Pulmonary Vein StenosisNCT04696289
Predicting Pediatric Pulmonary Vein Stenosis Outcomes Using Data Acquired During a Cardiac Catheterization
A study testing Standardized catheterization assessment in people with Pulmonary Vein Stenosis, run by Boston Children's Hospital at 1 site in United States.
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. Read more about this study
- Who is it for?
- Ages Up to 12 years
- Conditions the study is for
- Pulmonary Vein Stenosis
- What is being tested?
- Standardized catheterization assessment
- Type of treatment
- Injection or infusion
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 80 participants
- Where
- 1 siteUnited States
- Study dates
- 26 Feb 2021to Dec 2026
- Phase
- Interventional
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages Up to 12 years
- All sexes
- No healthy volunteers
- Child
Who may be able to join
- Pediatric patients undergoing cardiac catheterization for the indication of known or suspicion for intraluminal pulmonary vein stenosis.
Who cannot join
- Hemodynamically unstable patients as determined by the patient care team.
Trial locations and contacts
1 site in 1 country.
United States1 site
Boston Children's Hospital
Boston, Massachusetts, 02115
About this study
Official title: Predicting Pediatric Intraluminal Pulmonary Vein Stenosis Outcomes Using a Comprehensive Standardized Catheterization Assessment
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
- Being tested
- Standardized catheterization assessment
- Medical subject headings
- Stenosis, Pulmonary Vein
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Standardized catheterization assessmentDiagnostic test
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.
| Study group | Role | What they receive |
|---|---|---|
| Standardized catheterization assessment | Experimental | Assessment of pulmonary veins including angiography, intravascular ultrasound, pressure assessment and compliance testing. Receives: Diagnostic Test: Standardized catheterization assessment |
What is being measured
The primary outcome is the main question the study is designed to answer.
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).
Measured: 12 months +/- 6 weeks or prior to study exit due to death or lung transplant
Secondary outcomes (1)
Patient status
Transplant free survival
Measured: 12 months
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted6 Jan 2021
- Study start26 Feb 2021
- Primary completion1 Dec 2025
- Study completionDec 2026 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (5)Papers the sponsor has linked to this study.
- Other trials for Pulmonary Vein StenosisStudies of the same condition, open and under way.
- More trials run by Boston Children's HospitalThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase N/A
- Primary purpose
- Diagnosis
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Enrolment
- 80 participants (actual)
Sponsor and investigators
- Lead sponsor
- Boston Children's Hospital
- Sponsor type
- University, hospital or other
- Collaborators
- Children's Hospital of Philadelphia
- Responsible party
- Principal investigator, Ryan Callahan, Boston Children's Hospital
- Investigators
- Jesse Esch, MD, Principal investigator, Boston Children's Hospital/Harvard Medical School
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04696289
- Sponsor's protocol ID
- IRB-P00035534
- Status last verified
- Jan 2026
- First submitted
- 4 Jan 2021
- Last updated
- 30 Jan 2026
- Results posted
- No
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
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.
PubMed 11009286Kovach 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.
PubMed 28622179Callahan 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.
PubMed 29576325Callahan 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.
PubMed 32936535Callahan 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.
PubMed 40654078
Study at a glance
From the registry record, last updated by the sponsor on 30 Jan 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Predicting Pediatric Intraluminal Pulmonary Vein Stenosis Outcomes Using a Comprehensive Standardized Catheterization Assessment
- ClinicalTrials.gov ID
- NCT04696289
- Acronym / sponsor ID
- IRB-P00035534
- Conditions
- Pulmonary Vein Stenosis
- Study type
- Interventional
- Recruitment status
- Active, not recruiting
- Enrolment
- 80 participants (actual)
- Lead sponsor
- Boston Children's HospitalUniversity, hospital or other
- Collaborators
- Children's Hospital of Philadelphia
- Study start
- 26 Feb 2021 (actual)
- Primary completion
- 1 Dec 2025 (actual)
- Study completion
- Dec 2026 (estimated)
- First posted
- 6 Jan 2021 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Study design
- Primary purpose
- Diagnosis
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Standardized catheterization assessment | Experimental | Diagnostic Test: Standardized catheterization assessmentAssessment of pulmonary veins including angiography, intravascular ultrasound, pressure assessment and compliance testing. |
Who may qualify
- Ages
- Up to 12 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child
Key inclusion criteria (1 listed)
- Pediatric patients undergoing cardiac catheterization for the indication of known or suspicion for intraluminal pulmonary vein stenosis.
Key exclusion criteria (1 listed)
- Hemodynamically unstable patients as determined by the patient care team.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Standardized catheterization assessmentThere 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. | Diagnostic test | Standardized catheterization assessment |
Primary outcome
Pulmonary vein status
Time frame: 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).
1 secondary outcome
Patient status
Time frame: 12 months
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 30 Jan 2026.
- Background: PubMed 11009286Sadr 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.
- Background: PubMed 28622179Kovach 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.
- Background: PubMed 29576325Callahan 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.
- Background: PubMed 32936535Callahan 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.
- Derived from this study: PubMed 40654078Callahan 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.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Jesse Esch, MD principal investigator
Boston Children's Hospital/Harvard Medical School
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A study testing Standardized catheterization assessment in people with Pulmonary Vein Stenosis, run by Boston Children's Hospital at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 1 inclusion and 1 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: under way, not looking for participants, as of the sponsor's last update on 30 Jan 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Pulmonary vein status (time frame: 12 months +/- 6 weeks or prior to study exit due to death or lung transplant). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT04696289, which also lists 5 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 30 Jan 2026.