Clinical trial NCT04696289: Predicting Pediatric Pulmonary Vein Stenosis Outcomes Using Data Acquired During a Cardiac Catheterization
Official title: Predicting Pediatric Intraluminal Pulmonary Vein Stenosis Outcomes Using a Comprehensive Standardized Catheterization Assessment
Study type: INTERVENTIONAL
Overall status: ACTIVE_NOT_RECRUITING
Phase: NA
Conditions: Pulmonary Vein Stenosis
Lead sponsor: Boston Children's Hospital
Sponsor class: OTHER
Collaborators: name: Children's Hospital of Philadelphia
First posted: 2021-01-06
Enrollment: 80
Sex: ALL
Maximum age (years): 12
Standard ages: CHILD
Accepts healthy volunteers: no
Allocation: NA
Intervention model: SINGLE_GROUP
Primary purpose: DIAGNOSTIC

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.


Eligibility criteria: 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.

Primary outcomes:
measure: Pulmonary vein status
timeFrame: 12 months +/- 6 weeks or prior to study exit due to death or lung transplant
Secondary outcomes:
measure: Patient status
timeFrame: 12 months
Other outcomes:
Locations:
city: Boston
country: United States
facility: Boston Children's Hospital
state: Massachusetts
Study officials:
affiliation: Boston Children's Hospital/Harvard Medical School
name: Jesse Esch, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: Sadr IM, Tan PE, Kieran MW, Jenkins KJ. Mechanism of pulmonary vein stenosis in infants with normally connected veins. Am J Cardiol. 2000 Sep 1;86(5):577-9, A10. doi: 10.1016/s0002-9149(00)01022-5.
citation: Kovach AE, Magcalas PM, Ireland C, McEnany K, Oliveira AM, Kieran MW, Baird CW, Jenkins K, Vargas SO. Paucicellular Fibrointimal Proliferation Characterizes Pediatric Pulmonary Vein Stenosis: Clinicopathologic Analysis of 213 Samples From 97 Patients. Am J Surg Pathol. 2017 Sep;41(9):1198-1204. doi: 10.1097/PAS.0000000000000892.
citation: Callahan R, Kieran MW, Baird CW, Colan SD, Gauvreau K, Ireland CM, Marshall AC, Sena LM, Vargas SO, Jenkins KJ. Adjunct Targeted Biologic Inhibition Agents to Treat Aggressive Multivessel Intraluminal Pediatric Pulmonary Vein Stenosis. J Pediatr. 2018 Jul;198:29-35.e5. doi: 10.1016/j.jpeds.2018.01.029. Epub 2018 Mar 23.
citation: Callahan R, Jenkins KJ, Gauthier Z, Gauvreau K, Porras D. Preliminary findings on the use of intravascular ultrasound in the assessment of pediatric pulmonary vein stenosis. Catheter Cardiovasc Interv. 2021 Feb 15;97(3):E362-E370. doi: 10.1002/ccd.29264. Epub 2020 Sep 16.