Pulmonary vein stenosis

PDF download is not available for Arabic and Urdu languages at this time. Please use the browser print function instead

Pulmonary vein stenosis (PVS) is when there is a narrowing or blockage in the pulmonary veins. It is a rare disease and if it progresses, it can lead to pulmonary hypertension and right heart failure.

Key points

  • Each child is unique. The number of pulmonary veins affected and where the stenosis is located is not the same in every child.
  • There are two main categories: primary PVS and post-repair PVS. Primary PVS is when the child develops PVS without having any surgery on the pulmonary veins, while post-repair PVS develops after surgical repair of the pulmonary veins.
  • Progression of the disease can be clinically silent, meaning there are no symptoms.
  • PVS is a rare but very serious disease.
  • Children can have surgery or catheter-based interventions (balloon angioplasty or stents) to help relieve the narrowing and help blood flow better through the lungs.
Last updated: March 22nd 2018