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

NCT04696289 | Active Not Recruiting DMC

• 1 other identifier: IRB-P00035534
• Study Type: interventional
• Target: 80
• Locations: 1 country
• Sites: 1

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.

Conditions

Pulmonary Vein Stenosis

Outcome Measures

Primary Outcomes (1)

• 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 Outcomes (1)

• Patient status

  12 months

Study Arms (1)

Standardized catheterization assessment

EXPERIMENTAL

Assessment of pulmonary veins including angiography, intravascular ultrasound, pressure assessment and compliance testing.

Diagnostic Test: Standardized catheterization assessment

Interventions

Standardized catheterization assessment DIAGNOSTIC_TEST

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.

Standardized catheterization assessment

Eligibility Criteria

• Age: Up to 12 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17)

You may qualify if:

• Pediatric patients undergoing cardiac catheterization for the indication of known or suspicion for intraluminal pulmonary vein stenosis.

You may not qualify if:

• Hemodynamically unstable patients as determined by the patient care team.

Sponsors & Collaborators

• Boston Children's Hospital: lead
• Children's Hospital of Philadelphia: collaborator

Study Sites (1)

Boston Children's Hospital

Boston, Massachusetts, 02115, United States

Location

Related Publications (5)

• 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.

  PMID: 11009286 BACKGROUND

• 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.

  PMID: 28622179 BACKGROUND

• 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.

  PMID: 29576325 BACKGROUND

• 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.

  PMID: 32936535 BACKGROUND

• Callahan R, Gauvreau K, Keochakian M, Esch JJ, Porras D, Bergersen L, Beroukhim R, Farias M, Harrild DM, Ireland CM, Kwatra N, Jenkins KJ. Predicting Outcomes in Pediatric Intraluminal Pulmonary Vein Stenosis Using a Comprehensive Standardized Catheterization Assessment: A Prospective Study. Circ Cardiovasc Interv. 2025 Sep;18(9):e015002. doi: 10.1161/CIRCINTERVENTIONS.124.015002. Epub 2025 Jul 14.

  PMID: 40654078 DERIVED

MeSH Terms

Conditions

Stenosis, Pulmonary Vein

Condition Hierarchy (Ancestors)

Vascular Diseases; Cardiovascular Diseases

Study Officials

• Jesse Esch, MD

  Boston Children's Hospital/Harvard Medical School

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: DIAGNOSTIC
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Assistant Professor of Pediatrics

Study Record Dates

• First Submitted: January 4, 2021
• First Posted: January 6, 2021
• Study Start: February 26, 2021
• Primary Completion: December 1, 2025
• Study Completion (Estimated): December 1, 2026
• Last Updated: January 30, 2026

Record last verified: 2026-01

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)