Pathology
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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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+ Introduction
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+ Atlas of fetal pathology
Transposition of the great arteries
Macroscopic appearance
  • incorrect connection of ventricles and great vessels: the aorta arises from the morphologic right ventricle, the pulmonary artery from the morphologic left ventricle
  • the great arteries arise in an unusual position from the base of the heart, the aorta in front of the pulmonary artery, aorta slightly to the right
  • ventricular septal defect is found in 40% of affected hearts
Clinical signs
  • the most common congenital cyanotic heart defect in the newborns
  • the infants present with severe and progressive cyanosis within hours after birth with closure of the ductus
  • the systemic and pulmonary circulations are separated
  • oxygenation of systemic blood is totally dependent on mixing of pulmonary and systemic blood flow at arterial level (patent ductus arteriosus) and the cardiac level (foramen ovale, atrial septal defect, ventricular septal defect)
  • palliation: prostaglandin E infusion to maintain patency of the ductus, balloon atrial septostomy is performed as soon as possible after delivery
  • corrective surgary: arterial switch procedure (anatomical correction) is strongly preferred. The aorta and pulmonary artery are transected and removed in their normal position. Coronary arteries must be transferred as well. After the surgery the left ventricle works as the systemic ventricle.