test 5 PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM Flashcards

1
Q

What 5 things are associated with Pulmonary Atresia with Intact Ventricular Septum

A
  1. ASD
  2. Atretic Pulmonary Valve
  3. PDA (very important for survival)
  4. Hypoplastic right ventricle
  5. Hypoplastic tricuspid valve
    - NO VSD
    - lack of valve/small valve
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2
Q

What is Pulmonary Atresia with intact ventricular septum (PA w/IVS)

A

 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

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3
Q

Pulmonary Atresia with intact ventricular septum (PA w/IVS) pathophys

A

 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

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4
Q

Pulmonary Atresia with Intact Ventricular Septum Treatment

A

 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

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5
Q

Pulmonary Atresia with intact ventricular septum Post operative course

A

 Prone to hemodynamic instability
 Possibly delay chest closure
 Length of Stay: 1-2 weeks

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