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

Pulmonary Vein Stenosis Clinical Trial

Official title:

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

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT04696289
• Other study ID #: IRB-P00035534
• Status: Recruiting
• Phase: N/A
• Start date: February 26, 2021
• Est. completion date: December 2025

Study information

• Verified date: April 2024
• Source: Boston Children's Hospital
• Contact: Jesse Esch, MD
• Phone: 6173558228
• Email: jesse.esch[@]cardio.chboston.org
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 80
• Est. completion date: December 2025
• Est. primary completion date: December 2024
• Accepts healthy volunteers: No
• Gender: All
• Age group: N/A to 12 Years

Eligibility

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.

Related Conditions & MeSH terms

• Constriction, Pathologic
• Pulmonary Vein Stenosis

Intervention

Diagnostic Test:
• Standardized catheterization assessment
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.

Locations

Country	Name	City	State
United States	Boston Children's Hospital	Boston	Massachusetts

Sponsors (2)

Lead Sponsor	Collaborator
Boston Children's Hospital	Children's Hospital of Philadelphia

Country where clinical trial is conducted

United States, 

References & Publications (4)

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. — View 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. — View 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. — View 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. — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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	Patient status	Transplant free survival	12 months