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.

Influence of percutaneous catheter intervention for congenital perimembranous ventricular septal defects in children on the cardiac conduction system and associated risk factors: a meta-analysis.

BACKGROUND: The aim of this study was to investigate adverse outcomes and risk factors for the cardiac conduction system in children with perimembranous ventricular septal defects (pmVSDs) who had been treated by catheter intervention. METHOD: PubMed, EMBASE, Web of Science, and the Cochrane Library were searched for studies in English on interventional treatment of pmVSDs in pediatric patients published up to the end of October 15, 2020. We used random- or fixed-effect models to obtain pooled estimates of the success rate and postoperative complications. RESULTS: A total of 1650 pediatric patients from 8 publications were included, with a mean age ranging from 3.44 to 8.67 years old. The pooled estimate of successful implantation was 98.2% (95% CI 97.1-99.4%, I2 = 69.4%; P < 0.001), and the incidence of cardiac conduction system complications was 17.4% (95% CI 8.4-26.4%, I2 = 96.1%; P < 0.001), among which the incidence of heart block was 14.8% (95% CI 6.4-23.3%, I2 = 96.9%; P = 0.001). The incidence of impulse formation disorders was 4.1% (95% CI 0.7-7.6%, I2 = 91.7%; P = 0.019), and the incidence of complete atrioventricular block was 0.8% (95% CI 0.3-13%, I2 = 0.0%; P = 0.001). Risk factors for newly emerging arrhythmias included the VSD size MD = 0.89 (95% CI 0.46-1.32, I2 = 0%; P < 0.0001) and device size MD = 1.26 (95% CI 0.78-1.73, I2 = 0%; P < 0.00001). CONCLUSIONS: Percutaneous catheter intervention is safe and effective in treating pediatric patients with pmVSD, and the risk factors leading to arrhythmias include the sizes of the pmVSD and device.

Prenatal diagnosis of isolated perimembranous ventricular septal defects undergoing primary surgical repair in infancy.

OBJECTIVE: We retrospectively analyzed our center's experience with the prenatal diagnosis of isolated perimembranous ventricular septal defects that underwent primary surgical repair in infancy. METHODS: We identified patients born in Southern Nevada, between October 2012 and October 2020, with prenatal care that underwent surgical closure of an isolated large perimembranous ventricular septal defect between 1 and 12 months of age. The description at surgery defined ventricular septal defect morphology. We included only those with situs solitus, levocardia without dextroposition, and without any other cardiovascular abnormality. We analyzed prenatal detection rates for each of the eight years. RESULTS: We identified 81 patients. Of the 81, 35 (43%) had trisomy 21. We identified no other aneuploidies in those that underwent surgical repair; however, 1 had a 15q13.3 deletion syndrome, and 1 had a 22 q11.2 deletion syndrome. Of the 81, 27 (33%) overall were prenatally diagnosed. Increasing prenatal detection rates strongly correlated with time (R = 0.92, p = .002). CONCLUSIONS: Trisomy 21 is common in isolated perimembranous ventricular septal defects undergoing primary repair in infancy. Further, prenatal detection rates significantly improved over time, up to 65% detection in the current years.

Results of two different echocardiography-guided approaches to closure of perimembranous ventricular septal defects.

OBJECTIVES: The feasibility of mini-invasive closure of perimembranous ventricular septal defects has been proven, but can cause surgical incision or sternum injury. A relevant but, to date, unanswered question is whether there exists a treatment without surgical trauma, radiation exposure and arterial complications. METHODS: From May 2017 to January 2020, a total of 449 patients with perimembranous ventricular septal defect [mean age 5.0 ± 6.1 years (range 0.8-52.0 years)] were involved in this study and underwent 2 different echocardiography-guided operative procedures [percutaneous device closure (group A) or percardiac device closure (group B)] based on the patients' or their parents' choice. The clinical data were collected and a retrospective analysis was performed. RESULTS: Fifty-five (96.5%) cases were successfully occluded in group A, and 2 (3.5%) patients were converted to percardiac device closure; 379 (96.7%) patients in group B underwent percardiac device closure, and 13 patients (3.3%) were turned to open-heart surgery after occlusion procedure failure. There were statistically significant differences (P < 0.05) between the 2 groups in operation time, postoperative hospitalization time and blood transfusion requirement. No acute complications or severe adverse events (death, valve injury, complete atrioventricular block and embolism) occurred either in the early period or during the follow-up. CONCLUSIONS: Percutaneous device closure can achieve the same validity and safety as percardiac device closure for treating perimembranous ventricular septal defects with a more rapid recovery and less trauma.

Transcatheter Device Closure of Perimembranous Ventricular Septal Defect in Pediatric Patients: Long-Term Outcomes.

AIMS: The aim of this study is to evaluate the safety and efficacy of transcatheter device closure of perimembranous ventricular septal defects in pediatric patients at long-term follow-up. MATERIALS AND METHODS: We prospectively studied 376 patients with perimembranous VSDs between September 2008 and December 2015 who underwent percutaneous closure at our center. Transthoracic echocardiography (TTE) and electrocardiogram were done before and after the procedure in all the patients. All patients were subjected to follow-up evaluation at 1, 3, 6, 12 months, and annually thereafter with TTE and electrocardiogram. RESULTS: A total of 376 patients (210 males and 166 females) underwent transcatheter closure of perimembranous VSD. Mean age of patients was 8.67 ± 3.02 (range 3-18 years) and mean weight was 21.15 ± 8.31 (range 8-65 kg). The procedure was carried out successfully in 98.93% of patients with no reported mortality. Rhythm disturbances occurred in 8.5% of patients after the procedure which included three cases of complete atrioventricular block. CONCLUSION: This study shows that in experienced hands transcatheter closure of perimembrnous VSD is safe and effective at long-term follow-up. With minimal morbidity and no mortality, the transcatheter is an effective alternative to surgical closure in selected patients.

Transcatheter closure, mini-invasive closure and open-heart surgical repair for treatment of perimembranous ventricular septal defects in children: a protocol for a network meta-analysis.

INTRODUCTION: Both transcatheter device closure and surgical repair are effective treatments with excellent midterm outcomes for perimembranous ventricular septal defects (pmVSDs) in children. The mini-invasive periventricular device occlusion technique has become prevalent in research and application, but evidence is limited for the assessment of transcatheter closure, mini-invasive closure and open-heart surgical repair. This study comprehensively compares the efficacy, safety and costs of transcatheter closure, mini-invasive closure and open-heart surgical repair for treatment of pmVSDs in children using Bayesian network meta-analysis. METHODS AND ANALYSIS: A systematic search will be performed using Chinese Biomedical Literature Database, China National Knowledge Infrastructure, PubMed, EMBASE.com and the Cochrane Central Register of Controlled Trials to include random controlled trials, prospective or retrospective cohort studies comparing the efficacy, safety and costs of transcatheter closure, mini-invasive closure and open-heart surgical repair. The risk of bias for the included prospective or retrospective cohort studies will be evaluated according to the risk of bias in non-randomised studies of interventions (ROBINS-I). For random controlled trials, we will use risk of bias tool from Cochrane Handbook version 5.1.0. A Bayesian network meta-analysis will be conducted using R-3.3.2 software. ETHICS AND DISSEMINATION: Ethical approval and patient consent are not required since this study is a network meta-analysis based on published trials. The results of this network meta-analysis will be submitted to a peer-reviewed journal for publication. PROTOCOL REGISTRATION NUMBER: CRD42016053352.

Genome-wide methylation analysis identifies novel CpG loci for perimembranous ventricular septal defects in human.

AIM: Congenital heart diseases are the most common birth defects worldwide and leading cause of infant mortality. The perimembranous ventricular septal defect is most prevalent. Epigenetics may provide an underlying mechanism of the gene-environment interactions involved. MATERIALS & METHODS: We examined epigenome-wide DNA methylation using the Illumina HumanMethylation450 BeadChip in 84 case children and 196 control children. RESULTS: We identified differential methylation of a CpG locus (cg17001566) within the PRDM16 gene after Bonferroni correction (p = 9.17 × 10-8). This was validated by bisulfite pyrosequencing. PRDM16 functions as a repressor of TGF-ß signaling controlling tissue morphogenesis crucial during cardiogenesis. At 15% false-discovery rate, we identified seven additional CpG loci. CONCLUSION: These findings provide novel insights in the pathogenesis of perimembranous ventricular septal defect, which is of interest for future prediction and prevention.

Application of perimembranous ventricular septal defects closure solely by femoral vein approach under transesophageal echocardiography guidance / 中南大学学报(医学版)

Objective:To investigate the feasibility and safety of perimembranous ventricular septal defects (PmVSD) closure solely by femoral vein approach under transesophageal echocardiography (TEE) guidance.Methods:From January 1,2014 to May 31,2016,26 patients with PmVSD in Second Xiangya Hospital were selected,with age at 3.2-6.0 (4.3±0.7) years old and body weight at 15.0-19.5 (16.7±1.4) kg.The diameter of VSD was 3.5-4.8 (4.1±0.3) mm.All patients were treated by percutaneous PmVSD closure solely by femoral vein approach under TEE guidance.The effect of the procedure was evaluated by TEE and transthoracic echocardiography (TTE).The clinical follow-up study was conducted by TTE at 1,3,6 and 12 month (s) after the procedure.Results:Twenty cases were successfully treated with percutaneous PmVSD closure solely by femoral vein approach under TEE guidance,and the success rate was 76.9％.Six patients were converted to perventricular closure under TEE guidance because the guide wire in two cases or catheter in other cases could not pass through PmVSD.The diameter of symmetrical VSD occluder was 6.0-7.0 (6.2±0.4) mm.The procedural time was 12.0-64.0 (26.8±6.3) min.The residence time at ICU was 1.8-2.4 (26.8±6.3) h.The in-hospital time was 4.0-5.0 (4.4±0.5) d.There were 3 patients with immediate post-operative trivial residual shunt and incomplete right bundle branch block (IRBBB).All patients survived with no peripheral vascular injury or complications such as tricuspid regurgitation,pericardial tamponade and pulmonary infection.The residual shunt disappeared in 3 patients and IRBBB became normal rhythm in 3 patients at 1 month follow-up time point.No patients suffered from occluder malposition,residual shunt,pericardial effusion,arrhythmia (atrio-ventricular block),aortic valve regurgitation and tricuspid regurgitation.Conclusion:TEE-guided percutaneous PmVSD closureby femoral vein approach is safe and effective.

Clinical analysis of electrocardiogram after transcatheter closure of perimembranous ventricular ;septal defects / 中国介入心脏病学杂志

Objective To explore if any rules in electrocardiogram changes after transcatheter closure of perimembranous ventricular septal defects ( PMVSD ) . Methods We included all the 358 patients who have accepted transcatheter closure of PMVSD in our hospital between July 2006 to October 2014 and the electrocardiogram (ECG) done in hospital and during follow up in 1,3, 6 and 12 months after operation were all reviewed. Results No changes were found in heart rates and electrical axis during follow-up as compared to preclosure ECG. PR interval was shorter, the QRS duration and QT interval were longer than preclosure. Incidence rate of arrhythmia was 38. 0% ( 136/358 ) and incidence rate of serious arrhythmias ( including Ⅱ° or Ⅲ° atrioventricular block and complete left bundle branch block) was 5. 0%(18/358). Among the 180 patients who had ECG done in all follow up between the first 12 months post closure, the rates of new developed arrhythmias was 12. 8% ( 23/180 ) and severe arrhythmia was 0. 6%(1/180) during follow-up. Conclusions Incidence rate of serious arrhythmias after transcatheter closure of PMVSD is low and most patients have good clinical outcome.

Embolisation of perimembranous ventricular septal defect occluder and transcatheter retrieval.

AIMS: In this study, we aim to summarise our experience with techniques used for the transcatheter retrieval of embolised devices. METHODS: We retrospectively reviewed the transcatheter retrieval of embolised devices in seven patients who underwent an attempted transcatheter closure of perimembranous ventricular septal defects (PMVSDs) between October 2002 and October 2013. The incidence, the main causes for the device's embolisation, and the techniques for transcatheter retrieval of the embolised device are discussed. RESULTS: The incidence of device embolisation in our centre was 0.82% (seven embolisations in 852 device placements). The main causes for device embolisation included undersized devices and inadequate subaortic rims. Among the seven embolisations, six of the devices were retrieved percutaneously without mortality, while one was retrieved during surgery. Of these patients, five had a HeartR(TM) Membranous VSD occluder of their PMVSDs, and the remaining two had surgical PMVSD closures. CONCLUSIONS: Our approach to the transcatheter retrieval of the embolised devices is associated with good results.

Atrioventricular and ventricular septal defects; topographical analysis and impact of associated cardiac and extracardiac findings and postpartum outcome.

OBJECTIVE: The aim of this study was to evaluate prognosis of types of ventricular septal defects and coexistence of associated cardiac and extracardiac defects. METHODS: 120 prenatal diagnosed pregnancies associated with ventricular septal pathology were retrospectively evaluated and divided into four groups, as atrioventricular septal defects, perimembranous septal defects, muscular septal defects and univentricular formation. Each group was divided further into four groups, as isolated defect, co-existing extracardiac defect, septal defect with extracardiac defect and septal defect with co-existing cardiac and extracardiac defect. Postnatal follow-up was continued at least until 8 months of life. RESULTS: Median gestational age at diagnosis was 26.3 weeks, 47 cases were diagnosed before 24 weeks. After dispersion of septal defects there was a statistical significance of p=0.0089 between groups. Of 31 cases with atrioventricular septal defects, only one case survived (3.2%) and there was a high association with extracardiac defects and abnormal karyotype (p=0.002). 69 cases with perimembranous ventricular septal defects were diagnosed, and 24 cases (34.8%) survived with significance for abnormal karyotype (p=0.039). Of 18 cases born with muscular septal defects 12 cases (66.7%) stay alive. We had two cases with univentricular structure; both cases decided for termination of pregnancy. CONCLUSION: The more complicated and severe the pathology, the worse the prognosis. Individualized counseling is the most important point in decision making together with families.