Congenital Heart Defects - Pulmonary Atresia with Ventricular Septal Defect (PA-VSD)
 

What’s wrong?

  • Pulmonary atresia (absent pulmonary artery)
  • Blood can't flow from the RV into the PA and on to the lungs

Corrective Procedures?

  • Create a shunt between the (systemic) usually subclavian artery and the Pulmonary artery that may help increase blood flow to the lungs (Modified BT shunt)
  • Complete repair depends on;

    1. size of both ventricles
    2. presence and size of branch pulmonary arteries

  • If Right Ventricle is hypoplastic (small) one may have towards univentricular pathway

Surgical options include:

  • BT shunt soon after birth
  • If Ventricles are of good size → Biventricular repair (closure of VSD and RV-PA Conduit [tube with Valve inside])
  • If Right Ventricle is hypoplastic → Univentricular repair (Glenn or Fontan procedure)