test 5 PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM Flashcards
What 5 things are associated with Pulmonary Atresia with Intact Ventricular Septum
- ASD
- Atretic Pulmonary Valve
- PDA (very important for survival)
- Hypoplastic right ventricle
- Hypoplastic tricuspid valve
- NO VSD
- lack of valve/small valve
What is Pulmonary Atresia with intact ventricular septum (PA w/IVS)
Complete atresia of pulmonary valve
Pulmonary valve fails to form late in development
Right ventricle and tricuspid valve Hypoplastic
But RV may be large enough for bi-ventricular correction
PA is normal size
Large ASD will decompress RA
Severe hypoplasia of RV results in creation of Coronary Artery Sinusoids which can be catastrophic
Connections between the RV and coronaries
Coronaries may depend on these connections
Pulmonary Atresia with intact ventricular septum (PA w/IVS) pathophys
Pulmonary Blood flow entirely dependent on PDA
Requires PGE-1 infusion after birth
R L shunting via atria
Coronary perfusion may be dependent on increased driving forces of obstructed RV (RV increased resistance is good)
Decompressing RV = Ischemia
Pulmonary Atresia with Intact Ventricular Septum Treatment
PGE-1 to maintain ductus arteriosus patency
RV dependent sinusoids and/or RV too hypoplastic
Balloon atrial septostomy to decompress the RA
Blalock-Taussig shunt as neonate with Fontan completion later
NO RV dependent Sinusoids and RV large enough
Open the atretic Pulmonary valve via transcatheter or surgical valvotomy
Systemic to PA shunt or PDA stent
Need shunt b/c RV is poorly compliant and hypertrophied
Poor RV output
Bi-directional Glenn would allow RV to handle IVC flow and SVC flow routed directly to the right pulmonary artery
Pulmonary Atresia with intact ventricular septum Post operative course
Prone to hemodynamic instability
Possibly delay chest closure
Length of Stay: 1-2 weeks
