A reassessment of the anatomical features of multiple ventricular septal defects

Abstract

Background Over the course of time, new developments associated with the embryogenesis of the murine heart have served to clarify the developmental processes observed in the human heart. This evidence allows for the creation of a developmental framework for many congenital cardiac defects. Aims We aim to solidify the framework related to the categorization of both solitary and multiple ventricular septal defects. Materials and Methods 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. Results Basis of 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. Conclusions As we show, appropriate surgical management requires an 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.

Effect of deletion of the protein kinase PRKD1 on development of the mouse embryonic heart

“…On external examination, this heart had a more rounded appearance with prominent and separate right and left ventricular apices (Figure 6b and Figure S4b ) in comparison to WT (Figure 6a ; Figure S4a ). Although dual apices are seen in the normal developing mouse heart (Spicer et al., 2022 ), the position of the right ventricular apex in this heart was abnormal. This heart was processed with other hearts which were found to be morphologically normal, and there was no suggestion that this heart had acquired a separate processing artefact; this heart was not mechanically damaged.…”

Multiple ventricular septal defects associated to anomalous venous returns, mitral valve disease, myocardium hypertrophy, and right outflow obstruction: a multimodality imaging assessment

“…The third arrangement is found when a large and solitary defect in the apical part of the muscular septum is crossed in the RV by trabeculations, giving the spurious impression of multiple defects. 2 Our case belonged to the first arrangement because coexist a huge perimembranous and a muscular VSD.…”

“…Associated cardiac and extracardiac anomalies were reported in one‐third to three‐quarters of patients such as bilateral superior caval veins, left superior caval vein to coronary sinus, Ebstein‐like anomaly of the tricuspid valve, mitral valve diseases, double outlet right ventricle, coarctation of aorta 2 …”

“…The second arrangement is found when a failed coalescence of muscular ventricular septum occurs during cardiac development. The third arrangement is found when a large and solitary defect in the apical part of the muscular septum is crossed in the RV by trabeculations, giving the spurious impression of multiple defects 2 …”

Outlet Ventricular Septal Defect: A Case Report on the Importance of Multi-Window Sonographic Assessment

“…Ventricular septal defects are the most common form of congenital heart disease in children and the second most common in adults. [4][5][6] VSDs have a wide range of appearances and can be located across all areas of the ventricular septum. These left-to-right shunts are created due to early fetal morphogenesis failure of the membranous, trabecular, infundibular, atrioventricular, and inlet areas of the ventricular septum.…”