Assessing the criteria for definition of perimembranous ventricular septal defects in light of the search for consensus
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(2019) 14:76
RESEARCH
Open Access
Assessing the criteria for definition of perimembranous ventricular septal defects in light of the search for consensus Justin T. Tretter1,2* , Vi-Hue Tran3, Seth Gray1, Hieu Ta1, Rohit S. Loomba4, William O’Connor5, Diane E. Spicer6, Andrew C. Cook3 and Robert H. Anderson7
Abstract Background: Discussions continue as to whether ventricular septal defects are best categorized according to their right ventricular geography or their borders. This is especially true when considering the perimembranous defect. Our aim, therefore, was to establish the phenotypic feature of the perimembranous defect, and to establish the ease of distinguishing its geographical variants. Methods and results: We assessed unrepaired isolated perimembranous ventricular defects from six historic archives, subcategorizing them using the ICD-11 coding system. We identified 365 defects, of which 94 (26%) were deemed to open centrally, 168 (46%) to open to the outlet, and 84 (23%) to the inlet of the right ventricle, with 19 (5%) being confluent. In all hearts, the unifying phenotypic feature was fibrous continuity between the leaflets of the mitral and tricuspid valves. This was often directly between the valves, but in all instances incorporated continuity through the atrioventricular portion of the membranous septum. In contrast, we observed fibrous continuity between the leaflets of the tricuspid and aortic valves in only 298 (82%) of the specimens. When found, discontinuity most commonly was seen in the outlet and central defects. There were no discrepancies between evaluators in distinguishing the borders, but there was occasional disagreement in determining the right ventricular geography of the defect. Conclusions: The unifying feature of perimembranous defects, rather than being aortic-to-tricuspid valvar fibrous continuity, is fibrous continuity between the leaflets of the atrioventricular valves. While right ventricular geography is important in classification, it is the borders which are more objectively defined. Keywords: Classification, Congenital heart disease, Nomenclature, Perimembranous ventricular septal defect, Ventricular septal defect
Introduction Excluding the aortic valve with two leaflets, isolated ventricular septal defects constitute the commonest form of congenital cardiac disease [1]. Despite their frequency, their description has become a tower of Babel. Various systems of nomenclature are in use, often within the same institution, with many of the systems being based on expert opinion rather than evidence. The International Society for Nomenclature of Paediatric and * Correspondence: [email protected] 1 The Heart Institute, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA 2 Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA Full list of author information is available at the end of the article
Congenital Heart Disease (ISNPCHD) was formed in 2000 with the goal of formulating a universal
