Truncus arteriosus

Case contributed by Vincent Tatco , 6 Nov 2015
Diagnosis certain
Changed by Vincent Tatco, 12 Mar 2017

Updates to Case Attributes

Age changed from 3 to 3 years.
Body was changed:

Truncus arteriosus is an uncommon congenital cardiac abnormality that occurs due to the failure of conotruncal septation during development of the fetus. It is characterized by a single arterial trunk that originates from the heart and supplies the systemic, pulmonary, and coronary circulation1.

The original and more popular classification of truncus arteriosus was devised by Collett and Edwards in 1949, which divides this anomaly into four types2:

  • type I: Commoncommon pulmonary artery arising from truncus – 80%
  • type II: Rightright and left pulmonary arteryarteries arise separately from the posterior part of truncus
  • type III: Separateseparate origins of the pulmonary arteries from the lateral aspect of the truncus
  • type IV: Neitherneither pulmonary arterial branch arising from the common trunk (pseudotruncus), is now recognized to be a form of pulmonary atresia with VSD rather than truncus arteriosus.

In 1965, Van Praagh modified the classification system which also includes four primary types3:

  • type A1: Identicalidentical to the Type I of Collett and Edwards
  • type A2: Separateseparate origins of the branch pulmonary arteries from the left and right lateral aspects of the common trunk
  • type A3: Originorigin of one branch pulmonary artery (usually the right) from the common trunk, with other lung supplied either by collaterals or a pulmonary artery arising from the aortic arch
  • type A4: Coexistencecoexistence of an interrupted aortic arch.
  • -<li>type I: Common pulmonary artery arising from truncus – 80%</li>
  • -<li>type II: Right and left pulmonary artery arise separately from the posterior part of truncus</li>
  • -<li>type III: Separate origins of the pulmonary arteries from the lateral aspect of the truncus</li>
  • -<li>type IV: Neither pulmonary arterial branch arising from the common trunk (pseudotruncus), is now recognized to be a form of pulmonary atresia with VSD rather than truncus arteriosus.</li>
  • +<li>type I: common pulmonary artery arising from truncus – 80%</li>
  • +<li>type II: right and left pulmonary arteries arise separately from the posterior part of truncus</li>
  • +<li>type III: separate origins of the pulmonary arteries from the lateral aspect of the truncus</li>
  • +<li>type IV: neither pulmonary arterial branch arising from the common trunk (pseudotruncus), is now recognized to be a form of pulmonary atresia with VSD rather than truncus arteriosus.</li>
  • -<li>type A1: Identical to the Type I of Collett and Edwards</li>
  • -<li>type A2: Separate origins of the branch pulmonary arteries from the left and right lateral aspects of the common trunk</li>
  • -<li>type A3: Origin of one branch pulmonary artery (usually the right) from the common trunk, with other lung supplied either by collaterals or a pulmonary artery arising from the aortic arch</li>
  • -<li>type A4: Coexistence of an interrupted aortic arch.</li>
  • +<li>type A1: identical to the Type I of Collett and Edwards</li>
  • +<li>type A2: separate origins of the branch pulmonary arteries from the left and right lateral aspects of the common trunk</li>
  • +<li>type A3: origin of one branch pulmonary artery (usually the right) from the common trunk, with other lung supplied either by collaterals or a pulmonary artery arising from the aortic arch</li>
  • +<li>type A4: coexistence of an interrupted aortic arch.</li>

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