PV atresia

Question 1

PA atresia def

Answer 1

Imperforated pulmonary valve obstructing the flow to pulmonary circulation

Question 2

Types of PA atresia

Answer 2

Intact IVS
Concurrent VSD

Question 3

PA w/ intact IVS: features

Answer 3

o Usually normal atrial, AV and arterial connections
o 3 fused, well formed leaflets
o Valvular atresia in 75% of cases, muscular in 25%

Question 4

PA w/ intact IVS: pathophys

Answer 4

 Restrictive ASD is the only exit from RA
 PDA must be present to provide pulmonary blood flow

Question 5

PA w/ intact IVS: mx intervention

Answer 5

 Maintain DA patency: prostaglandins

Question 6

PA w/ intact IVS: sx intervention

Answer 6

 Early correction
* Balloon valvuloplasty
* Ductal stent placement in PDA
* Balloon atrial septostomy to open FO
* Systemic to pulmonary artery shunt (Blalock)
 Later procedures
* Biventricular repair ideally
* Bidirectionnal Glenn procedure (CrVC → PA)
* Fontan procedure

Question 7

PA atresia w/ VSD

Answer 7

o Most severe form of Tetralogy of Fallot

Question 8

PA atresia w/ VSD: mx management

Answer 8

phlebotomy

Question 9

PA atresia w/ VSD: sx management

Answer 9

 Palliative: if PA anatomy precludes repair
* Systemic to PA shunt → ↑ pulmonary flow
 Complete repair: PA size at least >50% its normal size
* RVOT reconstruction → connect RV to PA using conduit
* Closure of septal defects