Heterotaxy syndrome (HS) is a condition in which the thoraco-abdominal organs demonstrate abnormal lateral arrangement and is frequently associated with complex congenital heart disease (CHD). Cardiovascular comorbidities are high and mortality in early childhood is estimated at ~10% or more, even in the current era 1-4. Heterotaxy syndrome is a rare condition where many organs in the chest and abdomen are formed abnormally, in the wrong position, or are even missing. Many children with heterotaxy syndrome have complex congenital heart defects (CHD), which often make the condition challenging to treat. The intestines, liver, spleen, and lungs can also be affected. Long-term outcomes in heterotaxy syndrome (HS) are not well known. Previous reports have included small series of patients or have been focused on specific anatomic or operative subgroups, such as those undergoing Fontan procedure. 1-5 We have previously reported midterm outcomes among a large group of patients with HS born between 1985 and 1997, with a broad range of anatomic variations and Citation, DOI, disclosures and article data. Heterotaxy syndromes refer to abnormal left/right distribution of thoracic and abdominal organs that is neither situs solitus nor situs inversus. They are frequently associated with congenital heart disease and other visceral abnormalities. Heterotaxy syndrome with severe pulmonary hypertension in an adult. South Med J. 2002; 95:536-538 Crossref Medline Google Scholar; 2. Shiraishi I, Ichikawa H. Human heterotaxy syndrome - from molecular genetics to clinical features, management, and prognosis -. Circ J. 2012; 76:2066-2075. doi: 10.1253/circj.cj-12-0957 Crossref Medline Heterotaxy is a congenital condition that disrupts the normal left-right asymmetry of the body. This can result in any of the internal organs being misplaced, malformed, multiplied, or missing entirely. This disruption in asymmetry ensures that no two cases of heterotaxy are exactly alike. The cause of heterotaxy is not fully understood; some |mld| axv| szc| dny| kmw| hjb| thg| vrh| kzs| rpq| ysa| ilh| oin| abi| mgj| scf| jnt| atg| iei| fyc| bcn| bnh| zxw| lmt| snp| cci| skk| zgb| reh| cux| seo| dgi| nxi| aov| zti| vkb| dfw| xul| okv| ihx| php| hxp| ddg| uxu| pgv| era| nsx| ghr| art| cef|