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The problems that exist when considering the anatomic variability between the channels that permit interventricular
Frédérique Bailliard1, Diane E Spicer2, Timothy J Mohun3
11Bailliard Henry Pediatric Cardiology,Raleigh,North Carolina,United States of America.
Insights
Developing a universal coding system for congenital heart defects, specifically those allowing interventricular shunting, requires consensus on terminology and classification. This review proposes a method based on embryologic development for a widely accepted categorization.
Area of Science:
- Cardiology
- Developmental Biology
- Medical Informatics
Background:
- Existing coding systems for congenital heart malformations face challenges in achieving universal acceptance.
- Interventricular shunting lesions present significant classification difficulties due to semantic and anatomical disagreements.
- Discrepancies exist between Germanic (ventricular septal defects) and Romance (interventricular communications) terminology.
Purpose of the Study:
- To review the evolution of terminology for interventricular shunting.
- To propose a standardized categorization system for congenital heart malformations involving interventricular shunting.
- To address the need for consensus in classifying these complex cardiac defects.
Main Methods:
- Review of historical and theoretical approaches to naming interventricular shunting channels.
- Analysis of embryologic techniques in murine heart development.
- Correlation of murine cardiac development with human congenital heart lesion anatomy.
Main Results:
- Embryologic studies demonstrate that murine heart development mirrors the anatomical variations seen in human congenital heart lesions.
- Attention to both lesion location and border anatomy is crucial for understanding phenotypic variability.
- A proposed categorization framework integrates temporal development and precise definitions of ventricular septation.
Conclusions:
- A universally acceptable categorization system for congenital heart malformations with interventricular shunting is feasible.
- Utilizing embryologic principles and strict anatomical definitions can resolve current classification obstacles.
- This approach promises to unify the understanding and coding of these cardiac defects.
Abstract:
Although steps are being taken to produce a universally acceptable coding system for categorisation of the congenitally malformed hearts, obstacles remain in the search for consensus. One of the groups of lesions continuing to produce the greatest problems is those that permit interventricular shunting. The difficulties relate partly to the words used to describe the group itself, as those using Germanic languages describe the holes as ventricular septal defects, whereas those using Romance languages consider them to represent interventricular communications. The two terms, however, are not necessarily synonymous. Further disagreements relate to whether the lesions placed within the group should be sub-categorised on the basis of their geographical location within the ventricular mass, as opposed to the anatomic nature of their borders. In reality, attention to both the features is necessary if we are to recognise the full extent of phenotypic variability. In this review, we first review the evolution and theories of analysis naming the channels that permit interventricular shunting. We then demonstrate that embryologic techniques provide evidence that the changing morphology of the developing murine heart parallels the anatomy of the different lesions encountered in the congenitally malformed human heart. We suggest that, with attention paid to the temporal development of the normal murine heart, combined with a strict definition of the plane of separation between the right and left ventricular cavities, it will be feasible to produce a categorisation that is acceptable to all.
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