scholarly journals A Reassessment of the anatomical features of multiple ventricular septal defects

Author(s):  
Diane E. Spicer ◽  
Robert Anderson ◽  
Ujjwal Chowdhury ◽  
Lakshmi Sankhyan ◽  
Niwin George ◽  
...  

Over the course of time, new developments associated with embryogenesis of the murine heart have served to clarify the developmental processes observed in the human heart. This evidence allows for creation of a developmental framework for many congenital cardiac defects. Here, 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. Based on developmental evidence, furthermore, all interventricular communications can be placed into one of three groups, namely, those 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 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.

1996 ◽  
Vol 6 (4) ◽  
pp. 281-290 ◽  
Author(s):  
David A. Lewis ◽  
Christopher A. Loffredo ◽  
Adolfo Correa-Villaseñor ◽  
P. David Wilson ◽  
Gerard R. Martin

AbstractAlthough ventricular septal defect is the most common form of congenital heart disease, its epidemiology is not completely understood. The Baltimore-Washington Infant Study, a case-control study of congenital cardiovascular malformations, enrolled 4,390 case infants during the study period 1981–1989. Ventricular septal defect, the most common malformation, was present in 1,465 (33%) of affected infants. The purpose of this report was to describe the epidemiological characteristics of membranous and muscular ventricular septal defects, the most common types of ventricular septal defects. Membranous ventricular septal defects (n=895) and muscular ventricular septal defects (n=429) accounted for 90% of all ventricular defects. During the study, prevalence increased from 0.9 to 2.1 /1000 live births. Associated non-cardiac defects, such as chromosomal defects, genetic syndromes, and defects of other organs were more common among infants with ventricular septal defect than among control infants, and more common among infants with membranous ventricular defect (20.8%) than among those with muscular defects (7.5%). Infants with ventricular septal defects and non-cardiac defects had increased mortality in comparison to infants with isolated ventricular septal defects or controls. Epidemiological information derived from population-based studies provide valuable data for counseling families and may allow for examination of etiologic factors.


2002 ◽  
Vol 124 (5) ◽  
pp. 984-990 ◽  
Author(s):  
Rudolf P. Beekman ◽  
Margot M. Bartelings ◽  
Mark G. Hazekamp ◽  
Adriana C. Gittenberger-de Groot ◽  
Jaap Ottenkamp

1997 ◽  
Vol 7 (4) ◽  
pp. 471-484 ◽  
Author(s):  
Michael A. Gatzoulis ◽  
Jia Li ◽  
Siew Yen Ho

AbstractMany of the controversies surrounding the description of ventricular septal defects arise from differences in the perspectives from which they are viewed. In this review, we analyse these defects as seen in cross-sectional echocardiographic images, correlating them with morphologic specimens. The classification we advocate, which now has a suitable pedigree, is a simple descriptive method distinguishing between perimembranous, muscular, and doubly committed types of defects. The approach is to categorise the defects as seen from the right ventricle, the usual port of access for surgeons. The term ‘perimembranous’ highlights the proximity of the atrioventricular conduction axis to the margin of the defects in which the remnant of the membranous septum forms a direct border. This system is applicable to all interventricular communications, no matter how malformed the heart may be in which they are enclosed.


1991 ◽  
Vol 12 (2) ◽  
pp. 123-125
Author(s):  
Elizabeth R. de Oliveira e Silva ◽  
Michael S. Snyder ◽  
John E. O'Loughlin ◽  
Arthur A. Klein ◽  
Margret S. Magid ◽  
...  

2014 ◽  
Vol 25 (6) ◽  
pp. 1099-1110 ◽  
Author(s):  
Adrian Crucean ◽  
William J. Brawn ◽  
Diane E. Spicer ◽  
Rodney C. Franklin ◽  
Robert H. Anderson

AbstractBackgroundAlthough holes, or channels, between the ventricles are the commonest congenital cardiac malformations, there is still no consensus as to how they can best be described and categorised. So as to assess whether it is possible to produce a potentially universally acceptable system, we have analysed the hearts categorised as having ventricular septal defects in a large archive held at Birmingham Children’s Hospital.Materials and methodsWe analysed all the hearts categorised as having isolated ventricular septal defects, or those associated with aortic coarctation or interruption in the setting of concordant ventriculo-arterial connections, in the archive of autopsied hearts held at Birmingham Children’s Hospital, United Kingdom.ResultsWe found 147 hearts within the archive fulfilling our criterions for inclusion. All could be classified within one of three groups depending on their borders as seen from the right ventricle. To provide full description, however, it was also necessary to take account of the way the defects opened to the right ventricle, and the presence or absence of alignment between the septal components.ConclusionsBy combining information on the phenotypic specificity defined on the basis of their borders, the direction of opening into the right ventricle, and the presence or absence of septal malalignment, it proved possible to categorise all hearts examined within the archive of Birmingham Children’s Hospital. Our findings have necessitated creation of new numbers within the European Paediatric Cardiac Code.


2009 ◽  
Vol 19 (6) ◽  
pp. 563-567 ◽  
Author(s):  
Alessia Del Pasqua ◽  
Gabriele Rinelli ◽  
Alessandra Toscano ◽  
Roberta Iacobelli ◽  
Cristina Digilio ◽  
...  

AbstractAimsWe investigated the prevalence, type, and course of congenital cardiac defects and systemic hypertension in our patients with Williams-Beuren-Beuren syndrome.Methods and resultsWe reviewed the clinical records of all patients with Williams-Beuren syndrome examined between 1981 and 2006. We identified 150 patients, aged from 7 months to 45 years, with a follow-up from 6 months to 25 years, the mean being 6.4 years. A cardiac anomaly was present in 113 of the 150 patients (75%). Defects were typical in over four-fifths of the group. We found supravalvar aortic stenosis in 73 of 113 patients (64.6%), isolated in 43. Pulmonary stenosis, isolated in 18 cases, was found in 51 of 113 (45.1%), while aortic coarctation and mitral valvar prolapse were each found in 7 (6.2%), 3 of the lesions is isolation. Atypical defects were found in 19 patients, tetralogy of Fallot in 2, atrial septal defects in 4, aortic and mitral valvar insufficiencies in 1 each, bicuspid aortic valves in 2, and ventricular septal defects in 9, 4 of the last being isolated. Systemic hypertension, observed in 33 patients (22%), was poorly controlled in 10. Diagnostic and/or interventional cardiac catheterization was undertaken in 24 patients, with 30 surgical procedures performed in 26 patients. Of the group, 3 patients died.ConclusionCardiac defects were present in three-quarters of our patients. Pulmonary arterial lesions generally improved, while supravalvar aortic stenosis often progressed. Atypical cardiac malformations, particularly ventricular septal defects, occurred frequently. Systemic hypertension was found in one-fifth, even in the absence of structural cardiac defects. The short-term mortality was low.


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