Aortopulmonary septal defect

From Wikipedia, the free encyclopedia
Jump to: navigation, search
Aortopulmonary septal defect
Classification and external resources
Specialty medical genetics
ICD-10 Q20.0-Q20.3, Q21.4
ICD-9-CM 745.0-745.1
MeSH D001028

Aortopulmonary septal defect is a rare congenital heart disorder accounting for only 0.1-0.3% of congenital heart defects worldwide.[1] It is characterized by a communication between the aortic and pulmonary arteries, with preservation of two normal semilunar valves. It is the result of an incomplete separation of the aorticopulmonary trunk that normally occurs in early fetal development with formation of the spiral septum.[2] Aortopulmonary septal defects occur in isolation in about half of cases, the remainder are associated with more complex heart abnormalities.[3]

Causes[edit]

Pathophysiology[edit]

Diagnosis[edit]

Subtypes[edit]

There are numerous types, differentiated by the extent of the defect.[3] These types are:

  • Type I: simple defects leading to communication between the ascending aorta and pulmonic trunk
  • Type II: defects that extend to the origin of the right pulmonary artery
  • Type III: anomalous origin of the right pulmonary artery from the ascending aorta

It is also classified as simple or complex. Simple defects are those that do not require surgical repair, occur with no other defects, or those that require minor stright-forward repair (ductus arteriosus, atrial septal defect). Complex defects are those that occur with other anatomical anomalies or require non-standard repair.

Management[edit]

References[edit]

  1. ^ Love, Barry (05 02 2015). "Aortopulmonary Septal Defect". Medscape.  Check date values in: |date= (help)
  2. ^ Burakovsky, V. I., Falkovsky, G. E., & Ivanitsky, A. V. (1984). Surgical repair of truncus arteriosus. Pediatric cardiology, 5(2), 111-114.
  3. ^ a b McElhinney DB, Reddy VM, Tworetzky W, Silverman NH, Hanley FL (January 1998). "Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants <6 months of age". Am. J. Cardiol. 81 (2): 195–201. PMID 9591904. doi:10.1016/S0002-9149(97)00881-3.