Transcatheter Closure of Perimembranous Ventricular Septal Defect Using KONAR-MF™: A Multicenter Experience.

Transcatheter closure of perimembranous ventricular septal defect (PmVSD) is an established procedure. However, the occurrence of complete heart block limits its scope. The newer KONAR-MF™ occluder has specific design characteristics that may improve the safety of PmVSD closure. The objective of the study was to describe the efficacy and mid-term follow-up of transcatheter closure of PmVSD using KONAR-MF™. The study was conducted prospectively in 3 Indian centers (January 2018-December 2022). PmVSD closure was done by both antegrade and retrograde methods, and patients were followed up at 1, 3, 6, 12 months, and annually after that. 121 out of 123 patients were included with the following characteristics: median age 4.4 (0.18-40) years; weight 15 (2.1-88) kg; mean Qp/Qs ratio 1.87 ± 0.52 and pulmonary artery mean pressure: 22 ± 6.9 mmHg. The procedure was successful in all but 3; the device was removed due to significant residual shunt (n = 2) and new development of aortic regurgitation (AR) (≥ mild) in 1. The median defect size was 5.2 (2.5-12) mm. Device sizes from 6/4 to 14/12 were deployed (median fluoroscopy time 13.3 min; range 3.6-47.8). Shunt occlusion rates were 90%-Immediate, 95%-pre-discharge, and 97%-1 month, with no instances of complete heart block after the procedure and during follow-up. Six had new onset AR (mild: 2, trivial 4), and one had increased tricuspid regurgitation. All patients were well during follow-up (median: 20 months; range: 6-46). The new KONAR-MF™ occluder appears to be a promising and safe alternative for the closure of the PmVSD; further long-term follow is merited.

Transfemoral Perimembranous Ventricular Septal Defect Device Closure in Infants Weighing ≤ 10 kg.

Transcatheter closure of Perimembranous VSDs (PMVSD) remains challenging particularly in infants. The aim of this study is to evaluate the efficacy and safety of transfemoral PMVSD device closure in infants weighing ≤ 10 kg in a single centre. Retrospective review of departmental databases and medical charts to define patient cohort and collect demographic, procedural and follow-up data. Between July 2014 and March 2021, 16 patients underwent attempted transfemoral PMVSD device closure (12 retrograde) at a median age of 11 months (interquartile range [IQR] 9-15.5) and a median weight of 8.3 kg (IQR 7.2-9.5). All patients were either symptomatic, had progressive left heart dilation or had VSD associated valve regurgitation. Median defect size on pre-procedural transoesophageal echocardiography was 6.8 mm (IQR 6-8.5). Median device waist size was 6 mm (IQR 4.5-8). Successful device placement was achieved in 14 patients (88%). One patient developed moderate aortic and tricuspid valve regurgitation upon retrograde and antegrade device deployment, respectively, and subsequently underwent surgical closure. The second patient developed progressive aortic regurgitation (AR) 2 days post procedure, and also underwent surgical removal with no residual AR. There was no cases of device embolization and no femoral arterial compromise. On median follow-up of 40.5 months (IQR 25-64), none of the patients developed complete heart block. Three patients (18.75%) had small residual shunts at latest follow-up which have not required any further intervention. Device closure of PMVSD's in children weighing ≤ 10 kg is feasible and safe with good procedural success rates. Use of both the antegrade and retrograde approaches may be necessary depending on anatomical variances.

Three centers experience with device closure of congenital Gerbode-type perimembranous ventricular septal defects.

OBJECTIVES: We aim to evaluate our experience with interventional closure of Gerbode-type perimembranous ventricular septal defects (pmVSDs). METHODS: We performed three-center retrospective data review of patients with congenital indirect Gerbode-type pmVSDs treated percutaneously between August 2017 and May 2021. Standard safety and latest follow-up outcomes were assessed. RESULTS: Ten patients (six females) were identified with a median age of 6.8 years (range: 2.5-54) and a median weight of 26.5 kg (range: 12-88). The median left ventricular defect size was 10 mm (range: 3-15.5). On baseline ultrasound, 6 patients had absent subaortic rim , 6 patients had trivial aortic regurgitation, and 3 patients had tear-drop-type (small) aortic cusp prolapse. The tricuspid regurgitation was graded II (n = 5) and III (n = 5). Five Lifetech Konar-Multifunctional occluders, four Amplatzer duct occluders II and one Amplatzer duct occluder I were implanted. The median fluoroscopy time was 10.4 min (range: 4.3-20.2). Pre-existing aortic regurgitations remained identical. One new aortic regurgitation was identified before discharge and remained trivial after 48 months of follow-up. No heart block or tricuspid stenosis was observed on a median follow-up of 17 months (range: 3-48). All patients are symptom-free with complete shunt closure and significant regression or resolution of tricuspid regurgitation. CONCLUSIONS: Despite anatomical challenges, interventional closure of congenital indirect Gerbode-type pmVSD appears to be feasible, safe, and most importantly clinically effective using different commercially available devices. Amplatzer duct occluder II and Lifetech Konar-Multifunctional occluder offer interesting specifications to retrogradely target this specific defect with success.

Transcatheter Versus Surgical Closure of Acute Ruptured Sinus of Valsalva Aneurysms with Associated Ventricular Septal Defects.

Transcatheter ruptured sinus of valsalva aneurysm (RSOVA) closure is an alternative to surgery. When a restrictive ventricular septal defect (rVSD) coexists, there are doubts about support for an occluder and persistent left ventricular (LV) dilatation. We compared the outcomes of patients from a single centre with rVSD and RSOVA after surgery or transcatheter intervention in the past 8 years in this study. Location of rVSD did not alter the approach. Compliant balloon interrogation before transcatheter closure was occasionally used to assess significance of interventricular shunt or aortic regurgitation. Procedural success and LV dimensions before and after treatment were analyzed. Seventeen patients with a mean age of 36.64 ± 12.58 years were analyzed. 12/13 catheter procedures (92%) were successful, of whom eight patients had an outlet VSD. rVSD was closed with device in one patient with perimembranous rVSD, but unaddressed in others. Four patients underwent balloon interrogation. Surgery included the single patient who failed intervention. Transcatheter group had more acute symptoms and advanced functional class than surgical group, though not significant. At a median follow-up of 4.5 (1-7.5) years, the mean LV dimensions (50.3 ± 10.1 mm and 50.1 ± 3.1 mm, p = 0.46) were not different in the transcatheter and surgical groups. Mean end-diastolic volumes (110.5 ± 42. 1 ml and 98.5 ± 37.5 ml, p = 0.91) were not significantly different either. Transcatheter RSOVA closure success was not altered by rVSD despite their location underneath the aneurysms, including outlet rVSD with defect in aortoventricular junction. Persistent LV dilatation was not observed after catheter intervention even when the VSD was not closed.

Mapping the Conduction System in Patients Undergoing Transcatheter Device Closure of Perimembranous Ventricular Septal Defect: A Proof-of-Concept Study.

Earlier studies that investigated the relation of atrioventricular (AV) conduction system to perimembranous ventricular septal defect (pmVSD) were based on cardiopathological specimens. To study the relationship of conduction system to pmVSD using 3-dimensional electroanatomic mapping system (EAMS) in patients undergoing device closure. Fifteen consecutive cases of pmVSD from January 2014 to July 2017 (age > 2 years and weight > 8 kg) were included in the study. The course of conduction system and its relationship with the pmVSD was mapped before and after device closure, with the use of EAMS. Median age and weight of the cohort was 10 years (range 4-21 years) and 25 kg (range 13-55) respectively. Device implantation was successful in all patients except 1. The course and relation of the conduction system were posteroinferior to the pmVSD in all cases (100%), and away from the defect in 67% (10/15). In patient with baseline RBBB, the right-sided conduction system was in close proximity to the pmVSD. Two patients had part of left-sided conduction system in close proximity to pmVSD or device edges. Two patients developed RBBB following device deployment, which reverted to normal on follow up. No patient developed high grade AV block during the median follow-up of 34 months (range 24-62). This experimental study has shown the feasibility of 3D EAM of conduction system during device closure of pmVSD. This novel concept can be utilized to understand the anatomy of conduction system in other congenital heart diseases.

Transcatheter closure of large perimembranous ventricular septal defects with inlet to outlet extension with the Amplatzer Vascular Plug-II.

Transcatheter closure of perimembranous ventricular septal defect is an alternative to surgery in selected small to medium defects with a reasonable distance between the defect and the aortic or tricuspid valves. Surgical closure is generally preferred for large defects with an inlet to outlet extension, aortic malalignment, or deficient aortic rim. We report two successful cases of percutaneous closure of large perimembranous ventricular septal defects with inlet to outlet extension using an Amplatzer Vascular Plug-II via a retrograde approach. Transcatheter closure of large perimembranous ventricular septal defect with inlet to outlet extension is feasible and facilitated by the characteristics of the Amplatzer Vascular Plug-II device and the specific implantation technique.

Novel Strategy for Predicting Conduction Abnormalities During Transcatheter Closure of Perimembranous Ventricular Septal Defect in Adults.

BACKGROUND: In this study we evaluated the feasibility and efficacy of predicting conduction system abnormalities under 3-dimensional (3D) electroanatomic mapping guidance during transcatheter closure of perimembranous ventricular septal defects (pmVSDs) in adults.Methods and Results:The distribution of the His-Purkinje system (HPS) close to the margins of pmVSDs in the left ventricle was identified using 3D electroanatomic mapping and near-field HPS was further confirmed by different pacing protocols. Of the 20 patients in the study, 17 (85%) were successfully treated by transcatheter intervention. The minimum distance between the margins of the pmVSD and near-field HPS, as measured by 3D electroanatomic mapping, ranged from 1.3 to 3.9 mm (mean [± SD] 2.5±0.7 mm). Five patients with a minimum distance <2 mm had a higher risk (3/5; 60%) for adverse arrhythmic events, whereas patients with a distance >2 mm were at a much lower risk (1/15; 6.7%) of procedure-related conduction block (P=0.032). No other adverse events were recorded during the follow-up period (median 30 months). CONCLUSIONS: A minimum distance between the pmVSD and near-field HPS <2 mm was associated with a relatively high risk of closure-related conduction block. 3D electroanatomic mapping may be helpful in guiding decision making for transcatheter closure and reduce the incidence of adverse arrhythmic events.

Echocardiographic evaluation of ventricular septal defects.

Ventricular septal defects (VSDs) are the most common forms of acyanotic congenital heart disease accounting for 37% of congenital heart disease in children. A VSD is defined by parts of the ventricular septum involved. There are four major types of VSDs: perimembranous, muscular, outlet, and inlet VSDs. Echocardiography is the most important clinical tool to help diagnose and characterize a VSD. Although most VSDs are clinically nonsignificant or close on their own, echocardiography with Doppler and color flow mapping can be used to provide accurate anatomic and hemodynamic evaluation of VSDs in order to determine if surgical or transcatheter-based intervention is needed. Hence, understanding how to use echocardiography to characterize VSDs is of crucial importance when caring for patients with adult congenital heart disease.

Feasibility and 1-year outcome of transcatheter closure of perimembranous ventricular septal defects with different devices.

OBJECTIVE: To analyze feasibility of closing perimembranous ventricular septal defect (pmVSD) with different devices by percutaneous approach and determining initial 1-year outcome of the procedure. BACKGROUND: Transcatheter closure of pmVSD remains controversial due to a previous higher incidence of complete heart block (CHB), especially with the Amplatzer pmVSD occluder. Recently, several devices have been used to minimize the procedure-related complications. METHODS AND MATERIALS: A retrospective longitudinal cohort study of 133 patients who underwent transcatheter closure of pmVSD from September 2009 to March 2015. The median age and weight at intervention were 7.1 years (ranging from 9 months to 28 years) and 21.2 kg (ranging from 6.4 to 93 kg). RESULTS: Transcatheter pmVSD closure was successfully performed in 129 cases (97%) using 13 different devices. There were two new onset severe aortic regurgitation (AR) (1.5%), one new onset severe tricuspid regurgitation (0.7%), and one CHB (0.7%). Immediately after procedure, 41 patients (30.8%) had mild to moderate residual shunt and 27 patients (20.8%) had trivial to mild AR. At 1 year, there was no CHB and 10 patients (9.6%) had tiny to mild residual shunt and 10 patients (9.6%) had trivial AR. CONCLUSION: With proper case selection, good expertise, and judicious use of various devices with respect to anatomic details of pmVSD, transcatheter closure is feasible with satisfactory early outcome.

Off-label use of duct occluder devices to close hemodynamically significant perimembranous ventricular septal defects: A multicenter experience.

OBJECTIVE: We sought to evaluate the feasibility, technical aspects, and outcome of transcatheter perimembranous ventricular septal defect (pmVSD) closure using duct occluder devices with a single retention disc. BACKGROUND: Use of duct occluder devices to close pmVSD seems a promising alternative therapy. However, limited data exist on this technique. METHODS: From 2010 to 2016, 222 patients (female 47.7%) were identified from databases of five participating institutions in whom pmVSD closure was attempted using an Amplatzer Duct Occluder I or Lifetech duct occluder device. RESULTS: Patients ranged in age from 0.7 to 52 years (median, 7.0 years) and in weight from 4.0 to 70 kg (median, 18.0 kg). The mean size of the VSD was 6.8 ± 2.2 mm. A large defect (> 6 mm) was present in 137 patients (61.7%). Device closure was successful in 218 patients (98.2%). The 10/8 mm device was used in most patients (n = 85, 38.3%), and the vascular approach was from the femoral vein in 169 patients (76.1%). There were 18 early complications in 17/218 patients (7.8%). Three patients (1.4%) developed complete heart block (transient n = 2; requiring permanent pacing n = 1). Median follow-up was 6 months (6 months-6 years). A mild residual shunt was seen in 10 patients at 6 months follow-up. CONCLUSIONS: The immediate results of transcatheter pmVSD closure using a duct occluder device with a single retention disc are promising. It is an effective technique with a lower rate of complications than for other currently available devices.

Echocardiography-guided percutaneous closure of perimembranous ventricular septal defects without arterial access and fluoroscopy.

BACKGROUND: Traditional percutaneous device closure of perimembranous ventricular septal defects (PmVSDs) is a minimally invasive technique, but can result in high radiation exposure and can result in potential arterial complications. Here, we aimed to assess the safety and feasibility of device closure of PmVSDs via the femoral vein approach under transesophageal echocardiography (TEE) guidance in children. METHODS: From January 2014 to December 2017, a total of 46 PmVSD patients (mean age, 6.5 ± 2.3 years [range, 4.2-12.0 years]; mean body weight 22.1 ± 6.6 kg [range, 16.0-38.5 kg]; VSD diameter, 4.1 ± 0.6 mm [range, 3.2-5.0 mm]) underwent attempted transcatheter closure via the femoral vein approach under the guidance of TEE without fluoroscopy. RESULTS: The transcatheter occlusion procedure under TEE guidance was successful in 44 (95.7%) patients. Surgery was necessary in 2 (4.3%) patients. The procedure duration was 28.2 ± 8.7 min (range, 12.0-42.0 min). One patient had immediate post-operative trivial residual shunt and three patients had immediate incomplete right bundle branch block (IRBBB) after operation; the new IRBBB in 1 case was noted in the first postoperative month. No residual shunt was noted at 3 months after the procedure, and no intervention related complications were detected at 1-24 months follow-up. CONCLUSIONS: Percutaneous device closure of PmVSDs under TEE guidance solely by femoral vein approach is effective and safe, avoids radiation exposure, potential arterial complications and a surgical incision.

A complex ventricular septal defect following transcatheter aortic valve implantation evaluated by 3D transthoracic echocardiography.

Transcatheter aortic valve implantation (TAVI) has revolutionized the treatment of elderly patients with symptomatic severe aortic valve stenosis. Among the possible TAVI complications, a rare one is the annular/left ventricular outflow tract rupture. We report a rare case of a late complex ventricular septal defect (VSD) following TAVI with a balloon-expandable prosthesis, conservatively managed. Our case demonstrates the role of 3D transthoracic echocardiography (3DTTE) in the accurate diagnosis of this TAVI complication and suggests that, in some cases, it can be used as an alternative to other diagnostic tools, such as transesophageal echocardiography, cardiac catheterization, and computed tomography.

Quantification of the area and shunt volume of multiple, circular, and noncircular ventricular septal defects: A 2D/3D echocardiography comparison and real time 3D color Doppler feasibility determination study.

BACKGROUND: Quantification of defect size and shunt flow is an important aspect of ventricular septal defect (VSD) evaluation. This study compared three-dimensional echocardiography (3DE) with the current clinical standard two-dimensional echocardiography (2DE) for quantifying defect area and tested the feasibility of real time 3D color Doppler echocardiography (RT3D-CDE) for quantifying shunt volume of irregular shaped and multiple VSDs. METHODS: Latex balloons were sutured into the ventricles of 32 freshly harvested porcine hearts and were connected with tubing placed in septal perforations. Tubing was varied in area (0.13-5.22 cm²), number (1-3), and shape (circle, oval, crescent, triangle). A pulsatile pump was used to pump "blood" through the VSD (LV to RV) at stroke volumes of 30-70 mL with a stroke rate of 60 bpm. Two-dimensional echocardiography (2DE), 3DE, and RT3D-CDE images were acquired from the right side of the phantom. RESULTS: For circular VSDs, both 2DE and 3DE area measurements were consistent with the actual areas (R² = 0.98 vs 0.99). For noncircular/multiple VSDs, 3DE correlated with the actual area more closely than 2DE (R² = 0.99 vs 0.44). Shunt volumes obtained using RT3D-CDE positively correlated with pumped stroke volumes (R² = 0.96). CONCLUSIONS: Three-dimensional echocardiography (3DE) is a feasible method for determining VSD area and is more accurate than 2DE for evaluating the area of multiple or noncircular VSDs. Real-time 3D color Doppler echocardiography (RT3D-CDE) is a feasible method for quantifying the shunt volume of multiple or noncircular VSDs.

Severe right ventricular hypertrophy in a patient with extracardiac and intracardiac shunt.

Cardiac MRI is a complementary and confirmatory modality to a clinical echocardiography in diagnosing patients with complex adult congenital heart disease, especially in presence of great vessel abnormalities. We present a unique case of a patient with pulmonary hypertension (PH), severe right ventricular hypertrophy, Gerbode defect, and a large patent ductus arteriosus (PDA). The diagnosis of PDA was not visualized on prior serial echocardiograms and discovered on a comprehensive cardiac MRI/Chest MR angiogram.

Simultaneous transcatheter therapy for perimembranous ventricular septal defect combined with patent ductus arteriosus.

OBJECTIVE: This study aims to assess the clinical efficiency and safety of simultaneous transcatheter interventional treatment for perimembranous ventricular septal defect (pmVSD) combined with patent ductus arteriosus (PDA). METHODS: Twenty-five patients with pmVSD and PDA treated with simultaneous transcatheter interventions from April 2004 to December 2015 were included in this study. The mean age was 9.80 ± 8.14 years and the mean weight was 29.76 ± 14.82 Kg. Transthoracic echocardiography (TTE) and angiography were performed immediately after the procedure. Patients were re-examined by electrocardiogram, X-ray, and TTE at 2 days, 1 month, 3 months, and 6 months postoperatively. RESULTS: The interventional procedure was successfully performed in all 25 patients. No intraoperative complication was noted. TTE examination of the VSD and PDA immediately after the procedure showed no residual shunt and the occluder was well positioned. Among these patients, four patients showed electrocardiogram changes after the procedure that resolved after drug therapy. The cardiothoracic ratio, left atrial diameter, left ventricular end-systolic diameter, and left ventricular end-diastolic diameter recovered to normal in most patients at 6 months postoperatively. CONCLUSIONS: Simultaneously transcatheter interventional therapy is a safe and effective method for pmVSD combined with PDA.

Transcatheter Closure of Perimembranous Ventricular Septal Defects with Left Ventricular to Right Atrial Shunt.

During the development of so-called aneurysmal transformation of perimembranous ventricular septal defects (pmVSD), tricuspid valve (TV) morphology and function may be altered resulting in left ventricular (LV) to right atrial (RA) shunting. The feasibility and outcome of interventional closure of these pmVSD has not been investigated so far. Interventional closure of pmVSD associated with mild to moderate LV-to-RA shunt was performed in four patients (aged 6.5-12.5 years). pmVSD were closed under fluoroscopic guidance by establishing an arteriovenous wire loop via a femoral artery and advancing the delivery sheath from a femoral vein. Before device release (or withdrawal if necessary), residual shunting across the device and TV valve function was investigated by transthoracic echocardiography and LV angiography. pmVSD sizes of 4, 5.5, 8 and 8.5 mm were closed with a 4/4 and 6/6 Amplatzer duct occluder II and an 8- and 10-mm Amplatzer muscular VSD occluder device, respectively. There were no or only minor residual postinterventional LV-to-RA shunts. No atrioventricular blocks were observed during a mean follow-up of 12.5 months (range 6.5-17 months). Transthoracic echocardiography indicated that the elimination of the VSD jet pushing the antero-superior TV leaflet open is the key mechanism for LV-to-RA shunt reduction after transcatheter pmVSD closure. Interventional closure in pmVSD associated with mild to moderate indirect LV-to-RA shunting is feasible and results in significant reduction in or elimination of LV-to-RA shunting.

Periconceptional parental conditions and perimembranous ventricular septal defects in the offspring.

BACKGROUND: The perimembranous ventricular septal (pVSD) defect is the most common congenital heart disease phenotype. Several parental factors are associated with pVSD risk in the offspring. To contribute to the future prevention of pVSDs, we investigated associations with nongenetic parental conditions. METHODS: In a case-control study with standardized data collection at 17 months after birth, 115 parents of a child with pVSD and 484 parents of a healthy child completed questionnaires about periconceptional nongenetic conditions. Univariable and multivariable logistic regression analyses were used to estimate odds ratios (OR) with 95% confidence intervals (95% CI). RESULTS: Complete data were available for 588 families (98%). Maternal risk conditions associated with pVSD offspring were a positive family history of congenital heart disease (OR, 2.61; 95%CI, 0.98-6.91), medication use (OR, 1.80; 95%CI, 1.13-2.85) and advanced age (OR, 1.07; 95%CI, 1.02-1.12). Exposure to phthalates (OR, 1.93; 95%CI, 1.05-3.54) was the only paternal risk condition associated with pVSD offspring. CONCLUSION: Four periconceptional parental conditions contributed to pVSD risk in the offspring. Couples planning pregnancy should be counseled on these risk conditions which are partially modifiable to contribute to the future prevention of pVSDs.

Transcatheter closure of perimembranous ventricular septal defects: initial human experience with the Amplatzer® membranous VSD occluder 2.

Although effective, transcatheter closure of perimembranous ventricular septal defects (pmVSD) with the Amplatzer Membranous VSD Occluder (AGA Medical Corporation, MN) carries a substantial risk of complete heart block, prompting many to abandon this intervention. A newly designed Amplatzer device for pmVSD was modified, in part, to minimize this risk. After rigorous preclinical testing, we report the first human experience with the Amplatzer Membranous VSD Occluder 2 (AGA Medical Corporation) in two patients (a 5-year old with a 12-mm pmVSD and a 26-year-old male with a 8-mm defect). Both procedures were successful, with no adverse events at 7 and 4 weeks of follow-up, respectively. Herein, we discuss characteristics of the new device, potential advantages compared to the prior version, and main technical aspects related to the procedure.

Double-chambered right ventricle with pulmonary valve endocarditis.

We describe a rare case of pulmonary valve endocarditis associated with a double-chambered right ventricle in an adolescent male highlighting the two-dimensional and three-dimensional transthoracic echocardiographic findings. He was managed with aggressive antibiotic therapy followed by surgery. The echocardiographic findings were confirmed during surgery.

Ventricular Septal Defect and Right-Sided Infective Endocarditis.

Right-sided infective endocarditis (IE), which represents a small but not negligible percentage of IE cases, can be observed in patients with congenital heart diseases. We discuss the case of a young woman with unrepaired perimembranous ventricular septal defect and repeated episodes of right ventricle and tricuspid valve IE with septic embolism.