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Cardiol Young. 2017 Jul;27(5):825-836. doi: 10.1017/S1047951117000452.

The independence of the infundibular building blocks in the setting of double-outlet right ventricle.

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1Department of Pathology,InCor,Sao Paulo,Brazil.
2Division of Pediatric Cardiology,University of Florida,Gainesville,Florida,United States of America.
4Division of Biomedical Sciences,St George's University of London,United Kingdom.
5Institute of Genetic Medicine,Newcastle University,Newcastle upon-Tyne,United Kingdom.
6Division of Developmental Biology,Crick Institute for Medical Research,London,United Kingdom.


It has long been contentious as to whether the presence of bilateral infundibulums, or conuses, is a prerequisite for the diagnosis of double-outlet right ventricle. As the use of such a criterion would abrogate the so-called "morphological method", which correctly states that one variable entity should not be defined on the basis of another entity that is itself variable, it is now accepted that double outlet can exist in the setting of fibrous continuity between the leaflets of the atrioventricular and arterial valves. Although this debate has now been resolved, there are other contentious areas still requiring clarification in the setting of hearts unified because of the presence of this particular ventriculo-arterial connection - for example, it is questionable whether the channel between the ventricles should be described as a "ventricular septal defect", whereas it is equally arguable that the mere presence of fibrous continuity between the leaflets of the arterial valves does not necessarily place the channel in a doubly committed location. In this review, we describe a series of autopsied hearts in which the anatomical features serve to illuminate these various topics. We then discuss recent findings regarding cardiac development that point to the individuality of the building blocks of the ventricular outflow tracts, specifically the outlet septum, the inner heart curvature, or ventriculo-infundibular fold, and the septomarginal trabeculation, or septal band.


Outlet septum; inner heart curvature; septal band; septomarginal trabeculation; ventriculo-infundibular fold

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