Ventricular septal defect complicating acute myocardial infarction: diagnosis and management. A Clinical Consensus Statement of the Association for Acute CardioVascular Care (ACVC) of the ESC, the European Association of Percutaneous Cardiovascular Interventions (EAPCI) of the ESC and the ESC Working Group on Cardiovascular Surgery.

Ventricular septal defects are a rare complication after acute myocardial infarction with a mortality close to 100% if left untreated. However, even surgical or interventional closure is associated with a very high mortality and currently no randomized controlled trials are available addressing the optimal treatment strategy of this disease. This state-of-the-art review and clinical consensus statement will outline the diagnosis, hemodynamic consequences and treatment strategies of ventricular septal defects complicating acute myocardial infarction with a focus on current available evidence and a focus on major research questions to fill the gap in evidence.

Double-chambered left ventricle misdiagnosed as ventricular septal defect.

A 35-year-old woman was initially misdiagnosed with a muscular ventricular septal defect but was later correctly diagnosed with a double-chambered left ventricle following evaluation by echocardiography and cardiac computed tomography.

Word of caution: silent late device embolisation after perimembranous ventricular septal defect closure in a 6-Kg infant.

We report on a 6-month-old infant (6 Kg/ 64 cm) with perimembranous ventricular septal defect (absent sub-aortic rim, 10 mm left ventricular entry, and 4 and 6 mm right ventricular exists) and successful retrograde closure using an 8x6 mm KONAR-MF™ VSD occluder (Lifetech, China). Immediate and 48 hours post-procedure ultrasounds showed an accurately positioned device and two jets of mild-to-moderate residual shunts. At the 2-week follow-up, the device did not change position and the shunt was stable and intra-prosthetic. The scheduled 3-month follow-up was skipped for familial reasons. The patient came back without alarming symptoms for the regular 6-month follow-up, and the device was found embolised to the left pulmonary artery. The device was retrieved surgically, and the defect was patch-closed with excellent outcomes. There was a pseudoaneurysm involving the tricuspid valve chordae and the device was endothelialized partially on one edge suggesting that embolization occurred somewhere between 3 months and 6 months post-operative. Defects with compromised anatomies should be closed surgically to avoid suboptimal results, especially in small infants.

Imaging and surgical management of congenital heart diseases.

Congenital heart disease affects approximately 1% of live births per year. In recent years, there has been a decrease in the morbidity and mortality of these cases due to advances in medical and surgical care. Imaging plays a key role in the management of these children, with chest radiography, echocardiography and chest ultrasound the first diagnostic tools, and cardiac computed tomography, catheterization and magnetic resonance imaging reserved to assess better the anatomy and physiology of the most complex cases. This article is a beginner's guide to the anatomy of the most frequent congenital heart diseases (atrial and ventricular septal defects, abnormal pulmonary venous connections, univentricular heart, tetralogy of Fallot, transposition of the great arteries and coarctation of the aorta), their surgical management, the most common postsurgical complications, deciding which imaging modality is needed, and when and how to image gently.

Prognosis Analysis of Children with Interrupted Aortic Arch Complicated with Ventricular Septal Defect and Other Associated Intracardiac Defects after One-Stage Radical Surgery.

BACKGROUND: High rates of mortality and aortic arch stenosis have been reported for one-stage radical surgery of interruption of aortic arch (IAA) with ventricular septal defect (VSD) and other associated intracardiac defects, but the sample size of the study is relatively small, and the credibility of the study is not high. The risk factors of death and aortic arch stenosis will be analyzed in a large sample size of infants with IAA, VSD and other associated intracardiac defects after one-stage radical resection. METHODS: A retrospective analysis was performed on 152 children with IAA, VSD and other associated intracardiac defects from January 2006 to January 2017 who had undergone one-stage radical resection, including 95 cases of type A and 57 cases of type B. January 2006-December 2011 as the early period, and January 2012-January 2017 as the late period. Cox proportional hazards regression model was used to analyze the risk factors for mortality and aortic arch stenosis after surgery, the overall survival rate was analyzed by the Kaplan-Meier method, and the survival curve was drawn by GraphPad Prism 8 software. RESULTS: 22 cases (14.47%) died, 27 cases (17.76%) developed aortic arch stenosis. The 1-month, 3-month, 6-month, 1-year, 3-year, and 5-year survival rates were 85.53%, 85.53%, 85.53%, 84.21%, 78.95% and 75.66%, respectively. Low age (Hazard Ratio (HR) = 0.551, 95% Confidence Interval (CI): 0.320-0.984, p = 0.004), low body weight (HR = 0.632, 95% CI: 0.313-0.966, p = 0.003), large ratio of VSD diameter/aortic diameter (VSD/AO) (HR = 2.547, 95% CI: 1.095-7.517, p = 0.044), long duration of cardiopulmonary bypass (HR = 1.374, 95% CI: 1.000-3.227, p = 0.038), and left ventricular outflow tract obstruction (LVOTO) (HR = 3.959, 95% CI: 1.123-9.268, p = 0.015) were independent risk factors for postoperative death. The surgical period (January 2006-December 2011) (HR = 0.439, 95% CI: 0.109-0.964, p = 0.046) and the addition of pericardial anastomosis to the anterior aortic wall (HR = 0.398, 95% CI: 0.182-0.870, p = 0.021) were independent risk factors for postoperative aortic arch stenosis. CONCLUSIONS: Children with low age, low body weight, large ratio of VSD/AO, long duration of cardiopulmonary bypass, LVOTO, the surgical period (January 2006-December 2011) and pericardial anastomosis with anterior aortic wall have poor prognosis.

Experience of repair ventricular septal defect with left superior vena cava through right axillary thoracotomy.

OBJECTIVE: To summarize the experience in the treatment of repair ventricular septal defect with left superior vena cava (LSVC) through right axillary thoracotomy. To explore the surgical strategy of treating VSD with LSVC through right axillary thoracotomy. METHODS: right axillary thoracotomy and median sternotomy were performed in 73 cases of ventricular septal defect with LSVC in our center from 2018 to 2019. Perioperative data and surgical information were analyzed retrospectively. RESULTS: There were 54 cases of R-group and 19 cases of S-group with median age of 0.8 years (0.5-1.6years). In the 73 patients, 21(38.9%) were men and 52 (61.1%) women. The operation time of R-group was shorter than S-group, p<0.05. The postoperative drainage in R-group was less than S-group, p<0.05. The mechanical ventilation time was longer in the S-group than in the R-group, p<0.05. There were no deaths, serious complications and readmission in the follow-up 6 months(3-10months). CONCLUSION: Right axillary thoracotomy is a safe procedure with excellent cosmetic and clinical results for ventricular septal defect with left superior vena cava. It has the advantages of short operation time, less bleeding and short postoperative time.

The Interventricular Septal Hematoma Following Surgical Correction of Ventricular Septal Defect in Infants: A Single-Center Experience.

OBJECTIVE: To review and analyze the cases of interventricular septal hematoma (IVSH) following surgical correction of the ventricular septal defect (VSD) in infants in our center. METHODS: Retrospective analysis was performed on five infants with IVSH after surgical correction of VSD in our center from January 2020 to January 2022. The general preoperative information and intraoperative and postoperative results were collected and analyzed. RESULTS: All five infants with VSD were repaired under cardiopulmonary bypass and occurred IVSH. The cardiac arrest occurred in one patient five hours after return to the intensive care unit (ICU). The patient's hemodynamics were difficult to maintain after cardiopulmonary resuscitation, and the patient died. Two other patients had arrhythmia and unstable hemodynamics during the perioperative period, the hematoma puncture was performed, and the patients' symptoms lessened. Perioperative and postoperative echocardiography showed that the hematoma gradually was shrunk, and the hemodynamics became stable. The hemodynamics were stable in the remaining two infants during the perioperative period. No specific medical intervention was required other than clinical observation in these two patients. Finally, the four infants successfully were discharged with good clinical results. CONCLUSION: IVSH is a rare complication of surgical repair of VSD. Prevention and early detection of IVSH during operation in infants with VSD are essential.

A Rare Cause of Right Heart Failure in An Adult: Congenital Gerbode Defect.

Gerbode defect is a rare defect describing a left ventricle to right atrium shunt with symptoms dependent on the size and degree of the shunt. It is either a congenital defect detected in infancy, or an acquired case reported in older age. Diagnosis by means of echocardiography and cardiac magnetic resonance imaging is of paramount importance before surgical correction is contemplated to achieve a good prognosis.

Genetic analysis of the CITED2 gene promoter in isolated and sporadic congenital ventricular septal defects.

Ventricular septal defect (VSD) is the most common congenital heart defect. Previous studies have reported genetic variations in the encoding region of CITED2 highly associated with cardiac malformation but the role of CITED2 gene promoter variations in VSD patients has not yet been explored. We investigated the variation of CITED2 gene promoter and its impacts on gene promoter activity in the DNA of paediatric VSD patients. A total of seven variations were identified by Sanger sequencing in the CITED2 gene promoter region in 400 subjects, including 200 isolated and sporadic VSD patients and 200 healthy controls. Using dual-luciferase reporter assay, we found four of the 7 variations identified significantly decreased the transcriptional activity of the CITED2 gene promoter in HEK-293 cells (P < .05). Further, a bioinformatic analysis with the JASPAR databases was performed and a cluster of putative binding sites for transcription factors was created or disrupted by these variations, leading to low expression of CITED2 protein and development of VSD. Our study for the first time demonstrates genetic variations in the CITED2 gene promoter in the Han Chinese population and the role of these variations in the development of VSD, providing new insights into the aetiology of CHD.

Surgical Repair for Acute Myocardial Infarction Induced Ventricular Septal Defect: Does Time Matter?

OBJECTIVE: Ventricular septal defect (VSD) induced by acute myocardial infarction (AMI) is rare but lethal, with high mortality even after surgical repair. Our aim was to assess the association between the time interval and surgical repair effects in patients with VSD following AMI. METHODS: From January 2003 to December 2017, 14 patients with VSD induced by AMI received surgical therapy in our department. We retrospectively reviewed the patients' clinical manifestations, surgical methods, and outcomes. According to the time interval from AMI onset and surgery, we divided the patients into two groups: Group 1 (N = 9), more than one week, and Group 2 (N = 5), less than one week. A comparison study was performed, and differences were analyzed. RESULTS: The mean age of the entire group was 65.5±3.3 years, with 78.6% males (11/14). VSDs were anterior apical in 10 (71.4%) and posterior inferior in 4 (28.6%) patients. The average size of the VSD was 15.8±5.8 mm. Compared with Group 1, Group 2 had poorer left ventricular function (LVEF 40.8±10.3% vs. 30.4±2.3%, P = 0.035) and a higher rate of urgent procedures (11.1% vs. 100.0%, P = 0.003). The mortality rate was 14.3% (2/14). Mechanical support was more common in Group 2 than Group 1. No resistant shunt or death was found during follow up. CONCLUSIONS: VSD following AMI is safer for more than one week, but surgical treatment is also acceptable for patients requiring urgent surgery due to hemodynamic instability. Mechanical assistive devices can improve the perioperative success rate.

Membranous septal aneurysm causing severe right ventricular outflow obstruction in an adult with trisomy 18.

Membranous ventricular septal aneurysm is a known entity but rarely causes severe right ventricular outflow obstruction. We report a 40-year-old female with trisomy 18 who developed severe right ventricular outflow obstruction caused by an enormous membranous septal aneurysm associated with unrepaired inlet ventricular septal defect with perimembranous extension.

Isolated right subclavian artery in a left aortic arch with ventricular septal defect.

Isolated origin of the subclavian artery is a congenital anomaly of the aortic arch in which one subclavian artery is attached to the ipsilateral pulmonary artery through ligamentous arteriosus. An isolated right subclavian artery with the left-sided aortic arch is an extremely rare condition. We report on an asymptomatic 2-year-old-girl, who was referred because of an incidental cardiac a murmur. She was diagnosed by echocardiography to have an isolated right subclavian artery connected to the right pulmonary artery in a left aortic arch with a ventricular septal defect. MRI confirmed the findings.

The Application and Teaching Value of a Ventricular Septal Defect Canine Model Established by Transcatheter Puncture.

This study aimed to improve and further explore a ventricular septal defect (VSD) canine model on the basis of the transcatheter puncture method and to evaluate its application and teaching value.In order to lessen the complications of VSD closure, it is necessary to improve the currently available treatment devices using appropriate animal models.In this study, we used 16 healthy adult canines as our models. After anesthesia, the VSD puncture was performed, followed by balloon dilatation of the perforation. VSD was confirmed by angiography. The venous-artery orbit was established, and the VSD was then closed once the catheter and occluder were across the defect.Of the experimental canines, 14 of the 16 canines were successfully modeled, giving a success rate of 87.5%. The canines underwent an immediate creation of a venous-artery orbit for teaching practice and were implanted with an occluder during the procedure. After 4 weeks, 13 canines survived. As per our findings, most VSD types established by the puncture were perimembranous (10 of 13, 77%).The current model has a high success rate. The model can not only avoid the risk of infection and hemodynamic disorders associated with an open thoracotomy, but can also be effectively used in evaluating the impact of occluders. It can also directly measure the parameters of the devices during the procedure, thus having a very high experimental and teaching value.

[Takotsubo syndrome after palliative transcatheter treatment of acquired aortic stenosis in patient with congenital ventricular septal defect. Case report].

The authors report the clinical case of secondary Takotsubo syndrome developed after transcatheter aortic valve replacement that was performed in compassionate manner in female patient with combination of congenital ventricular septal defect and acquired severe aortic stenosis. In the teams view, Takotsubo syndrome was triggered with profound changes of intracardial hemodynamics subsequent to iatrogenic impairment of preexisting interventricular shunt.

LACHT syndrome (Mardini-Nyhan association) with tracheal stenosis in a Thai newborn.

LACHT syndrome, or Mardini-Nyhan association, is an ultra-rare disorder, diagnosed solely by the clinical characteristics of lung agenesis, complex cardiac defects, and thumb anomalies. Only 12 patients have been reported worldwide, and here, we report a new clinical diagnosis of LACHT syndrome. Our patient was a male full-term newborn with left lung agenesis, congenital heart defects including ventricular septal defect, right-sided aortic arch, with aberrant left subclavian artery and Kommerell diverticulum, as well as left preaxial polydactyly and hemivertebra. Our patient appears to be the second LACHT syndrome case to also suffer from tracheal stenosis, which has only been reported once before in conjunction with this syndrome. In light of this, tracheal stenosis may be a phenotype for LACHT syndrome.

What have we learned from 691 prenatal chromosomal microarrays for ventricular septal defects?

INTRODUCTION: Ventricular septal defect (VSD) represents the most common type of congenital cardiac anomaly, affecting more than 1 in 300 live births. The objective of this study was to examine the incidence and nature of abnormal chromosomal microarray analysis (CMA) results in a large cohort of pregnancies with VSD. MATERIAL AND METHODS: Data acquisition was performed through the Ministry of Health computerized database. All CMA results performed due to VSD during 2013-2017 were included. The rates of clinically significant CMA results of cases with isolated and non-isolated VSD were compared with two control populations-a systematic review of 9272 pregnancies and a local cohort of 5541 fetuses with normal ultrasound. RESULTS: Overall, 691 CMA analyses performed due to a sonographic indication of VSD were detected. Of 568 pregnancies with isolated VSD, eight (1.4%) clinically significant copy number variants were detected, a nonsignificant difference compared with low risk pregnancies. Of the 123 pregnancies with non-isolated VSDs, 18 (14.6%) clinically significant CMA results were detected, a considerably increased risk compared with control pregnancies. Karyotype-detectable anomalies constituted 12 of the 18 abnormal CMA results in non-isolated VSD group (66.7%), a significantly higher proportion compared with 2 of 8 (25%) in isolated VSD cohort. CONCLUSIONS: The outcomes of our study, representing the largest number of CMA results in pregnancies with VSD, suggest that the rate of abnormal CMA findings in isolated VSD does not differ from pregnancies with normal ultrasound. This observation is true for populations undergoing routine common trisomy screening tests and early sonographic evaluation, as well as widely available non-invasive prenatal screening. Conversely, CMA analysis yields a high detection rate in pregnancies with non-isolated VSD. Our results question the recommendation to perform invasive prenatal testing for CMA in pregnancies with isolated VSD.

Prenatal Identification of Restrictive and Non-restrictive Ventricular Septal Defects Based on End-Systolic Flow Patterns in the Fetal Aortic Isthmus.

Fetuses with large ventricular septal defects (VSDs) must be closely monitored during the perinatal period. Intrauterine assessment of size of septal defects with bidimensional echocardiography are unreliable. The objective of the present study is to document the contribution of flow pattern analysis in the fetal aortic isthmus for prenatal identification of large non-restrictive VSDs requiring immediate postnatal attention. We conducted a cross-sectional retrospective study of Doppler recordings from patients referred to our Fetal Cardiology Unit and diagnosed with one or multiple VSDs from January 2006 to November 2015. Three groups were defined: (1) large non-restrictive VSDs (n = 11) with postnatal cardiac failure (2) small restrictive VSDs (n = 28) asymptomatic, and (3) absence of cardiac abnormality (n = 66). The Isthmic end-Systolic Index (ISI) was computed to quantify aortic isthmus flow and compared between the three groups. Fetuses with restrictive VSD and control group showed similar ISI: stable at 0.20 ± 0.01 up to 27 weeks of gestation and dropping later due to the fall of end-systolic velocities in the aortic isthmus. By 35 weeks, a brief end-systolic retrograde flow was present, associated with a further fall of ISI down to -0.26 ± 0.05. In contrast, ISI of fetuses with large non-restrictive VSDs were unchanged throughout gestation (0.18 ± 0.06), becoming significantly higher during the last weeks of gestation compared to the two other groups (p < 0.001). In fetuses with VSDs, the ISI measurement from the aortic isthmus Doppler flow during the second half of gestation is a reliable predictor of a large non-restrictive defect with risk of major postnatal clinical impact.

Diagnosis and Management of Anomalous Left Anterior Descending Coronary Artery from the Pulmonary Artery in an Asymptomatic Infant.

Anomalous origin of the left anterior descending coronary artery from the pulmonary artery is a rare variant of anomalous origin of the left main coronary artery from the pulmonary artery. We report on a seemingly asymptomatic patient with ALADCAPA and a small restrictive muscular ventricular septal defect diagnosed by echocardiogram in the neonatal period. Our patient underwent elective repair at 3.5 months of age after which feeding and growth improved dramatically. Multimodality imaging is helpful to confirm this rare anomaly; however, echocardiographic clues including lack of left coronary branching or an abnormal coronary course should raise suspicion for ALADCAPA. This case provides support for early repair in children with an incidental finding of this anomaly as subclinical ischemia may be under-recognized by available testing but may lead to symptoms later in life.

Safe Surgical Retrieval of Endothelialized Ventricular Septal Defect Closure Device.

Surgical retrieval of endothelialized ventricular septal defect closure devices is associated with significant morbidity. We herein present a technique for the safe removal of such devices (Shanghai Shape Memory Alloy, China) from the heart.

Surgical management for an adult, female patient, with Ebstein Anomaly on Tricuspid Valve that has Subvalvular membrane with severe PS and multiple VSDs-A case report.

Ebstein's anomaly is a rare and complexed heart defect that affects the tricuspid valve and is accountable for around 1% of congenital cardiac abnormalities. It is one of the most common congenital causes of tricuspid valve regurgitation. Ebstein's anomaly is often diagnosed prenatally due to its severe cardiomegaly. Some individuals with this anomaly do not experience any complications until adulthood and even then its mostly minor complaints like exercise intolerance.  Atrial septal defect is most commonly (70-90%) associated with Ebstein's anomaly. However, ventricular septal defect (VSD) can be associated with 2-6% of the cases. This particular report presents a case of surgical intervention for a 20 years old female with Ebstein's anomaly that had multiple VSD's and a severe Pulmonary Stenosis (PS).