Multiple Ventricular Septal Defects Research Articles

Unique Case of Anomalous Anatomy of the Right Coronary Artery from the Pulmnary Artery

Anomalous origin of the right coronary artery from the pulmonary artery is one of the rare congenital coronary diseases. The percentage of population damage is 0.002 %. In most cases this occurrence is asymptomatic and diagnosed accidentally like our case. Here, we report a 9-month-old female infant who was admitted with a diagnosis multiple muscle ventricular septal defects. For diagnostic we used echocardiogram, as a result an anomalous origin of the right coronary artery from the pulmonary artery was suspected. After that, coronary computed tomography angiography confirmed the diagnosis. The patient underwent surgical correction by redeployment of the coronary artery in aorta. The aim of our report is to describe the successful correction of a potentially fatal disease. Early diagnostic can provide effective treatment.
 
  

Anatomical Correction of Transposition of the Great Arteries at the Arterial Level with Dacron Patch Closure of Multiple Ventricular Septal Defects under Integrated Extracorporeal Membrane Oxygenation: A Video Presentation

A 5-week-old male child, weighing 4 kg diagnosed with d-transposition of the great arteries with multiple muscular ventricular septal defects, Yacoub’s type-A coronary arterial pattern, successfully underwent arterial switch operation with Dacron patch closure of ventricular septal defects under moderately hypothermic cardiopulmonary bypass and St. Thomas based cold blood cardioplegia under integrated extracorporeal membrane oxygenation. Postoperatively, he required mechanical circulatory assistance for 72 h. At 12 months of follow-up, there was no mitral or tricuspid regurgitation, no neoaortic valve insufficiency with good biventricular function in Ross clinical score of 2.

Ectopia cordis with multiple ventricular septal defect and sternal cleft in a newborn: a case report.

This case report is of a 34-week- and 6-day-old female fetus with a birth weight of 2040g, a height of 41cm, and a head circumference of 32cm. The initial physical examination revealed a responsive newborn with an externalized heart outside the chest, with pericardium protection. In addition, a thoracic wall defect was detected suggesting incomplete formation of the septum bone. Moreover, in this case, the echocardiography report showed a multiple ventricular septal defect. The management of ectopia cordis is a challenge for any obstetrician and pediatric surgeon due to its rarity. It causes mental agony and anxiety to the parents. With an early diagnosis, termination of pregnancy can be one of the options. Once it is diagnosed late, it needs a multidisciplinary approach, and the services of a very experienced pediatric surgeon to improve the prognosis.

Three-dimensional printing in the closure of multiple muscular ventricular septal defects

Three-dimensional printing in the closure of multiple muscular ventricular septal defects

Multiple ventricular septal defects associated to anomalous venous returns, mitral valve disease, myocardium hypertrophy, and right outflow obstruction: a multimodality imaging assessment.

We report a rare congenital heart disease characterized by multiple ventricular septal defects associated to anomalous systemic and pulmonary venous returns, marked apical myocardial hypertrophy of both ventricles and of right outflow, and hypoplastic mitral anulus. Multimodality imaging is mandatory to assess anatomical details.

Trans-Atrial Approach to the “Swiss-Cheese” Ventricular Septal Defects: Sandwich Technique

<h2>Abstract</h2> The closure of Swiss-cheese multiple ventricular septal defects remains a difficult technical challenge and it is associated with increased mortality. Recently, the felt sandwich technique has been widely used to close muscular ventricular septal defects because the technique is simple, safe, effective, and reproducible. This technique may be useful, especially in complex cases, because it does not require prolonged surgical time. However, the use of numerous felt patches may disturb the movement of interventricular septum and cause cardiac dysfunction in infants. To avoid postoperative cardiac dysfunction, our current strategy is implemented to close muscular ventricular septal defects directly as much as possible. When the ventricular septal defect is considered unsuitable for direct closure, the felt sandwich technique is a useful tool that can be applied as needed by surgeons.

Clinical Presentation and Outcome of Management of Congenital Heart Diseases Associated with Down Syndrome in a Tertiary Care Hospital

Introduction: Treatment of children with Down syndrome having congenital heart defects has not been reported well in Bangladesh. This study has been carried out to inform practitioners about findings from cases in Combined Military Hospital, Dhaka. Methods: Three years retrospective observational study (clinical data, hospital course and follow-up) was carried out on 114 patients (49 males and 65 females, age range 1 month to 12 years) who underwent diagnostic and therapeutic work up in the pediatric cardiology department CMH Dhaka between January 2018 and December 2020. Results: Among 114 patients ,64 were (57%) were infants and 50 were older children (43%) , twenty-five had congestive cardiac failure and eighteen had hypothyroidism. The study showed that 33.3% were atrioventricular septal defect (AVSD), 28.1% were patent ductus arteriosus (PDA),8.75% were tetralogy of Fallot (TOF) ,5.25% were coartation of aorta (COA) ,3.5% were patent ductus arteriosus with coarctation of aorta 3.5%, double outlet right ventricle with pulmonary stenosis were 3.5%, ventricular septal defect were 7% whereas atrial septal defect along with ventricular septal defect were 5.3% ,atrial septal defect were 3.5%, transposition of great arteries with multiple ventricular septal defect were 1.8%.Forty (35%) patients using per cutaneous intervention,had 100% procedural success and no mortality .45 patients underwent surgery,few of them were not operated due to severe pulmonary hypertension. Conclusion: Congenital heart disease was more frequent in DS children,having worse prognosis.Children with DS required special attention due to increased risk of pulmonary hypertension. Delayed treatment was an important risk factor for poor prognosis.So early diagnosis is needed in order to provide optimal intervention and surgery ,before occurrence of irreversible PAH. J Bangladesh Coll Phys Surg 2023; 41: 7-14

Conduction mapping during complex congenital heart surgery: Creating a predictive model of conduction anatomy

ObjectivesThe study objectives were to report on a growing experience of conduction system mapping during complex congenital heart surgery and create a predictive model of conduction anatomy. MethodsPatients undergoing complex cardiac repair with conduction mapping were studied. Intraoperative mapping used a multielectrode catheter to collect His bundle electrograms in the open, decompressed, beating heart. Patient anatomy, operative details, His bundle location, and postoperative conduction status were analyzed. By using classification and regression tree analysis, a predictive model of conduction location was created. ResultsA total of 109 patients underwent mapping. Median age and weight were 1.8 years (range, 0.2-14.9) and 10.8 kg (range, 3.5-50.4), respectively. Conduction was identified in 96% (105/109). Median mapping time was 6 minutes (range, 2-33). Anatomy included atrioventricular canal defect, double outlet right ventricle, complex transposition of the great arteries, and multiple ventricular septal defects. By classification and regression tree analysis, ventricular looping and visceroatrial situs were the greatest discriminators of conduction location. A total of 94 of 105 patients (89.5%) were free of complete heart block. Only 1 patient (2.9%) with heterotaxy syndrome developed complete heart block. ConclusionsThe precise anatomic location of the conduction system in patients with complex congenital heart defects can be difficult for the surgeon to accurately predict. Intraoperative conduction mapping enables localization of the His bundle and adds to our understanding of the anatomic factors associated with conduction location. Predictive modeling of conduction anatomy may build on what is already known about the conduction system and help surgeons to better anticipate conduction location preoperatively and intraoperatively.

Trans-Ventricular Approach to “Swiss-Cheese” Ventricular Septal Defects: Septal Exclusion Technique

<h2>Abstract</h2> "Swiss-cheese" multiple muscular ventricular septal defects (VSDs) are often very challenging to close. Herein we describe our trans-ventricular approach that allows simple and effective closure of multiple muscular VSDs and does not appear to adversely affect ventricular function. (Video 2)

Feasibility of Low-Seated Composite Aortic Conduit for Surgical Treatment of Prosthetic Valve Endocarditis: A Case Report

Background: A 49-year-old male presented with a delayed diagnosis of infective endocarditis leading to extensive intracardiac destruction. Such cases present technical challenges to operative debridement as crucial anchoring points for replacement conduits are compromised. Case Presentation: Our patient presented at age 49 with nausea, lethargy, and diarrhea 2 weeks after recent travel. His prior history included bioprosthetic valve replacement for a bicuspid aortic valve. The patient was first given a trial of antimicrobials for a suspected UTI. Subsequently, he was admitted briefly to an outside hospital for a “cardiac work-up,” which returned negative. The patient sought care for the third time, during which he developed unstable supraventricular tachycardia, prompting echocardiography 16 days following the onset of symptoms. Echocardiography demonstrated a 6 cm abscess cavity invading the interventricular septum with a fistula into the left ventricular outflow tract, multiple ventricular septal defects (VSD), and suspected fistulae into the right ventricular outflow tract. The patient was treated with valve explant and extensive debridement. A valved-conduit for the aorta could not be sewn to the aortic annulus in the usual fashion due to destruction and debridement of the annulus, so a neo-annulus was created using the anterior leaflet of the mitral valve and the left ventricular outflow tract of the heart below the level of the VSDs. A mechanical-valved conduit was implanted onto the neo-annulus. A pacemaker was subsequently implanted. Conclusion: In patients with extensive intracardiac destruction with the compromise of the aortic annulus due to infective endocarditis, a low-seated, mechanical-valved conduit implanted directly to the aorto-mitral curtain and left ventricular outflow tract should be considered a novel, durable reconstructive option that allows complete debridement of infected tissues.

Clinical 3D modeling to guide pediatric cardiothoracic surgery and intervention using 3D printed anatomic models, computer aided design and virtual reality

BackgroundSurgical and catheter-based interventions for congenital heart disease require precise understanding of complex anatomy. The use of three-dimensional (3D) printing and virtual reality to enhance visuospatial understanding has been well documented, but integration of these methods into routine clinical practice has not been well described. We review the growth and development of a clinical 3D modeling service to inform procedural planning within a high-volume pediatric heart center.MethodsClinical 3D modeling was performed using cardiac magnetic resonance (CMR) or computed tomography (CT) derived data. Image segmentation and post-processing was performed using FDA-approved software. Patient-specific anatomy was visualized using 3D printed models, digital flat screen models and virtual reality. Surgical repair options were digitally designed using proprietary and open-source computer aided design (CAD) based modeling tools.ResultsFrom 2018 to 2020 there were 112 individual 3D modeling cases performed, 16 for educational purposes and 96 clinically utilized for procedural planning. Over the 3-year period, demand for clinical modeling tripled and in 2020, 3D modeling was requested in more than one-quarter of STAT category 3, 4 and 5 cases. The most common indications for modeling were complex biventricular repair (n = 30, 31%) and repair of multiple ventricular septal defects (VSD) (n = 11, 12%).ConclusionsUsing a multidisciplinary approach, clinical application of 3D modeling can be seamlessly integrated into pre-procedural care for patients with congenital heart disease. Rapid expansion and increased demand for utilization of these tools within a high-volume center demonstrate the high value conferred on these techniques by surgeons and interventionalists alike.

Association of Myocardial Muscle Non-Compaction and Multiple Ventricular Septal Defects by Echocardiography.

The aim of this study is to examine the possible high association between multiple ventricular septal defect (mVSDs) and noncompaction cardiomyopathy (NCM) as same embryological origin, and the effect of depressed ventricular function in NCM cases during the follow-up, using echocardiography. A total of 150 patients with mVSDs were diagnosed in a single center in Saudi Arabia; 40 cases with isolated or associated with minor congenital heart disease were recruited. Three specialist echocardiography consultants confirmed the NCM diagnosis separately using Jenni, Chin and Patrick criteria, and myocardial function was estimated by ejection fraction at admission and at follow-up after surgery. Stata-14 to analyze the data was used. In our cohort of 40 cases with mVSD (median age at diagnosis = 0.5 years; mean follow-up = 4.84 years), 13(33%) had criteria of non-compaction confirmed by the three specialist consultants. All were operated by surgery and 11 hybrid approach (interventional & surgery). A significant relationship between abnormal trabeculations and mVSD with or without non-compaction was observed, 34% vs 66% respectively (p < 0.03, Fisher's exact test). A repeated-measures t-test found the difference between follow-up and preoperative ejection-fractions to be statistically significant (t (39) = 2.07, p < 0.04). Further, the myocardial function in the mVSD non-compaction group normalized substantially postoperatively compared with preoperative assessment (mean difference (MD) 11.77, 95% CI: 4.40-19.14), whilst the mVSD group with normal myocardium had no significant change in the myocardium function (MD 0.74, 95% CI: -4.10-5.58). Thus, treatment outcome appears better in the mVSD non-compaction group than their peers with normal myocardium. Acknowledging the lack of genetic data, it is evident the high incidence of non-compaction in this cohort of patients with mVSD and supports our hypothesis of embryonic/genetic link, unlikely to be explained by acquired cardiomyopathy.

A game of Whack-A-Mole: Closing multiple ventricular septal defects.

A game of Whack-A-Mole: Closing multiple ventricular septal defects.

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

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

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

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

Dextroversion and D-Transposition of the Great Arteries, Left-Handed Ventricular Topology Related to Multiple Ventricular Septal Defects, and Left Juxtaposition of Atrial Appendage in a 1-Year-Old Child

We present a 1-year-old female infant case in natural history, affected by D-transposition of the great arteries with multiple ventricular septal defects in dextroversion, left juxtaposition of atrial appendages, and persistent left superior caval vein draining into coronary sinus. This is an uncommon case, and its particularity is due to clinical and anatomical findings diagnosed late in a setting of a complex cardiac malformation. After the diagnosis, the patient underwent palliative arterial switch operation without complications.

To What Extent Can Transcatheter Devices Replace Open-Heart Surgery in the Treatment of Cardiac Septal Defects?

Transcatheter treatments are widespread, having the advantages of being less invasive than surgery with quicker recovery times and reduced physical and psychological consequences. However correct patient selection is vital to optimise outcomes. In the case of an isolated atrial septal defect (ASD), transcatheter closure is preferred. Whilst multiple or large ASDs or ventricular septal defects (VSDs) are best treated through the transthoracic approach. Furthermore, the development of the transcatheter approach has yielded devices that can be used in the transthoracic approach resulting in hybrid techniques. This article aims to evaluate both transcatheter devices and open-heart surgery in the treatment of cardiac septal defects. A brief discussion follows on from the causes and history of cardiac defect treatments.

To What Extent Can Transcatheter Devices Replace Open-Heart Surgery in the Treatment of Cardiac Septal Defects?

Transcatheter treatments are widespread, having the advantages of being less invasive than surgery with quicker recovery times and reduced physical and psychological consequences. However correct patient selection is vital to optimise outcomes. In the case of an isolated atrial septal defect (ASD), transcatheter closure is preferred. Whilst multiple or large ASDs or ventricular septal defects (VSDs) are best treated through the transthoracic approach. Furthermore, the development of the transcatheter approach has yielded devices that can be used in the transthoracic approach resulting in hybrid techniques. This article aims to evaluate both transcatheter devices and open-heart surgery in the treatment of cardiac septal defects. A brief discussion follows on from the causes and history of cardiac defect treatments.

Abstract 12768: Development of a Clinical 3D Modeling Program to Guide Pediatric Cardiothoracic Surgery and Intervention

Introduction: Surgical and catheter-based interventions for congenital heart disease require precise understanding of complex anatomy. The use of three-dimensional (3D) visualization techniques to enhance visuospatial understanding has been well documented, but integration of these methods into routine clinical practice has not been well described. We report the growth and development of a clinical 3D modeling service to inform procedural planning within a high-volume pediatric heart center. Methods: Clinical 3D modeling was performed using cardiac magnetic resonance (CMR) or computed tomography (CT) derived data. Image segmentation and post-processing was performed using FDA-approved software. Patient-specific anatomy was visualized using 3D printed models, digital flat screen models and virtual reality. Surgical repair options were digitally designed using proprietary and open-source computer aided design (CAD) based modeling tools. Results: From 2018 to 2020 there were 112 individual 3D modeling cases performed, 16 for educational purposes and 96 clinically utilized for procedural planning. Over the 3-year period, demand for clinical modeling tripled and in 2020, 3D modeling was requested in more than one-quarter of STAT category 3, 4 and 5 cases. The most common indications for modeling were complex biventricular repair (n = 30, 31%) and repair of multiple ventricular septal defects (VSD) (n=11, 12%). Conclusions: Using a multidisciplinary approach, clinical application of 3D modeling can be seamlessly integrated into pre-procedural care for patients with congenital heart disease. Rapid expansion and increased demand for utilization of these tools within a high-volume center demonstrate the high value conferred on these techniques by surgeons and interventionalists alike.

An Alternative Technique of Intraoperative Identification of Multiple Muscular Ventricular Septal Defects

We propose the passage of a thick black braided silk SUTUPAK silk suture (Ethicon, Somerville, NJ) through the additional muscular ventricular septal defects to facilitate patch closure of the multiple muscular ventricular septal defects.