[Table/Fig-8a]:

Pulmonary atresia PA-IVS.

Subtypes	PA-IVS (Pulmonary atresia with intact ventricular septum)		PA-VSD (Pulmonary atresia with VSD)
PA-IVS (Hypoplastic right heart syndrome) Characterized by pulmonary atresia, hypoplastic RV and hypoplasia of tricuspid valve annulus
Incidence [13]	7.1-8.1 per 100,000 live births
Association	Coronary artery anomalies e.g. RV dependent coronary circulation (coronary circulation perfused entirely by desaturated RV blood with proximal coronary arteries), coronary ostial atresia ASD/PFO PDA.
Syndromes	Di George syndrome
Imaging features Plain	Normal or mild cardiomegaly	Pulmonary oligemia	Hyper translucent lung fields
Imaging features specific	Echo thick, immobile atretic pulmonary valve; smallish hypertrophied RV; small tricuspid valve; ASD and PDA Catheter angiography for coronary anomalies and hemodynamic assessment MRI depicts all findings including tricuspid valve and RV size CT preferred for coronary artery evaluation Imaging is useful in classifying pulmonary atresia based on presence or absence of 3 portions (inlet/trabecular/infundibular) of RV into tripartite, bipartite and monopartite types [13].
Management [13]	Medical: Start PGE1 to maintain ductal patency Surgical: depends on RV size and coronary artery anomalies Two-ventricular repair adequate RV size and RVOT necessary One and one-half ventricular repair in borderline RV size One ventricular repair (Fontan operation) in monopartite RV and/or RV dependent coronary circulation.