The PVS Network Registry Quick Facts

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  • Data is entered into a REDCap database , which uses a secure server
  • All the data is analyzed at the Hospital for Sick Children in Toronto, Canada
  • ​C​urrently there are over 18 institutions across North America contributing data
  • Funding is supplied by a generous donations by individuals invested in improving knowledge about pulmonary vein stenosis and grants from the Hospital for Sick Children.​​ 

PVS Network Retrospective Study 

  • To date, we have approximately 700 children enrolled in the retrospective database ( updated 2021)

  • This cohort will provide insights into clinical outcomes  and disease surveillance

  • The retrospective registry will also serve as a contemporary control cohort for the prospective studies

PVS Network Prospective Study 

  • This study is currently open and recruiting patients 

  • patients can enroll in  additional substudies investigating genetic modifiers and biomarkers in PVS​

Genetics Study

Biomarker Study
  • The etiology or cause of PVS is unknown. 
  • Whole genome sequencing ( Trios) to help understand the genetic determinants of PVS.​
  • Identification of de novo variants or common risk variants will provide insights into disease etiology, aid in risk stratification and lead to discovery of novel therapeutic adjuncts.  
 
  • Currently no biomarkers exist for PVS
  • Study will investigate  known pulmonary hypertensive biomarkers and novel biomarkers in PVS patients
  • Identification of biomarkers will help clinicians detect disease progression and potentially provide insights around prognosis