test 5 PULMONARY ATRESIA WITH VSD

Question 1

Pulmonary Atresia – with VSD

Answer 1

 Failure of the development of the pulmonary valve
 Underdeveloped RV outflow tract and main PA
 Branch PAs may be confluent and fed by ductus or discontinuous (might not make it to the lungs) and hypoplastic.
 Discontinous – Pulmonary blood flow provided via Aortopulmonary Collaterals
 Normal development of the RV
 Large VSD
 May have an ASD
 Wide variations

Question 2

Pulmonary Atresia – With VSD pathophys

Answer 2

 Complete intracardiac mixing
- Right to left shunt because we are trying to pump the blood out -> has nowhere to go but the LV
 Systemic desaturation/ cyanosis
 Aortopulmonary collaterals
 Progressives stenosis
 Hypoxemia
 “True pulmonary arteries” are hypoplastic

Question 3

If we have confluent branch PAs which are fed by the ductus, then we can do

Answer 3

 Complete surgical repair (create new pulmonary artery)
 Placement of RV to PA conduit (Rastelli Procedure)
 Close VSD

Question 4

If we have Hypoplastic branch PAs with aortopulmonary vessels, then we can

Answer 4

 Surgical approach is varied and patient specific
 Unifocalization of Aortopulmonary (A-P) collaterals
 RVOT reconstruction
 Staged or do it all together and incorporate AP collateral unifocalization into the RVOT conduit
 Eventual closure of the VSD after RVOT reconstruction/unifocalization
 Ensure pulmonary flow adequate