Pilot Trial: the Safety and Feasibility of Losartan for Pulmonary Vein Stenosis

Study Overview
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Brief Summary
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This pilot study evaluates the safety and feasibility of losartan in pediatric patients with pulmonary vein stenosis. It is an open label single arm interventional study. The patient population involves pediatric patients with stenosis in 2 or more pulmonary veins. Patients will be given losartan for 1 year. Outcomes include measures of safety/adverse events and progression of pulmonary vein stenosis.

Detailed Description
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This pilot study evaluates the safety and feasibility of losartan in pediatric patients with pulmonary vein stenosis. Large animal studies have demonstrated that losartan can ameliorate the progression of pulmonary vein stenosis in a surgical model of pulmonary vein stenosis. There is no safety data for losartan in children < 6months of age or in children with pulmonary vein stenosis. Therefore, this study will test the safety and feasibility of losartan in children in an open label single arm interventional study. Patient inclusion criteria includes stenosis involving 2 or more pulmonary veins. Patients who are consented into the trial with be on losartan therapy for one year. They will undergo study visits every 3 months. Patients will be screened for adverse events through biochemical tests, routine clinical tests and questionnaires. An exploratory efficacy analysis will compare progression of pulmonary vein stenosis in children enrolled in losartan to a contemporary cohort of children with pulmonary vein stenosis, who did not receive losartan.

Official Title
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Pilot Trial: the Safety and Feasibility of Losartan Therapy for Treatment of Pulmonary Vein Stenosis in Pediatric Patients

Conditions
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Pulmonary Vein Stenosis, Children

Intervention / Treatment
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* Drug: Losartan


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Stenosis involving 2 or more pulmonary veins Consent of parent/legal guardian or child(when appropriate) Exclusion Criteria: History of angioedema or allergic reaction to angiotensin converting enzyme inhibitors corrected gestational age of less than 40 weeks severe renal dysfunction pregnancy

Ages Eligible for Study
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Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Losartan<br>Losartan will be given to children with stenosis in greater or equal to 2 pulmonary veins. Maintenance daily dosing is 1mg/kg/day using suspension or tablet formulation of losartan. Losartan will be given for 1 year. | Drug: Losartan<br>* Losartan is given for 12 months<br>* Other names: Cozaar;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Safety:Number of participants with adverse events | adverse events include hypotension, hyperkalemia, renal dysfunction | 12 months | 
| Feasibility:Number of participants eligible and recruited | includes patient recruitment and compliance with protocol | 12 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Progression of pulmonary vein stenosis: survival | Progression of pulmonary vein stenosis measures include survival, morbidity and evidence of progression on clinical tests | 12 months |