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ENRight Atrial Isomerism (RAI)
Alias:
Ivemark Syndrome
Asplenia with Cardiovascular Anomalies
Rai
Right Isomerism
Isomerism of Right Atrial Appendage
Right Sided Atrial Isomerism
Asplenia Syndrome
Heterotaxy, Visceroatrial, Autosomal Recessive
Congenital Absence of Spleen
Bilateral Right-Sidedness
Splenic Agenesis Syndrome
Vah, Autosomal Recessive
Atrial Isomerism, Right
Polyasplenia
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Right Atrial Isomerism, also known as ivemark syndrome, is related to congenital heart defects, multiple types, 6 and heart septal defect. An important gene associated with Right Atrial Isomerism is GDF1 (Growth Differentiation Factor 1), and among its related pathways/superpathways are Nervous system development and Beta-2 adrenergic-dependent CFTR expression. Affiliated tissues include spleen and heart, and related phenotypes are asplenia and common atrium
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MALACARDS
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