Reference Work Entry

Encyclopedia of Molecular Mechanisms of Disease

pp 128-129

Aortopulmonary Septal Defects

  • Lucia K. MaAffiliated withRoyal College of Surgeons
  • , Patrick T. S. MaAffiliated withHeart Health Institute and Department of Cardiac Science, The University of Calgary
  • , Alexander K. C. LeungAffiliated withDepartment of Pediatrics, Alberta Children's Hospital, The University of Calgary


Aortopulmonary window; Aortopulmonary fenestration or fistula; Aortic septal defect; Partial persistent truncus arteriosus; APSD

Definition and Characteristics

Aortopulmonary septal defect (APSD) is a rare congenital cardiac abnormality defined as a communication between adjacent portions of the ascending aorta (AAo) and the pulmonary artery (PA), with the presence of separate semilunar valves. APSD may exist as an isolated lesion, the rest (47−77%) are found in conjunction with other congenital heart disease, including patent ductus arteriosus, ventricular septal defects (20%) tetralogy of Fallot (6%), right aortic arch (5–20%), transposition of the great arteries (10%), interrupted aortic arch (8%), coarctation of the aorta (13–20%) and coronary artery anomalies (23%) [1]. APSD is subdivided into three subtypes. Type I occurs between the origin of the main PA and the posteromedial wall of AAo immediately above the sinus of Valsalva. Type I ...

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