Classifying Heterotaxy Syndrome

Classifying Heterotaxy Syndrome Editorial Time for a New Approach Stephen P. Sanders, MD; Tal Geva, MD eterotaxy syndrome is a complex set of abnormalities expected pattern of visceral and cardiac anatomy, irrespec- Hrelated to abnormal left–right axis patterning. Although tive of classifying patients based on the status of the spleen or rare (occurring in just over 1 in 10 000 live births ), congeni- atrial appendages. tal heart defects associated with heterotaxy syndrome carry a Substantial progress has been made in recent decades 2,3 disproportionate burden of morbidity and mortality. Despite in understanding how lateralizing information is transmit- numerous investigations on the genetic underpinning, epi- ted to the lateral plate mesoderm, the 2-cilia model being most widely accepted. Around the end of gastrulation, motile demiology, anatomic pathology, diagnostic imaging, and management, agreement regarding the best method to clas- cilia in the pit of the node at the rostral end of the primitive streak normally establish flow of extracellular fluid across sify patients with heterotaxy continues to elude our field. Specifically, a growing body of experience has noted that the the node from right to left. This stimulates receptor cilia at the leftward edge of the node, initiating a transcription cas- 2 most http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Circulation: Cardiovascular Imaging Wolters Kluwer Health

Classifying Heterotaxy Syndrome

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Publisher
Wolters Kluwer
Copyright
© 2018 American Heart Association, Inc.
ISSN
1941-9651
eISSN
1942-0080
D.O.I.
10.1161/CIRCIMAGING.118.007490
Publisher site
See Article on Publisher Site

Abstract

Editorial Time for a New Approach Stephen P. Sanders, MD; Tal Geva, MD eterotaxy syndrome is a complex set of abnormalities expected pattern of visceral and cardiac anatomy, irrespec- Hrelated to abnormal left–right axis patterning. Although tive of classifying patients based on the status of the spleen or rare (occurring in just over 1 in 10 000 live births ), congeni- atrial appendages. tal heart defects associated with heterotaxy syndrome carry a Substantial progress has been made in recent decades 2,3 disproportionate burden of morbidity and mortality. Despite in understanding how lateralizing information is transmit- numerous investigations on the genetic underpinning, epi- ted to the lateral plate mesoderm, the 2-cilia model being most widely accepted. Around the end of gastrulation, motile demiology, anatomic pathology, diagnostic imaging, and management, agreement regarding the best method to clas- cilia in the pit of the node at the rostral end of the primitive streak normally establish flow of extracellular fluid across sify patients with heterotaxy continues to elude our field. Specifically, a growing body of experience has noted that the the node from right to left. This stimulates receptor cilia at the leftward edge of the node, initiating a transcription cas- 2 most

Journal

Circulation: Cardiovascular ImagingWolters Kluwer Health

Published: Feb 1, 2018

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