality and functional outcomes. Biventricular patients have a greater risk of reintervention. The Fontan procedure is a viable option when anatomic risk factors preclude biventricular repair., (Copyright © 2019 The American Association for Thoracic Surgery. All rights reserved.)

Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74-86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein's anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein's anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.

Objective: Despite decreasing mortality, functional impairments in children with transposition of the great arteries (TGA) are still a concern. This study analyses health-related physical fitness (HRPF), arterial stiffness and health-related quality of life (HRQoL) in children with anatomically repaired TGA regarding congenital ventricular septal defect (VSD) and coronary patterns., Patients and Methods: 68 children with anatomically repaired TGA with or without VSD (12.9±3.7 years, 19.1% female) were investigated between August 2014 and October 2017. HRPF was assessed by five tests of the FITNESSGRAM, arterial stiffness was measured by oscillometric measurement using Mobil-O-Graph and HRQoL was analysed with a self-report questionnaire (KINDL-R). All test results were compared with a healthy reference cohort (n=2116, 49.1% female) adjusted for sex and age., Results: Children with anatomically repaired TGA had significantly worse HRPF (z-score: -0.58±0.81, p<0.001), increased pulse wave velocity (TGA: 4.9±0.3 m/s vs healthy: 4.8±0.3 m/s, p=0.028) and central systolic blood pressure (TGA: 105.9±5.8 mm Hg vs healthy: 103.3±5.7 mm Hg, p=0.001). No difference was found for HRQoL between the two groups (total HRQoL score: TGA: 76.5±10.2 vs healthy: 75.2±10.1, p=0.315). Neither the coronary pattern nor a congenital VSD resulted in significant differences in all functional outcomes., Conclusion: Children with anatomically repaired TGA exhibit impaired HRPF and increased arterial stiffness whereas their HRQoL is normal. The underlying coronary pattern seems to have no influence on the functional outcome, nor does an accompanied congenital VSD., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)