Aortic pseudocoarctation associated with polysplenia/heterotaxy syndrome.
نویسندگان
چکیده
Polysplenia/heterotaxy syndrome is a rare congenital disorder associated with a wide spectrum of anomalies in various organ systems. Although anomalies of the cardiovascular system are common in this syndrome, the authors report a rare case of polysplenia syndrome associated with aortic pseudocoarctation, which to our knowledge has never been reported.
منابع مشابه
Dorsal pancreas agenesis and polysplenia/heterotaxy syndrome: a novel association with aortic coarctation and a review of the literature.
CONTEXT Agenesis of the dorsal pancreas is very rare and may be associated with other congenital disease states. It has a rare association with polysplenia/heterotaxy syndrome. Most commonly, these states occur due to errors in development of the asymmetric organs and may be associated with benign to severe congenital cardiac malformations. CASE REPORT We report a case of a 25-year-old male w...
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In 25% of cases of biliary atresia associated malformations are present; polysplenia constitute 10-50% of these associated anomalies. Biliary atresia found associated with polysplenia syndrome, heterotaxy, and reverse rotation of intestine, in isolation or in various combinations; however its association with polysplenia syndrome, situs inversus abdominus, and reverse rotation of gut in the sam...
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The pattern of anatomical organization of the thoraco-abdominal visceral and vascular structures which is not the expected normal arrangement, is called as situs ambiguous or heterotaxy syndrome. Patients with heterotaxy syndrome exhibit a wide spectrum of anatomical variations involving thoraco-abdominal structures. We present here an incidental finding of heterotaxy syndrome associated with u...
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BACKGROUND Laterality disorders of the abdominal organs include situs inversus totalis that mirrors the arrangements of all internal organs and heterotaxy syndrome (situs ambiguus) in which the thoracic or abdominal organs are abnormally arranged. Heterotaxy is often accompanied by multiple congenital malformations, and it generally comprises asplenia and polysplenia syndromes. To our knowledge...
متن کاملHeterotaxy Syndrome
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 the subsets of heterotaxy with isomerism of...
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ورودعنوان ژورنال:
- Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
دوره 34 1 شماره
صفحات -
تاریخ انتشار 2015