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A Rare Variation of the Heterotaxy Syndrome [PDF]
Case Reports in Medicine, 2012 Heterotaxy syndrome is a rare, complex, and confusing type of the situs anomalies. It is not possible to estimate the degree of lateralization, isomerism, and rotational variation in these types of cases.
Alper Dilli +4 more
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Korean Circulation Journal, 2011 Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. This broad term includes patients with a wide variety of very complex cardiac lesions. Patients with heterotaxy can be stratified into the subsets of asplenia syndrome and polysplenia syndrome, or ...
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, 2018 Mutation of ZIC3 causes X-linked heterotaxy, a syndrome in which the laterality of internal organs is disrupted. Analysis of model organisms and gene expression during early development suggests ZIC3-related heterotaxy occurs due to defects at the earliest stage of left-right axis formation.
Bellchambers, Helen M. +1 more
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International Journal of Cardiology Congenital Heart Disease, 2023 Background: Heterotaxy syndromes encompass left and right atrial isomerism (LAI and RAI respectively) and are associated with variable cardiac and non-cardiac anomalies which greatly influence outcomes.
Bradley MacDonald +4 more
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Heterotaxy syndrome: This is the left, right?
South African Journal of Radiology, 2015 Heterotaxy syndrome is a rare and complex disorder of the chest and abdominal organ arrangements, and presents a diagnostic challenge to the radiologist.
Lili Huang +4 more
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Heterotaxy Syndrome with Polysplenia, Fused Adrenal Glands, and Diabetes Mellitus
Clinical Medicine Insights: Cardiology, 2022 Heterotaxy syndrome is a rare congenital heart disease with a disarrangement of the heart and abdominal organs. We present a young African female with features of heart failure, diffuse irregular cardiac murmurs, and palpable, tender epigastric mass.
Abid M Sadiq, Adnan M Sadiq
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, 2019 Abstract This chapter reviews background information about the incidence, risk factors, genetics, family history, recurrence risk, and epidemiology of heterotaxy and disorders of situs, including polysplenia and asplenia. The relationship between heterotaxy and dysfunction of non-motile primary cilia is explained.
Robin D. Clark, Cynthia J. Curry
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Annals of Pediatric Cardiology, 2022 Pulmonary arteriovenous malformations after the Kawashima procedure causing severe hypoxemia are treated by Fontan surgery that redirects hepatic venous blood to the pulmonary circulation.
Kothandam Sivakumar
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Frontiers in Pediatrics, 2022 ObjectiveCardiac and extra-cardiac anomalies in 46 pre-natally diagnosed cases of heterotaxy were compared to post-natal anatomical patterns in order to reveal discordant findings.
Elisabeth Seidl-Mlczoch +9 more
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Journal of Marine Medical Society, 2022 Heterotaxy syndrome is an uncommon disease, with an incidence ranging from 1 in 6000 to 1 in 20,000 live births.[1] This syndrome is characterized by major cardiac vascular malformations, congenital asplenia, polysplenia, and abnormal arrangement of the chest and abdominal organs.
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