Fetopathology and developmental pathology of the embryo and fetus: Transposition of the great arteries

+ Introduction

+ Support

+ Atlas of fetal pathology

+ Congenital malformations of individual organ systems

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.

Pictures

Transposition of the great arteries, view of the heart base, newborn: Transposition of the great arteries, Macro, autopsy (72094)

Transposition of the great arteries, aorta arises from the right ventricle, newborn (the same case): Transposition of the great arteries, Macro, autopsy (72095)