[Table/Fig-9]:

TOF with absent pulmonary valve.

Components [13]	TOF components + rudimentary nubbins of tissue or complete absence of pulmonary valvular tissue Absent pulmonary valves, severe PR aneurismal dilatation of MPA and branch pulmonary arteries airway compression. Rabinovitch et al described abnormal tufts of smaller pulmonary arteries that compress the intrapulmonary bronchi with reduction of the number of alveoli.
Prevalence	Seen in ~3% of patients with TOF.
Association	Frequent absence of ductus arteriosus, Increased nuchal fold thickness (NFT) in I trimester [14]
Syndromes	Chromosomal abnormalities of chromosomes 6 and 7; deletion of chromosome 22q11 and DiGeorge syndrome
Imaging features Plain	Cardiomegaly (moderate to marked)	Grossly dilated central PAs	Decreased peripheral pulmonary vasculature	Tracheal compression	Air trapping in lungs
Imaging features specific	Echo TOF findings+ dysplastic pulmonary annulus+ massive PR on doppler Fetal MRI PA size+ symmetry of aeration of lungs secondary to airway compression and over-inflation + lung volume estimation [15,16] Post-natal MRI PR quantification CT better evaluation of airway compression and secondary lung changes.
Management [7]	Complete primary repair= VSD closure + pulmonary homograft placement to replace dysplastic pulmonary valve and dilated PAs