Tetralogy of Fallot with absent pulmonary valve syndrome

**Definition:** A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which the ventriculo-arterial junction of the right ventricle with the pulmonary trunk features an atypical valve with absent or rudimentary leaflets (cusps) that do not coapt.

Additional information: in its usual form there is dilatation of the pulmonary trunk and central right and left pulmonary arteries, which when extreme, is associated with abnormal arborization of lobar and segmental pulmonary artery branches and with compression of the trachea and mainstem bronchi, often with tracheobronchomalacia. The physiologic consequence is usually a combination of variable degrees of both stenosis and regurgitation of the pulmonary valve.