Measurement of Angiogenic Related Growth Factors in Urine and Blood From Patients With Pulmonary Vein Stenosis
Measurement of Angiogenic Related Growth Factors in Urine and Blood From Patients With Pulmonary Vein Stenosis
Prospective, single center, descriptive study in 14 infants/children with multivessel
 pulmonary vein stenosis stratified for patients with or without underlying cardiac disease.

 Group 1: Patients without additional structural heart disease and multivessel pulmonary vein
 stenosis.

 Group 2: Patients with additional structural heart disease and multivessel pulmonary vein
 stenosis.
Patients identified at the time of presentation to their cardiologist will be asked to
 provide a urine sample for analysis. If blood is being drawn for other clinical tests, an
 extra vial of blood will be collected for measurement of bFGF, VEGF, angiopoietin 1&2 and
 ephrin. At the time of blood draws, approximately 4 cc's of heparin blood will be obtained
 and processed as detailed below. Blood and/or urine samples will be coordinated through the
 data manager and will be performed only when blood is being obtained for other reasons.
 Initially, blood samples will be sent to Lab Control, spun down, and the plasma isolated.
 Plasma will be coded, then frozen at -200C in the research lab. Urine samples will be
 aliquoted into vials and stored at -20 until further analysis.

 All patient information will be kept confidential. Individual results will not be provided to
 patients. Identifiers to patient information will be maintained. No identifying patient
 information will be used in publications that might result from these studies. Information to
 be collected by the data manager will include age, sex, date of diagnosis and interventions.

 The specific aim is to assess angiogenic growth factor concentrations in urine and blood from
 patients with both congenital and acquired multivessel pulmonary vein stenosis.
Inclusion Criteria:

 -  Diagnosis can be based on clinical and radiographic grounds or at the time of biopsy
 or prior surgical procedures. The diagnosis must be consistent with multivessel
 pulmonary stenosis.

 -  There must be evidence of severe pulmonary vein stenosis in at least two pulmonary
 veins.

 -  Must give written informed consent according to institutional guidelines

 Exclusion Criteria:

 -  Mechanism of pulmonary venous obstruction due to obvious anatomic cause, such as
 extrinsic compression