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Cardiovascular Abnormalities Associated with Heterotaxy Syndrome: A Case Series
Heterotaxy syndromes result from the failure of normal lateralization, resulting in the abnormal arrangement of cardiothoracic and abdominal viscera. These are associated with a disproportionate morbidity and mortality rate, mandating early diagnosis ...
Shraddha Joshi +3 more
doaj +1 more source
Heterotaxy polysplenia syndrome in an adult female with complete endocardial cushion defect
Heterotaxy syndrome is a rare condition characterized by the abnormal arrangement of thoracoabdominal organs across the left-right axis of the body.It is generally classified as right and left atrial isomerism or asplenia and polysplenia syndrome, even ...
Habib Ahmad Esmat +2 more
doaj +1 more source
Heterotaxy Syndrome: Discordant Growth [PDF]
Heterotaxy syndrome implies a discordance between placement of thoracic organs with respect to abdominal organs. A large number of these have associated congenital heart defects. This syndrome is unique as every patient is different and can have any permutation and combination of symptoms.
Yadav, Pratiksha +3 more
openaire +2 more sources
Heterotaxy syndrome is associated with asplenia/polysplenia and complex congenital heart disease. Facial palsy in heterotaxy is very rare. The management is still challenging with a poor prognosis. Proper counseling to the family about the disease course,
Sanjeev Kharel +6 more
doaj +1 more source
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
doaj +1 more source
Heterotaxy – Res ipsos loquitur [PDF]
AbstractThe essence of so-called heterotaxy is the potential disharmony between the arrangement of the bronchuses, abdominal organs, and the atrial appendages. Accurate description of the heart, however, can only be provided by specific description of these features, all of which are readily discernible in the clinical setting.
Robert H. Anderson +4 more
openaire +3 more sources
Heterotaxy syndrome: This is the left, right?
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
doaj +1 more source
Atrioventricular canal defect and genetic syndromes: the unifying role of sonic hedgehog [PDF]
The atrioventricular canal defect (AVCD) is a congenital heart defect (CHD) frequently associated with extracardiac anomalies (75%). Previous observations from a personal series of patients with AVCD and "polydactyly syndromes" showed that the distinct ...
Baban, A +9 more
core +1 more source
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
openaire +2 more sources
NPHP4 Variants Are Associated With Pleiotropic Heart Malformations [PDF]
Rationale: Congenital heart malformations are a major cause of morbidity and mortality, especially in young children. Failure to establish normal left-right (L-R) asymmetry often results in cardiovascular malformations and other laterality defects of ...
Amack, J.D. +28 more
core +9 more sources

