Multiple Muscular Ventricular Septal Defects Research Articles

Surgical Closure of Multiple Muscular Ventricular Septal Defects in Children Using 3D-Printed Models.

Multiple muscular ventricular septal defects (VSDs) are often difficult to visualize and access surgically. The main challenge is identifying all defects intraoperatively, without which residual defects are inevitable. Patient-specific three-dimensional (3D) printed models can help accurately demonstrate intracardiac anatomy. We present our experience using this technology to surgically close multiple muscular VSDs . Data of all patients with multiple VSDs in whom a 3D-printed model was used to aid surgical planning between September 2021 and July 2023 was collected retrospectively. Our approach involved generating a 3D model from a preoperative computerized tomography scan for each patient, which was then used to precisely identify the location of the multiple VSDs and plan surgical intervention. Six patients underwent closure of multiple VSDs using a 3D model. The mean age at surgery was 3.5 years (SD ± 2.8 years). Five (83.3%) patients had previously undergone pulmonary artery banding. The VSDs were approached through the right atrium in three (50%) and the right ventricle in three (50%) patients. Mean cardiopulmonary bypass and myocardial ischemia times were 185.2 min (SD ± 94.8 min) and 147.5 min (SD ± 86 min), respectively. There was no postoperative heart block or a hemodynamically significant residual VSD. All six patients had normal biventricular function at a median follow-up duration of 1.7 months (interquartile range: 1.2-7.4 months). 3D printing to aid closure of multiple VSDs is safe, reliable, and reproducible. We recommend adding 3D printing to surgeons' armamentarium when faced with the challenge of closing multiple muscular VSDs in children.

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.

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

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

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)

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.

Upper mini sternotomy approach for pulmonary artery banding: A single centre experience.

Pulmonary artery banding (PAB) remains a crucial technique in modern cardiac surgery. Left lateral thoracotomy, median sternotomy, and left anterior thoracotomy are well-known approaches. With significant scarce reports addressing the application of the upper mini sternotomy approach for PAB, this study aims to share experience and report outcomes of patients operated upon using this approach and its impact on facilitating the redo surgery. Since 2015, we practiced the use upper mini sternotomy approach for PAB in the study center where we conducted this retrospective study of 22 patients who underwent banding through the upper mini sternotomy approach. Indications varied between complete atrioventricular septal defect, multiple muscular ventricular septal defects, and univentricular heart with increased pulmonary blood flow. At the time of PAB, the medians of age 2.0 (1-4.5) months and bodyweight of 3.1 (1.9-4.2) kg were reported against a surgery time range of 75- 135 min and peak gradient across the band of 54-78 mmHg. There was one unrelated mortality case (4.5%) due to a severe attack of pulmonary hypertensive crisis. Fifteen patients underwent the redo surgery. No mortality or sternotomy-related complications were reported following the second stage surgery while the reopening time ranged between 17 and 32 min. The upper mini sternotomy approach for PAB is safe and facilitates the subsequent redo surgery and could be a valuable alternative to other surgical approaches.

Senning With Aortic Translocation and Anatomic Repair for Congenitally Corrected Transposition

Anatomic repair for congenitally corrected transposition of the great arteries with ventricular septal defect (VSD) and pulmonic stenosis has been accomplished with atrial switch and Rastelli. Aortic translocation offers a direct left ventricular outflow without an extraanatomic right ventricular-to-pulmonary conduit, which may lead to decreased reoperations. We reviewed our entire experience performing Senning with aortic translocation (SAT). From 2007 to 2017, 8 patients (mean age, 14.1 months; size, 8.86 kg) underwent SAT. Associated anomalies included situs inversus (n= 2), dextrocardia (n= 6), multiple muscular VSDs (n= 2), abnormal or straddling atrioventricular valve chords (n= 5), and branch pulmonary artery stenosis (n= 3). Four of 8 had previous systemic arterial shunts. Mean cardiopulmonary bypass was 487 minutes, and mean cardiac ischemic time was 307 minutes. Additional procedures included repair of branch pulmonary artery stenoses and closure of multiple muscular VSDs. There was no hospital death. One patient was supported with extracorporeal membrane oxygenation because of junctional tachycardia on postoperative day 5. One patient required pacemaker placement for first-degree heart block. Median hospital length of stay was 31 days. Mean length of follow-up was 52 months. All patients remain well with mild or no aortic regurgitation. The first patient underwent a repeat surgical operation for pulmonary venous baffle obstruction 2 years after SAT. Despite the technical complexity, patient outcomes have been satisfactory. We believe SAT provides a superior anatomic repair in these complex defects. Longer-term follow-up is needed regarding late intervention.

P254 Congenitally corrected transposition of the great arteries with an ebstenoid tricuspid valve, aortic atresia, hypoplastic aortic arch and a dysplastic pulmonary valve

Abstract Funding Acknowledgements No financial support received Introduction Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac malformation accounting for around 0.5% of congenital heart disease. Common associations are ventricular septal defects (VSD), pulmonary stenosis and heart block. CCTGA associated with aortic atresia is very rarely described in the literature. Description We present a case of a term neonate weighing 2.5kg. Fetal echo had initially made the diagnosis at 21/40. The baby was born in good condition and the postnatal echo confirmed the diagnosis of CCTGA. There were 2 secundum atrial septal defects, the sub-systemic tricuspid valve was ebstenoid with remarkably trivial regurgitation, there were multiple muscular ventricular septal defects with predominantly left to right flow, although flow appeared bi-directional in some views. The atretic aortic valve was anterior and to the left of the dysplastic, bicuspid pulmonary valve. The pulmonary valve leaflet motion was restricted with peak velocity of 2.4m/s and mild incompetence. The hypoplastic aortic arch filled retrogradely via the arterial duct. There was discrete coarctation with peak velocity of 2.2m/s down the descending aorta. The persistent left SVC drained to the coronary sinus. Following several discussions there was agreement that the most appropriate management for him would be to receive palliative care. He was discharged home at a week of age and continued to have intensive support from his local hospice and the cardiac team, until he passed away peacefully at home at 16 days of age. Discussion The multidisciplinary team decided surgery was extremely high risk due to the anatomy described above particularly in the context of his dysplastic, bicuspid pulmonary valve, which would have provided systemic outflow if intervention had been undertaken. Figure 1: 4 chamber view showing reverse offsetting of the atrioventricular valves, arch view showing a hypoplastic aortic arch with restrograde flow from the arterial duct, short axis view showing the semilunar valves and their relative positions, long axis view showing aortic atresia with retrograde flow into the ascending aorta Abstract P254 Figure 1

Left ventricular aneurysm an unusual complication after muscular VSD device closure

Cardiac ventricular aneurysms are rare in the pediatric population. Left ventricular (LV) aneurysms can be congenital or acquired. The acquired form may be caused by trauma (7%), transmural myocardial infarction (55%), infection (5%), connective tissue disease, and cardiac surgery (33%). Most commonly LV aneurysms are of idiopathic origin. Here we report a 2-years-old girl, with multiple muscular Ventricular Septal Defects (VSDs), large atrial septal defect, secondum type (ASDII) and Patent Ductus arteriosus (PDA). Cardiac catheterization at 10 months showed reactive pulmonary hypertension, she underwent pulmonary artery banding and PDA ligation at the age of 11 months, and then three of the muscular VSDs were closed by Amplatzer devices at the age of 17 months. At the age of 22 months another cardiac catheter to close remaining VSDs showed left ventricular (LV) apical aneurysm. The LV aneurysm was successfully resected surgically.

Successful surgical management of combined presence of congenital diaphragmatic hernia, ventricular septal defect, and patent ductus arteriosus in a child with allergy to frusemide—a case report

A 6-week-old male child presented with a right-sided congenital diaphragmatic hernia (CDH), large perimembranous (PM) ventricular septal defect (VSD) and multiple muscular VSDs, and a patent ductus arteriosus (PDA) with severe pulmonary arterial hypertension (PAH). The child was found to be allergic to frusemide. The VSDs were closed, the PDA was ligated, and the CDH was repaired through a right subcostal incision. Postoperatively, there were recurrent episodes of right lung lobar collapse, but the child responded to intravenous antibiotics and nasal continuous positive airway pressure (CPAP). The child is medically stable and on outpatient follow-up. We present this rare combination of diseases in view of a successful simultaneous surgical repair.

Intraoperative customized double-patch device with twin sutures for multiple muscular septal defects.

Closure of multiple muscular ventricular septal defects (VSDs) remains a challenge because of anatomical complexity. We mapped all the VSDs using en face reconstruction of the right ventricular septal surface through echocardiography and then performed an 'Intraoperative Customized Double-Patch Device' technique to surgically close them in 39 patients (male:female = 25:14). The median age of the patients was 6 months (2 months-10 years), and mean weight was 5.98 ± 4.21 kg. A patch of polytetrafluoroethylene was placed on the left ventricular side of the defect and another on the right ventricular side, and they were anchored to each other using 2 polypropylene sutures. Residual shunts were evaluated using intraoperative echocardiography and measurements of right atrial-pulmonary arterial saturation were taken in all patients. The distribution of muscular VSDs was as follows: anterior muscular 12, posterior muscular 18, mid-muscular 11 and apical 9. The associated lesions included perimembranous VSD (n = 28), tetralogy of Fallot (n = 6), double-outlet right ventricle (n = 2) and supramitral membrane (n = 2). Mean clamp time and bypass time were 93 ± 19 min and 147 ± 26 min, respectively. Mean hospital stay was 11 ± 3.39 days with no in-hospital mortality. Five patients with significant residual shunts needed concomitant PA banding. All patients remained in New York Heart Association Class I. There was either no residual shunt (n = 3) or trivial shunt (n = 2) among the banded patients. All patients remained symptom-free and continued to thrive well at the most recent follow-up (3.48 ± 1.51 years). Muscular VSDs can be mapped through en face reconstruction and closed using intraoperative customized double-patch device technique in a variety of situations with satisfactory immediate and short-term results.

Single-stage repair for multiple muscular septal defects: a single-centre experience across 16 years.

Multiple muscular ventricular septal defects (VSDs) are surgically challenging and its management remains controversial. We present a technique of surgical repair for muscular VSDs, which includes surgical exposure and detection of these defects and has excellent clinical outcomes. We have analysed consecutive patients who underwent surgical repair of isolated multiple muscular VSDs under cardiopulmonary bypass over a 16-year period (from January 2001 to November 2016) in a single centre from the southern part of India. These defects were accessed through the right atrium in most cases and closed directly; completeness of closure was confirmed by pressurizing the left ventricle with blood cardioplegia. There were no haemodynamically significant residual VSDs following repair. One hundred and two patients with an average time of follow-up of 4.1 years (1 month-12 years) were included. The mean age of our patients at the time of operation was 23.5 months (3 months-22 years) with a mean weight of 7.9 kg (2-55 kg). The mean cardiopulmonary bypass and cross-clamp time was 118.8 ± 39.2 min (mean ± SD) and 76.5 ± 29.4 min (mean ± SD), respectively. There were 10 (9.8%) hospital deaths and 3 late deaths in the entire study group. Permanent pacemaker was implanted in 2 patients. Seventy patients could be followed up after discharge. Postoperative pulmonary artery pressure was normal in 52% of the patients, mild-to-moderate hypertension in 27% and severe in 7% of the patients. The ejection fraction was >60% among the survivors, and there were no reoperations or reinterventions. This surgical approach to multiple muscular VSDs is safe and effective with minimal risk of complete heart block and diminution of ventricular function.

Seven Coils in 1 Heart: Therapeutic Option for Multiple VSD

Multiple muscular ventricular septal defect (VSD) is a serious and difficult-to-treat congenital heart defect. In the event of multiple VSDs with difficult anatomy, surgical closure may be challenging or even impossible. A temporary palliative option is pulmonary artery banding that may incidentally

Clinical Experience of Transcatheter Closure for Residual Ventricular Septal Defect in Pediatric Patients.

Open cardiac surgery has traditionally been the gold standard for repair of ventricular septal defect (VSD). The inherent risks and complications associated with open surgery and the incidence of postoperative residual VSD are significant disadvantages of the open surgical approach. To evaluate the methodology and efficacy of transcatheter closure of postoperative residual VSD in children. Patients with postoperative VSD who underwent percutaneous device closure in the period from August 2010 and February 2014 in our center were analyzed retrospectively. Twelve children, including 8 males and 4 females, with mean age of 8.13 ± 5.39 years (range 0.67-17 years) and mean weight of 29.63 ± 20.86 kg (range 5.5-66 kg) were included in the study. Ten cases had residual shunts situated at the margin of the patches while the other two had residual shunts because of multiple muscular VSD. The mean VSD diameter was 3.84 ± 1.86 mm (range 2.3-8.7 mm). On cardiac catheterization, the reported mean pulmonary circulatory blood volume/systemic circulation volume (Qp/Qs) ratio was 1.86 ± 0.91 (range 1.1-4.25) and the mean pulmonary artery pressure was 25.92 ± 12.42 mm Hg (range 7-52 mm Hg). Successful transcatheter closure was obtained in all twelve patients with postsurgery residual VSD. Two cases with residual muscular VSD underwent retrograde catheterization via the femoral artery while one case with residual VSD located at the lower margin of the patch was accessed via the internal jugular vein. The mean procedure time was 81.25 ± 25.86 minutes (range 40-120 minutes). There was no evidence of residual VSD, and no instances of new-onset valvular regurgitation were reported, with the only two exceptions being patients who had multiple muscular VSDs where clinically insignificant residual shunts were detected. Our retrospective study highlights the potential safety and therapeutic efficacy of transcatheter approach for closing postoperative residual VSD in children. More robust studies with longer-term follow-up of outcomes are required to firmly establish the safety profile and respective indications for use of various occluder devices for treating different categories of residual VSD.

A Simple Technique for Closing Multiple Muscular and Apical Ventricular Septal Defects.

Multiple ventricular septal defects (VSDs) are difficult to close. In this report, we describe a simple and safe technique of closing multiple muscular and apical VSDs. Between January 2010 and December 2013, 52 patients with a muscular VSD either in isolation or in association with other congenital heart disease underwent surgery using this technique in which a black silk thread was passed through the suspected VSD opening into the left ventricle and brought out through the mitral valve and the interatrial septum. Sutures were placed around the silk thread to close the VSD. Out of 52 patients, 34 were male; age ranged from 45 days to 5.5 years. In addition to the large subaortic/lage muscular VSD, three additional VSDs were present in 34 patients, two additional VSDs were present in 13 patients, and five patients had "Swiss Cheese septum." The VSDs were mid-muscular in 35 patients and were apical in 17 patients. Eleven patients had associated complex lesions. Intraoperative transesophageal echocardiograms did not reveal any significant residual shunt in any of these patients. There was no step-up on oximetry. There was one hospital death due to sepsis. Follow-up was available in 48 patients; three patients were lost to follow-up. At follow-up, no patient had a residual VSD. The biventricular approach through trans-right atrial, trans-interatrial septum using a thread through the hole method for closing multiple muscular VSD is effective with no persistent residual defects.

Long-term result of perventricular closure of multiple muscular ventricular septal defects in 3-month-old child

Long-term result of perventricular closure of multiple muscular ventricular septal defects in 3-month-old child

Insights gained from three-dimensional imaging modalities for closure of ventricular septal defects.

Ventricular septal defects (VSDs) are the commonest group of congenital heart lesions, accounting for ≈30% of congenital heart defects in liveborn infants.1 Defects in the ventricular septum are classified according to their position when the ventricular septum is viewed from the right ventricle and these may vary in their location, size, and number.2,3 VSDs can occur as isolated lesions or as part of more complex disease, for example, transposition of the great arteries or double outlet right ventricle, where the location and size of such defects assume great importance in planning the optimal mode of surgical repair.4 In most cases, surgical closure remains the mainstay treatment for VSDs, but selected lesions may be amenable to catheter closure.5,6 Cross-sectional echocardiography is the most commonly used noninvasive diagnostic method to delineate the position of a VSD before surgery.7 Multiple sonographic cuts and cross-sectional sweeps of the region of interest are used to build a virtual reconstruction of the three-dimensional (3D) anatomy in the mind of the echocardiographer. However, for the surgeon who deals solely with 3D images there can remain an element of uncertainty when called to operate on the patient. The number, size, location of VSDs, and their relationship to adjacent structures are central for selecting whether the defects might be best closed via the tricuspid valve, via the semilunar valves, or via a ventriculotomy or whether these might not be readily approachable and a palliative pulmonary artery banding is desirable.8,9 In this review, we present key 3D echocardiographic projections and examples of different types of VSDs where the images have a direct effect on the approach taken to VSD closure. Comparison of 3D echocardiography (3DE) with other 3D imaging modalities is also described. Images are presented in an …

Multiple Muscular Ventricular Septal Defects: Use of Fluorescein Dye to Identify Residual Defects

Multiple muscular ventricular septal defects remain a challenge for the congenital heart surgeon. The optimal strategy for an infant or neonate with multiple muscular ventricular septal defects is still unclear. Perioperative identification and secure closure of these defects pose significant difficulties. We describe a novel technique of using fluorescein dye to identify small muscular ventricular septal defects.

Transvascular closure of single and multiple muscular ventricular septal defects in neonates and infants &lt; 20 kg

While surgical closure of ventricular septal defects (VSD) is still the gold standard, we review our experience with the interventional closure of single and multiple muscular VSD in newborns and infants under 20 kg. Between 2004 and 2012 transcatheter closure of ten Swiss cheese VSD, six single muscular and one apical VSD was attempted in 17 patients between 10 days and 7.3 years of age and with a bodyweight ranging from 2.2 to 19 kg. Seven patients had had a significant shunt after cardiac surgery, five patients a shunt induced congestive heart failure and in five patients postponement of surgery was intended. A total of 20 devices was successfully implanted in 15 of 17 (88%) patients, reducing the interventricular shunt and improving the haemodynamic situation in 14 patients. An acute AV-block led to immediate removal of the device in a patient of 2.2 kg. One Amplatzer muscular VSD occluder could not be delivered due to the sharp bending of the delivery sheath in 2004. Three patients died during follow-up not related to the intervention. Re-intervention was necessary in one patient with Swiss cheese VSD. Interventional closure of muscular VSD is possible in newborns and infants and presents an eligible treatment option. New occlusion systems with miniaturized introducer sheaths of 4-6 French have extended the spectrum of treatable lesions. An individual and interdisciplinary risk-benefit stratification is required to choose from surgical, interventional, or combined strategies.

Unique Echocardiographic Markers of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) in the Adult

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect in adults. We report a 38-year-old male presenting with exertional syncope. He was referred for the evaluation of multiple muscular ventricular septal defects diagnosed on an outpatient echocardiogram. Echocardiography revealed mild left ventricular enlargement, abnormal flow-pattern in the ventricular septum and dilatation of the right coronary artery. Pulsed-wave Doppler with sample volume placed in the coronary ostium showed systolic coronary flow predominancy. This unique finding is characteristic for ALCAPA and can differentiate it from other coronary anomalies. Coronary angiography confirmed ALCAPA syndrome. Surgical correction was planned.