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

NCT ID: NCT04696289

Last Updated: 2026-01-30

Basic Information

• Recruitment Status: ACTIVE_NOT_RECRUITING
• Clinical Phase: NA
• Total Enrollment: 80 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2021-02-26
• Study Completion Date: 2026-12-31

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

Study Design

• Allocation Method: NA
• Intervention Model: SINGLE_GROUP
• Primary Study Purpose: DIAGNOSTIC
• Blinding Strategy: NONE

Study Groups

Standardized catheterization assessment

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

Group Type: EXPERIMENTAL

Standardized catheterization assessment

Intervention Type: 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.

Interventions

Standardized 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.

Intervention Type: DIAGNOSTIC_TEST

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.

• Maximum Eligible Age: 12 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Children's Hospital of Philadelphia

OTHER

Sponsor Role: collaborator

Boston Children's Hospital

OTHER

Sponsor Role: lead

Responsible Party

Ryan Callahan

Assistant Professor of Pediatrics

Responsibility Role: PRINCIPAL_INVESTIGATOR

Principal Investigators

Jesse Esch, MD

Role: PRINCIPAL_INVESTIGATOR

Boston Children's Hospital/Harvard Medical School

Locations

Boston Children's Hospital

Boston, Massachusetts, United States

Countries

United States

References

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.

Reference Type: BACKGROUND

PMID: 11009286 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 28622179 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 29576325 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 32936535 (View on PubMed)

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.

Reference Type: DERIVED

PMID: 40654078 (View on PubMed)

Other Identifiers

IRB-P00035534

Identifier Type: -

Identifier Source: org_study_id