Pilot Study Using Avastin and Gleevec to Treat the Progression of Intraluminal Pulmonary Vein Stenosis

• Status: Completed
• Trial end date: 2015-12-01
• Target enrollment: 0
• Participant gender: All

Summary

The objective of this study is to conduct a pilot study using biologic agents Avastin and Gleevec to treat progression of multivessel intraluminal pulmonary vein stenosis in children.

• Phase: Phase 1/Phase 2
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: Boston Children's Hospital; Kathy Jenkins
• Treatments: Bevacizumab; Imatinib Mesylate

Criteria

Eligibility Criteria: (Both groups)

• Evidence of intraluminal pulmonary vein stenosis in > 1 vessel

• Evidence of myofibroblast neo-proliferation, if biopsies were obtained

• Acceptable organ function includes:

Creatinine < 1.5 x normal for age. Bilirubin < 1.5 x normal for age. ALT < or = 5x normal ANC > or = 1,500/mm3, Hemoglobin > or = 10g/dl, Platelets > or = 100,000/mm3.

Group A Eligibility Criteria: (begin treatment with Gleevec® only)

• Significant concomitant congenital heart defect

• Disease severity for each vessel Category 5 or lower or Category 6 or 7 in no more than 1 vessel

Group B Eligibility Criteria: (begin treatment with Gleevec® and Avastin®)

• Primary PVS (i.e. without concomitant congenital heart defect or lung disease)

• Significant concomitant lung disease

• Patients with PVS and underlying CHD who have category 6 or 7 disease in at least 2 of their pulmonary veins even after surgical or cath-based interventions.

• Accepted organ function includes:

Urine protein < 1