Importance of (perimembranous) ventricular septal aneurysm in the natural history of isolated perimembranous ventricular septal defect

doi:10.1016/0002-9149(86)90903-3

The American Journal of Cardiology

Volume 57, Issue 4, 1 February 1986, Pages 268-272

https://doi.org/10.1016/0002-9149(86)90903-3 Get rights and content

Abstract

records were reviewed of 247 patients found to have isolated perimembranous ventricular septal defect (VSD) on cross-sectional echocardiography. Patients were separated into 2 groups: those in whom a perimembranous ventricular septal aneurysm (VSA) was associated with the VSD (group A, 77% of patients) and those in whom a VSA was not present (group B, 23%). The VSD was assessed by clinical (125 in group A, 24 in group B) and hemodynamic criteria (65 in group A, 33 in group B). The median follow-up period was 27 months (range 3 months to 25 years). in group A, the VSD closed spontaneously in 11 % of the patients, improved clinically in 33% and required surgical closure in 11 %. in group S, the VSD closed spontaneously in only 2%, improved clinically in 16% and required surgical closure in 47%. When considering larger VSDs only, 28% required surgery in group A, whereas 84% required surgery in group B (p < 0.001). The VSA was found at the first echocardogaphc examination in 94% of the studies. Thus, VSA is found in a large proportion of patients with perimembranous VSD. it occurs as an early phenomenon and is an important mechanism of closure. The presence of a VSA appears to confer a more favorable prognosis in perimembranous VSD except in natients with Down's svndrome.

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Cited by (69)

Transcatheter closure of aneurysmal perimembranous ventricular septal defect with the Canine Duct Occluder in two dogs
2022, Journal of Veterinary Cardiology
Congenital membranous ventricular septal aneurysm has been reported in dogs and can be associated with a perimembranous ventricular septal defect (VSD). The windsock-like ventricular septal aneurysm is formed by tissue of the membranous ventricular septum and portions of the septal leaflet of the tricuspid valve. We report two dogs that underwent transcatheter closure of perimembranous VSD associated with membranous ventricular septal aneurysm using a commercial device marketed for transcatheter closure of patent ductus arteriosus, the canine duct occluder. Partial closure was achieved in the first dog with reduction in left heart dimensions documented on echocardiography both at one day and nine months after procedure. In the second dog, three-dimensional transesophageal echocardiography, cardiac computed tomography, and a three-dimensionally printed whole heart model were used to evaluate feasibility for transcatheter device closure. Complete closure of the VSD was subsequently achieved. Both cases had good short- to medium-term outcomes, no perioperative complications were observed, and both dogs are apparently healthy and receiving no cardiac medications at 34 months and 17 months after procedure. Transcatheter attenuation of perimembranous VSD with membranous ventricular septal aneurysm is clinically feasible using the canine duct occluder, and multimodal cardiac imaging allows accurate assessment and planning prior to transcatheter intervention for structural heart disease in dogs.
Congenital Heart Disease: A Clinical, Pathological, Embryological, and Segmental Analysis
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The Natural and Unnatural History of Ventricular Septal Defects Presenting in Infancy: An Echocardiography-Based Review
2020, Journal of the American Society of Echocardiography
Citation Excerpt :
The percentage of perimembranous defects that closed spontaneously was greater for those with tricuspid valve aneurysmal tissue at diagnosis than without. This has been described previously in the literature, although there is no clear consensus on the association between tricuspid valve aneurysmal tissue and rate of spontaneous VSD closure.15,29-31 For muscular VSDs that remained open or closed spontaneously, the average indexed VSD area was significantly smaller than muscular VSDs that required surgical intervention.
Ventricular septal defect (VSD), the most common congenital heart defect, accounts for 40% of heart malformations. Despite this prevalence, there remains no consensus on the utility of echocardiography to guide modern-era treatment. In this study, we evaluated patients with isolated VSDs to test the hypothesis that echocardiographic evidence of left ventricular (LV) volume overload and type of VSD are associated with surgical intervention and to identify useful echocardiographic indicators for management of VSDs in infants and children.
We reviewed 350 patients with VSDs diagnosed during the first year of life. Echocardiographic measurements were made at the time of diagnosis and at the endpoint. The VSD area was calculated using inner edge to inner edge dimensions obtained from two planes and indexed to body surface area. Aortic annulus dimension, left atrium to aortic root ratio, LV end-diastolic diameter, left atrial volume, VSD velocity-time integral, ejection fraction, and pulmonary to systemic blood flow ratio (Qp:Qs) were measured using conventional methods.
One hundred seventy-seven muscular (50.5%) and 162 perimembranous (46%) VSDs accounted for the vast majority of defects. Only seven (4%) muscular defects required surgical closure, while 76 (47%) perimembranous defects required surgery. Indexed VSD area, VSD to aortic valve ratio, indexed left atrium volume, LV end-diastolic diameter, VSD velocity-time integral, and Qp:Qs at diagnosis were significantly different between the surgical and nonsurgical groups. Ventricular septal defect area > 50 mm²/m² at initial diagnosis was independently associated with risk for surgery (P = .0055).
Indexed VSD area is an echocardiographic variable that can be easily measured at diagnosis and can provide insight into the likelihood of requiring surgical intervention regardless of the type and location of the defect.
Clinical significance of incidentally detected aneurysms of the membranous ventricular septum in adults by multidetector computed tomography
2015, American Journal of Cardiology
Citation Excerpt :
Therefore, further prospective studies with adequate control groups will be necessary not only to define AMVS as a definite independent predictor of embolic stroke but also to determine the therapeutic strategy for AMVS, such as anticoagulation therapy or surgical removal. Although AMVS has been related to the spontaneous closure or decrease in the size of VSDs in infancy and adolescence,23–26 there is a lack of information on the incidence and natural course of AMVS in adults. As far as we know, this is the first report demonstrating the natural course and clinical implication of AMVS in adults through a longitudinal study.
Although the clinical relevance of aneurysm of the membranous ventricular septum (AMVS) in adults is unclear, the frequency of AMVS detection has been increased because cardiac multidetector computed tomography has been widely adopted for the evaluation of coronary artery disease. Therefore, we aimed to assess the clinical significance of AMVS in a longitudinal study. In 30,120 adults with suspected coronary artery disease who underwent cardiac multidetector computed tomography in 3 hospitals, 52 patients with AMVS were retrospectively selected. We evaluated the clinical symptoms and electrocardiographic abnormalities (cross-sectional study) and the prevalence of embolic stroke (observational study) during a median 40-month (range 6 to 74 months) observation period. For the assessment of embolic stroke, we excluded 9 patients with other explainable embolic sources. Conduction abnormalities were noted in 13 of 52 adults (25%) with AMVS on electrocardiography and embolic stroke occurred in 6 of 43 patients (14%). The mean age and the prevalence of hypertension were significantly higher in the embolic stroke group than in the event-free group (p <0.05). Thrombi were detected in the 11.6% of AMVS, which was significantly related with embolic stroke (p <0.05). The size and morphology did not change in 15 patients with serial follow-up images. In conclusion, our study suggests that AMVS in adults should not be ignored because AMVS may be related to conduction abnormality or embolic stroke, and it does not spontaneously resolve or diminish in size.
Behavior of Unrepaired Perimembranous Ventricular Septal Defect in Young Adults
2010, American Journal of Cardiology
Citation Excerpt :
The mechanism of spontaneous pmVSD closure is less well understood. However, the presence of a ventricular septal aneurysm (VSA) has been considered an early mechanism of closure.4 It has been hypothesized that the septal leaflet of the tricuspid valve covers the defect and might explain why VSDs with a left ventricle to right atrium shunt close less frequently.5
The number of adolescents and young adults with congenital heart defects, including ventricular septal defect (VSD), increases continuously. We evaluated the mid-term outcome of small and unclosed perimembranous VSDs (pmVSDs). All patients with a known unrepaired pmVSD at 16 years of age were selected from our database. The clinical, electrocardiographic, and echocardiographic changes between baseline and the latest follow-up examination were compared. A total of 220 patients (119 males, median age 18 years, interquartile range 7) could be included. During a median follow-up of 6 years (interquartile range 4, range 38), 2 patients died (1%; 1 from sudden death and 1 from end-stage heart failure). Endocarditis occurred in 8 patients (4%). One patient required pacemaker implantation (0.5%) and one required implantable cardioverter-defibrillator implantation (1%). Fifteen patients (7%) required a closing procedure. In 8 patients (4%), the pmVSD closed spontaneously. In the remaining 203 patients (93%), the QRS morphology changed in 5% and 1% lost sinus rhythm (p = 0.0001 and p = 0.015, respectively). The left ventricular ejection fraction and stroke volume index increased from 62 ± 7% to 67 ± 8% and from 41 ± 11 to 44 ± 15 ml/m² (p = 0.0001 and p = 0.035, respectively), the end-systolic diameter decreased, and the end-diastolic diameter did not change. Finally, patients with an open pmVSD developed more pulmonary arterial hypertension during follow-up (from 3% to 9%, p = 0.002). In conclusion, mid-term follow-up of adolescents and young adults with a small and unrepaired pmVSD was not uneventful. Some patients required intervention, but in others, spontaneous closure occurred. Electrocardiographic and structural changes were noticed, for which the clinical significance needs to be determined.
Aneurysm of the membranous septum detected during left ventriculography and demonstrated by cardiac magnetic resonance imaging
2009, International Journal of Cardiology
Citation Excerpt :
Some of the membranous ventricular septal defects (VSD) in infancy transform into aneurysms of the ventricular membranous septums (AMS) later in adulthood [1,2].

View all citing articles on Scopus

¹: Dr. Ramaciotti is supported by a Fellowship in Pediatric Echocardiography, Rio de Janeiro, Brazil.

²: Dr. Keren is supported by the Heiden Israeli Fellowship and the Fogarty International Fellowship, Bethesda, Maryland

View full text

Importance of (perimembranous) ventricular septal aneurysm in the natural history of isolated perimembranous ventricular septal defect

Abstract

Am J Cordial

Am Heart J

Am J Cardiol

Am J Cardiol

Am J Cordial

J Pediatr

J Thorac Cardiovasc Surg

JACC

Am J Cardiol

JACC

Spopontaneous closure of ventricular septal defects

Can Med Assoc

Spontaneous closure of small ventricular septal defects: probability rates in the first five years of life

Am I Dis Child

Spontaneous closure of small ventricular septal defects: ten year follow-up

Pediatrics

Natural history of ventricular septal defect. A study involving 790 cases

Circulation