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Right Atrium
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Congenital Defects Tutorial
Normal Cardiac Development Fetal Circulation Congenital Heart Defects Cardiac Transplantation
Septal Defects Right Heart Lesions Left Heart Lesions Anomalies of Arteries and Veins
Atrial Septal Defects Ventricular Septal Defects Atrioventricular Septal Defects Aortopulmonary Window Persistent Truncus Arteriosus

Aortopulmonary window

  • Aortopulmonary window defects result from a failure in development of the endocardial cushion tissues in the fetal common outflow tract. Without proper fusion between the opposing cushion tissues, there is incomplete septation between the ascending aorta and main pulmonary artery. Importantly, there are commonly two distinct semilunar valves present at the proximal end of both the aorta and pulmonary trunk.
  • Defects can be classified by their relative locations.
    • Type I defects are most common, and are located just above the semilunar valves.
    • Type II defects exist in the most distal aspect of the ascending aorta.
    • Type III defects encompass both the proximal and more distal areas of the ascending aortic and pulmonary vessels.
  • Aortopulmonary window defects result in a left-to-right shunt which typically leads to early pulmonary hypertension and congestive heart failure. Pulmonary vascular disease typically develops when the window is not surgically repaired.
  • Surgical closure and repairs of associated lesions, are recommended to be performed soon after diagnosis in infancy.