Pulmonary Atresia With Ventricular Septal Defect Guidelines

Updated: Jan 10, 2022
  • Author: Edwin Rodriguez-Cruz, MD; Chief Editor: Stuart Berger, MD  more...
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Guidelines Summary

The European Society of Cardiology (ESC) updated their 2010 guidelines on the management of adult congenital heart disease (ACHD) in 2020. [2] Their recommendations regarding surgical intervention in and follow-up of patients with pulmonary atresia with ventricular septal defect (PA-VSD) are outlined below.

Surgical intervention

Note the following:

  • Patients with unrepaired PA-VSD, or those who underwent previous palliative procedures, who survive to adulthood: Modern surgical or interventional procedures may be beneficial.
  • Consider the following patients for surgery: Those with good-sized confluent PAs and those with large major aortopulmonary collateral arteries (MAPCAs) that are anatomically suitable unifocalization, who have not developed severe pulmonary vascular disease owing to protecting stenosis
  • Catheter interventions may include balloon dilation/stenting of collateral vessels for pulmonary blood flow enhancement; however, patients with severe hemoptysis may need to undergo coiling of the ruptured collateral vessels.
  • Patients with good-sized PAs absent a pulmonary trunk: Repair with a right ventricle (RV) to pulmonary artery (RV–PA) conduit.
  • Patients with confluent but hypoplastic PAs: An arterial shunt or reconstruction of the RV outflow tract (OT) (without VSD closure) is usually needed. This may enhance PA growth and then be reviewed at a later stage for repair using a valved conduit.
  • Patients with nonconfluent PAs and adequate, but not excessive, pulmonary blood flow in infancy: These patients can survive into adulthood without surgery.


Periodic follow-up of patients with PA-VSD is recommended at least once every year at a specialized ACHD institution. Monitor closely and evaluate and review sooner in the setting of symptoms such as dyspnea, increasing cyanosis, change in shunt murmur, heart failure, or arrhythmias.

Consider patients with segmental pulmonary arterial hypertension (PAH) for targeted PAH therapy.