[1] The first case of absent pulmonary valve syndrome was reported Crampton in 1830.

[3] The most typical form of APVS is a tetralogy of Fallot variant,[4] however, case studies have linked APVS to several different congenital cardiac syndromes, such as agenesis of ductus arteriosus,[5] persistent ductus arteriosus,[6] atrioventricular septal defect,[7] pulmonary branching abnormalities,[8][9] transposition of the great arteries,[10] and type B interrupted aortic arch.

In addition to heart issues, the majority of newborns experience respiratory distress-related problems.

Massive lobar emphysema may result from the compression of bronchi by dilated pulmonary arteries, which typically causes respiratory distress.

When the infundibulum in APVS is oriented horizontally and to the right, it causes the right pulmonary artery to dilate aneurysmally, further compressing the middle lobe bronchus.