Clinical and morphologic features of perimembranous ventricular septal defect with overriding of the aorta – the so-called Eisenmenger ventricular septal defect. A study making comparisons with tetralogy of Fallot and perimembranous ventricular defect without aortic overriding

2000 ◽  
Vol 10 (4) ◽  
pp. 343-352 ◽  
Author(s):  
Toyoki Fukuda ◽  
Takaaki Suzuki ◽  
Tsutomu Ito

AbstractThe aim of our study was to elucidate the clinical and morphologic features of those perimembranous ventricular septal defects which extend between the ventricular outlets, particularly when found in association with anterior deviation of the muscular outlet septum and overriding of the aorta – the so called Eisenmenger ventricular septal defect. From 1990 through 1998, we have undertaken surgical correction in 203 patients with perimembranous ventricular septal defect. Of these, 15 patients had the Eisenmenger ventricular septal defect. We conducted retrospective analyses of the clinical records, catheterization data, and angiocardiographic and echocardiographic finding of these patients. Comparative studies were then made with the patients having tetralogy of Fallot, and those with simple perimembranous ventricular septal defects without overriding of the aorta. In the patients with the Eisenmenger ventricular septal defect, the extent of anterior deviation of the outlet septum was comparable with that seen in tetralogy of Fallot, but there was less rightward displacement of the aortic valvar orifice. In contrast to earlier investigators, however, we found evidence of progressive narrowing of the subpulmonary infundibulum in those with the Eisenmenger defect. These morphological features were reflected in the clinical features, since all patients showed evidence of increased pulmonary flow and congestive heart failure in early infancy, but with twothirds of them subsequently developing right-to-left shunting. We conclude, therefore, that the Eisenmenger ventricular septal defect is a discrete cardiac abnormality in which the morphologic substrate of anterior deviation of the outlet septum gives rise to a potential for progressive narrowing of the subpulmonary infundibulum. Surgical management, therefore, needs to take account of such narrowing as an additional cardinal morphologic feature.

2005 ◽  
Vol 15 (5) ◽  
pp. 534-536 ◽  
Author(s):  
Per G. Bjørnstad ◽  
Bjarne Smevik

We describe a 6-year-old boy with a right-sided heart part of the Kartagener syndrome, complicated by presence of a perimembranous ventricular septal defect. The defect was closed interventionally using the Amplatzer™ asymmetrical occluder for ventricular septal defects. The procedure was event-free. Non-operative closure of a ventricular septal defect in this patient demonstrated no other difficulty than mirror-imaged thinking, thus giving evidence that such treatment also can be offered to patients with positional anomalies.


2019 ◽  
Vol 6 (5) ◽  
pp. 16-23
Author(s):  
I. A. Kozyrev ◽  
A. S. Golovkin ◽  
E. S. Ignatieva ◽  
P. M. Dokshin ◽  
E. V. Grekhov ◽  
...  

Objective. Phenotypic analysis of cardiac mesenchymal cells from patients with a ventricular septal defect and tetralogy of Fallot.Methods. The study included cardiac mesenchymal cells from 8 patients with ventricular septal defect and from 18 patients with tetralogy of Fallot who underwent surgery. Using the flow cytometry method, the content of the following antigens on the cell surface was evaluated: CD31, CD34, CD90, CD117, CD146, CD166, PDGFRB.Results. Cardiac mesenchymal cells from patients with ventricular septal defect are statistically significantly different from patients with tetralogy of Fallot in surface markers CD90 and PDGFRB. The content of a stem cell marker PDGFRB tends to decrease with age in patients with tetralogy of Fallot.Conclusion. The cells obtained from myocardial tissue from patients with tetralogy of Fallot and from patients with ventricular septal defects have characteristics of mesenchymal stem cells. The differences found between the cells from the two groups of patients indicate that pathology can affect the phenotype of cardiac mesenchymal cells. Apparently, the stem properties of the mesenchymal heart cells tend to decrease with age.


2011 ◽  
Vol 21 (3) ◽  
pp. 271-280 ◽  
Author(s):  
James R. Bentham ◽  
Arjun Gujral ◽  
Satish Adwani ◽  
Nick Archer ◽  
Neil Wilson

AbstractObjectiveTo describe the difficulties and differing techniques in the transcatheter placement of amplatz ventricular septal defect devices to close perimembranous ventricular septal defects and place these in the context of the expanding literature on ventricular septal defect catheter closure.BackgroundSurgery remains the established first-line therapy for closure of haemodynamically significant perimembranous ventricular septal defects. Transcatheter techniques appeared to promise a possible alternative, obviating the need for cardiac surgery. However, significant technical and anatomical constraints coupled with ongoing reports of a high incidence of heart block have prevented these hopes from being realised to any significant extent. It is likely that there are important methodological reasons for the high complication rates observed. The potential advantages of transcatheter perimembranous ventricular septal defect closure over surgery warrant further exploration of differing transcatheter techniques.MethodsBetween August, 2004 and November, 2009, 21 patients had a perimembranous ventricular septal defect closed with transcatheter techniques. Of these, 14 were closed with a muscular amplatz ventricular septal defect device. The median age and weight at device placement were 8 years, ranging from 2 to 19 years, and 18.6 kilograms, ranging from 10 to 21 kilograms, respectively.ResultsThere were 25 procedures performed on 23 patients using 21 amplatz ventricular septal defect devices. Median defect size on angiography was 7.8 millimetres, ranging from 4 to 14.3 millimetres, with a median device size of 8 millimetres, ranging from 4 to 18 millimetres, and a defect/device ratio of 1.1, with a range from 0.85 to 1.33. Median procedure time was 100 minutes, with a range from 38 to 235 minutes. Adverse events included device embolisation following haemolysis in one, and new aortic incompetence in another, but there were no cases of heart block. Median follow-up was 41.7 months, with a rangefrom 2 to 71 months.ConclusionsEvaluating transcatheter closure of perimembranous ventricular septal defect using amplatz ventricular septal defect devices remains important, if a technically feasible method with low and acceptable complication rates is to be identified. Incidence of heart block may be minimised by avoiding oversized devices, using muscular devices, and accepting defeat if an appropriately selected device pulls through. Given the current transcatheter technologies, the closure of perimembranous ventricular septal defects should generally be performed in children when they weigh at least 10 kilograms.


2002 ◽  
Vol 10 (4) ◽  
pp. 314-317 ◽  
Author(s):  
Sajan Koshy ◽  
Gopalraj Sumangala Sunil ◽  
Sivadas Radha Anil ◽  
Seetharaman Dhinakar ◽  
Krishnanaik Shivaprakasha ◽  
...  

Tricuspid leaflet detachment improves visualization and accuracy of closure of ventricular septal defects via the transatrial route. Between July 1998 and March 2001, surgical correction was performed in 296 cases of isolated ventricular septal defect, 215 cases of tetralogy of Fallot, and 16 cases of double-outlet right ventricle. Of these, 132 patients (79 with isolated ventricular septal defect, 49 with tetralogy of Fallot, and 4 with double-outlet right ventricle) underwent transatrial repair with temporary detachment of tricuspid leaflets for ventricular septal defect closure. The septal leaflet was detached in most cases, with anterior or posterior leaflets being detached when indicated. Median duration of intensive care was 3.6 days, and median hospital stay was 7 days. There was no incidence of tricuspid regurgitation attributable to leaflet detachment, as confirmed by postoperative echocardiography. Reoperation was not required for a residual defect or tricuspid regurgitation. The benefits of temporary leaflet detachment for transatrial repair of various difficult defects far outweigh the risk of postoperative tricuspid regurgitation.


2018 ◽  
Vol 11 (4) ◽  
pp. NP158-NP160
Author(s):  
Thomas Krasemann ◽  
Ingrid van Beynum ◽  
Pieter van de Woestijne

A planned combined perventricular and “open heart” surgical closure of multiple ventricular septal defects had to be modified intraoperatively due to a technical fault disabling echocardiographic guidance. Through an atriotomy, device closure of a muscular defect and patch closure of a perimembranous ventricular septal defect were performed. In unusual situations, collaboration of the surgical and interventional team is crucial.


PEDIATRICS ◽  
1989 ◽  
Vol 83 (2) ◽  
pp. 200-203
Author(s):  
Gerard R. Martin ◽  
Lowell W. Perry ◽  
Charlotte Ferencz

The Baltimore-Washington Infant Study is an ongoing case-control study of congenital cardiovascular malformations in infants in whom the clinical diagnoses have been confirmed by echocardiography, catheterization, surgery, or autopsy. An increase in the prevalence of ventricular septal defects was detected in 1,494 infants with congenital cardiovascular malformations between 1981 and 1984. The prevalence of congenital cardiovascular malformations increased from 3.6 to 4.5 per 1,000 live births (P<.025) and the prevalence of ventricular septal defect increased from 1.0 to 1.6 per 1,000 live births (P< .001). The increase in ventricular septal defects accounted for the total increase in congenital cardiovascular malformations. The prevalence of isolated ventricular septal defect increased from 0.67 to 1.17 per 1,000 live births (P<.001). The prevalence of ventricular septal defect with associated coarctation of the aorta, patent ductus arteriosus, atrial septal defect, and pulmonic stenosis did not change. The prevalence of ventricular septal defect diagnosed by catheterization, surgery, and autopsy did not change; however, defects diagnosed by echocardiography increased from 0.30 to 0.70 per 1,000 live births (P<.001). It is concluded that the reported increase in prevalence of ventricular septal defect is due to improved detection of small, isolated ventricular septal defects and that there is no evidence of an "epidemic."


2020 ◽  
Author(s):  
Long Wang ◽  
Lin Xie ◽  
Weiqiang Ruan ◽  
Tao Li ◽  
Changping Gan ◽  
...  

Abstract Background: This report presents updated data and mid-term follow-up information to a former study introducing the novel technique of percutaneous-perventricular device closure of doubly committed subarterial ventricular septal defect. Methods: Thirty-eight patients were added to the former series. There were 54 patients in total who had isolated doubly committed subarterial ventricular septal defects and underwent percutaneous-perventricular device closure. Closure outcomes and possible complications were measured in the hospital and during the 2.5-year follow-up. Results: Surgery was successful in 53 patients (98.1%). There was no death, residual shunt, new valve regurgitation or arrhythmia either perioperatively or during the entire follow-up period. Only one patient developed pericardial effusion and tamponade in the former series. The mean hospital stay was 3.2±0.6 days (range, 3.0 to 6.0 days), and only one unsuccessful case needed blood transfusion (1.9%). Conclusions: The percutaneous-perventricular device closure of isolated doubly committed subarterial ventricular septal defects appeared to be safe. Close monitoring for bleeding is essential postoperatively, especially in younger patients. This technique is generally safe with acceptable mid-term follow-up.


2019 ◽  
Vol 27 (7) ◽  
pp. 529-534
Author(s):  
Noor Mohammad Noori ◽  
Alireza Teimouri

Background Ventricular septal defect, the most common congenital heart defect, is characterized by an opening between the ventricles. This study aimed to evaluate the clinical course and associations between the characteristics of ventricular septal defect. Methods This cross-sectional study was conducted on 1498 children with ventricular septal defects, aged <19 years, who were referred to our center between 2003 and 2018. The diagnosis was suspected from a combination of clinical and laboratory findings, and confirmed by transthoracic echocardiography. Results Of the 1498 children, 54.9% were boys, 78.4% of defects were perimembranous, 30.4% of patients had pulmonary hypertension, 67.5% had regular follow-up, and 76 (5.1%) had complications including 28 (1.9%) with Eisenmenger syndrome; 10 died due to Eisenmenger syndrome during follow-up. The defects closed spontaneously in 38.9% and after surgery in 20.9%. Boys tended to have more perimembranous, inlet, and outlet forms (56.40%, 50.60%, 51.90%, respectively), whereas girls had more muscular types (51.80%). Most patients with pulmonary hypertension had perimembranous defects (83.10%). The majority of patients with pulmonary hypertension had large (63.40%) or moderate (36.60%) defects. Pulmonary hypertension had a significant association with defect size ( p < 0.001). After surgery, residual defects were found mostly in cases of large defects (84.60%). Most patients with spontaneous closure were younger than 4 years. Conclusion Almost four-fifths of children with ventricular septal defects had perimembranous types, and almost one-third had pulmonary hypertension which was associated with defect size. Two-fifths of the defects closed spontaneously. There was a low incidence of complications after surgery.


Sign in / Sign up

Export Citation Format

Share Document