Absent pulmonary valve syndrome represents a rare defect, characterized by rudimental pulmonary valve leaflets. The presence of a nonfunctional valve can result in right ventricle dilation ( Figure 1 ) and reduced ventricular compliance. There is often a ventricular septal defect with aortic override, as seen in tetralogy of Fallot ( Figure 2 , Video 1 ) while ductus arteriosus is frequently absent ( Figure 3 ). Pulmonary valve stenosis may coexist ( Figure 4 ). The main sonographic feature is massive dilation of pulmonary branches ( Figure 5 , Video 2 ). The prognosis is poor, and in addition to cardiac complications, most infants develop respiratory distress secondary to compression of bronchi by dilated pulmonary arteries.
A 4-chamber view
A sonogram showing right ventricle dilation and left axis deviation.
LV , left ventricle; RV , right ventricle.
Piacentini. Features of absent pulmonary valve syndrome. Am J Obstet Gynecol 2022.
