Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases—Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease

Abstract

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.

Development of the human heart

“…The ventricular foramen is divided in a left and right component by the two major AV cushions and the septal OFT ridge, becoming the membranous septum in the adult human from the second half of the sixth week of development (CS19) onward. One can now also understand why this septum has an interventricular and an atrioventricular portion, and why perimembranous or inlet VSDs are present as a spectrum (Lopez et al, ).…”

Spatio‐temporal image correlation rendering mode visualizes the specific location and surrounding structure of ventricular septal defect

“…Meanwhile, the specific location and adjacent structure of VSD were observed and recorded by STIC rendering mode. In the present study, the classification scheme advocated by the International Society for Nomenclature of Pediatric and Congenital Heart Disease was utilized to describe and classify the lesion (Lopez et al, ). According to the position of defects, the VSDs are divided into central perimembranous, inlet, outlet, and trabecular muscular defects ( Fig.…”

“…It is the most common congenital cardiac malformations, accounting for ~40% of all congenital cardiac anomalies (Penny and Vick, ). Based on the location of the VSD on the right ventricular surface of the interventricular septum, VSDs can be classified by several phenotypic variants, such as central perimembranous, inlet, outlet, and trabecular muscular defects (Lopez et al, ). Currently, the location and size of the defects have been implicated in the outcome of neonates with VSD (Huang et al, ).…”

Ендоваскулярне Закриття Дефектів Міжшлуночкової Перегородки

“…Згідно з найновішою класифікацією [2,3], що використовується в Міжнародному класифікаторі хвороб (МКХ-11), виділяють чотири типи ДМШП: центральний перимембранозний, приточний (атріовентрикулярний канал), трабекулярний м'язовий та інфундибулярний (вихідного тракту). Найбільш поширеними є перимембранозні дефекти, їх частка сягає до 70-80 %; на другому місці -м'язові дефекти [2,4,5].…”