ReviewJournal of cardiovascular development and disease2018
Is an Appreciation of Isomerism the Key to Unlocking the Mysteries of the Cardiac Findings in Heterotaxy?
Review in Journal of cardiovascular development and disease, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 32 citations in OpenAlex.
- Isomeric Bodily Situs in the Era of Universal Prenatal Detection: Highlighting Computed Tomographic Findings in Those with Complex Cardiovascular Malformations.Pediatric cardiology · 2026Article
- Effect of Aortic Arch Position on the Bronchial Length Ratio in Situs Determination.CJC pediatric and congenital heart disease · 2026Article
- Interrupted Inferior Caval Vein in the Era of High Prenatal Detection Highlighting Postnatal Computed Tomographic Findings in Those Without Complex Cardiac Malformations.Pediatric cardiology · 2026Article
- Isomerism of the right atrial appendages with bilateral eparterial bronchi: clinical challenges and surgical management guided by 3D cardiac computed tomography.Indian journal of thoracic and cardiovascular surgery · 2025Article
- Ventricular Topology in Congenital Heart Defects Associated with Heterotaxy: Can We Find Patterns Reflecting the Syndrome-Specific Tendency for Visceral Symmetry?Journal of cardiovascular development and disease · 2025Article
- Identification and Analysis of Atrial-Bronchial-Abdominal Disharmony in Patients with Isomeric Atrial Appendages.Pediatric cardiology · 2025Article
- Splenic Torsion in Heterotaxy Syndrome with Left Isomerism: A Case Report and Literature Review.Diagnostics (Basel, Switzerland) · 2022Article
- Discordant Post-natal Patterns in Fetuses With Heterotaxy Syndrome: A Retrospective Single-Centre Series on Outcome After Fetal Diagnosis.Frontiers in pediatrics · 2022Article
- Characterization of phenotypic spectrum of fetal heterotaxy syndrome by combining ultrasound and magnetic resonance imaging.Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology · 2021Article
- Congenitally Malformed Hearts: Aspects of Teaching and Research Involving Medical Students.Journal of cardiovascular development and disease · 2021Article
- Histopathological assessment of laterality defects in zebrafish development.Animal cells and systems · 2021Article
- Special Issue: Left-Right Asymmetry and Cardiac Morphogenesis.Journal of cardiovascular development and disease · 2018Article
- Asplenia in left isomerism.Annals of pediatric cardiologyArticle
- Atrioventricular Discordance with Double-Outlet Right Ventricle in Mirror Imagery and Levocardia: A Very Rare Case Report.Journal of cardiovascular echographyArticle
- Prenatal diagnosis of asplenia syndrome with sliding hiatus hernia in a fetus.Annals of pediatric cardiologyArticle
- Is transposition a defect of laterality?Annals of pediatric cardiologyArticle
- Transposition of the great arteries: A laterality defect in the group of heterotaxy syndromes or an outflow tract malformation?Annals of pediatric cardiologyArticle
Corrections and comments
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Authors and funding
3 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pediatric cardiologists treating patients with severe congenital cardiac defects define "visceral heterotaxy" on the basis of isomerism of the atrial appendages. The isomeric features represent an obvious manifestation of disruption of left-right asymmetry during embryonic development. Thus, there are two subsets of individuals within the overall syndrome, with features of either right or left isomerism. Within the heart, it is only the atrial appendages that are truly isomeric. The remainder of the cardiac components shows variable morphology, as does the arrangement of the remaining body organs. Order is provided in this potentially chaotic arrangement simply by describing the specific features of each of the systems. These features as defined by clinicians, however, seem less well recognized by those investigating the developmental origins of the disruption of symmetry. Developmental biologists place much greater emphasis on ventricular looping. Although the direction of the loop can certainly be interpreted as representing an example of asymmetry, it is not comparable to the isomeric features that underscore the clinical syndromes. This is because, thus far, there is no evidence of ventricular isomerism, with the ventricles distinguished one from the other on the basis of their disparate anatomical features. In similar fashion, some consider transposition to represent abnormal lateralization, but again, clinical diagnosis depends on recognition of the lateralized features. In this review, therefore, we discuss the key questions that currently underscore the mismatch in the approaches to "lateralization" as taken by clinicians and developmental biologists.
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