Pulmonary Vein Stenosis
Conditions
Brief summary
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.
Interventions
DIAGNOSTIC_TESTStandardized catheterization assessment
There are four components to the pulmonary vein assessment. 1. Angiography: pulmonary artery wedge injection and/or selective pulmonary vein injection. 2. Intravascular ultrasound: manual pullback of IVUS catheter from lobar segment to left atrium. 3. Pressure assessment: pulmonary vein pressure (mmHg) measured before and after (if applicable) intervention. 4. Compliance testing: the compliance of the vein will be measured using a compliant conventional balloon.
Sponsors
Boston Children's Hospital
Children's Hospital of Philadelphia
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
No minimum to 12 Years
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Pulmonary vein status | 12 months +/- 6 weeks or prior to study exit due to death or lung transplant | 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). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Patient status | 12 months | Transplant free survival |
Countries
United States
Contacts
PRINCIPAL_INVESTIGATORJesse Esch, MD
Boston Children's Hospital/Harvard Medical School
Outcome results
None listed