Heterotaxy Syndrome

  • Etiology: congenital
  • Asplenia:
    Imaging: bilateral right sidedness with bilateral eparterial bronchus (bronchus is posterio-superior to pulmonary artery) and bilateral trilobed lungs
    Clinical: severe cyanosis, severe congenital heart disease, susceptible to infection
  • Polysplenia:
    Imaging: bilateral left sidedness with bilateral hyparterial bronchus (bronchus is anterio-inferior to pulmonary artery) and bilateral bilobed lungs
    Clinical: less congenital heart disease, presents later, associated with biliary atresia

Cases of Heterotaxy Syndrome