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Lateral Heterotaxy Syndrome in a Newborn Caucasian Male

Heterotaxy syndrome is a varied spectrum of rearrangements of thoracic and abdominal organs that present many unique complications. Among all congenital deformities, heterotaxy syndrome is rare although this is likely an underestimate without routine imaging due to the benign nature of some defects....

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Detalles Bibliográficos
Autores principales: Koenig, Zachary A, Verhoeven, Alex, Rosen, David, Petrone, Ashley B
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7704025/
https://www.ncbi.nlm.nih.gov/pubmed/33269136
http://dx.doi.org/10.7759/cureus.11205
Descripción
Sumario:Heterotaxy syndrome is a varied spectrum of rearrangements of thoracic and abdominal organs that present many unique complications. Among all congenital deformities, heterotaxy syndrome is rare although this is likely an underestimate without routine imaging due to the benign nature of some defects. Numerous genes have been identified that play a role in its pathogenesis, and it has been hypothesized that heterotaxy syndrome is a consequence of both genetic and environmental impacts on the body axis. This case report also demonstrates the fundamental role of cardiac catheterization and imaging in further specifying the subtype of heterotaxy. Furthermore, it highlights the inconsistency of laterality with functional asplenia, visceral situs ambiguus, double-outlet right ventricle, and a left-sided inferior vena cava apart from other anomalies in a newborn male.