Predicting Pediatric Pulmonary Vein Stenosis Outcomes Using Data Acquired During a Cardiac Catheterization

Study Overview
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Brief Summary
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This is a prospective, single center study which applies a standardized, comprehensive catheterization assessment to patients with a known or suspected diagnosis of pulmonary vein stenosis (PVS) who are undergoing a cardiac catheterization at Boston Children's Hospital. As part of the assessment, each pulmonary vein will undergo angiography (pictures using moving x-rays and contrast dye), intravascular ultrasound (IVUS; pictures of the vein wall using a catheter inside the vein), pressure assessment and compliance testing. The status of each pulmonary vein will then be assessed 12 months after the catheterization (i.e. no disease, severe disease, etc.). Using statistics, the investigators will determine which patient and vein characteristics (obtained at the of catheterization) can predict whether or not a pulmonary vein will have disease. The investigators hypothesize that this comprehensive, standardized, invasive assessment of pediatric intraluminal PVS can predict vein outcome.

Official Title
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Predicting Pediatric Intraluminal Pulmonary Vein Stenosis Outcomes Using a Comprehensive Standardized Catheterization Assessment

Conditions
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Pulmonary Vein Stenosis

Intervention / Treatment
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* Diagnostic Test: Standardized catheterization assessment


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Pediatric patients undergoing cardiac catheterization for the indication of known or suspicion for intraluminal pulmonary vein stenosis. Exclusion Criteria: Hemodynamically unstable patients as determined by the patient care team.

Ages Eligible for Study
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Maximum Age: 12 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: Diagnostic
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Standardized catheterization assessment<br>Assessment of pulmonary veins including angiography, intravascular ultrasound, pressure assessment and compliance testing. | Diagnostic Test: Standardized catheterization assessment<br>* There are four components to the pulmonary vein assessment. Angiography: pulmonary artery wedge injection and/or selective pulmonary vein injection. Intravascular ultrasound: manual pullback of IVUS catheter from lobar segment to left atrium. Pressure assessment: pulmonary vein pressure (mmHg) measured before and after (if applicable) intervention. Compliance testing: the compliance of the vein will be measured using a compliant conventional balloon.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Pulmonary vein status | Severity of pulmonary vein stenosis (score 0 to 3); Score 0: Unobstructed vein (best outcome), Score 1: Proximal vein disease, Score 2: Distal vein disease, Score 3: Vein atresia (worst outcome). | 12 months +/- 6 weeks or prior to study exit due to death or lung transplant | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Patient status | Transplant free survival | 12 months |