Standardized Catheterization Assessment for Pulmonary Vein Stenosis

Overseen By Jesse Esch, MD

Age: < 18

Sex: Any

Trial Phase: Academic

Sponsor: Boston Children's Hospital

Disqualifiers: Hemodynamic instability

No Placebo Group All trial participants will receive the active study treatment (no placebo)

Approved in 1 Jurisdiction This treatment is already approved in other countries

Compensation: Varies

Number of Visits: 1

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial aims to determine how well a specific test, the standardized catheterization assessment, predicts the future health of pulmonary veins in children with pulmonary vein stenosis (PVS). The test involves taking detailed pictures and measurements of the veins using special tools during a heart procedure. Researchers aim to discover if these early results can forecast the condition of the veins after one year. Children already undergoing a heart procedure who may have PVS could be suitable candidates for this study. As an unphased trial, this study offers a unique opportunity to contribute to medical knowledge and potentially enhance future care for children with PVS.

Do I have to stop taking my current medications for the trial?

The trial protocol does not specify whether you need to stop taking your current medications.

Will I have to stop taking my current medications?

The trial information does not specify whether you need to stop taking your current medications.

What prior data suggests that this standardized catheterization assessment is safe for patients with pulmonary vein stenosis?

Research has shown that the standard catheterization test is generally safe for patients. This test uses a thin tube, called a catheter, to take detailed pictures and measurements of the blood vessels in the lungs. Studies have applied this method to children under 13 who needed heart tests, and it has yielded good results. Specifically, one study found that 92% of patients did not require a heart or lung transplant within a year after the procedure.

Although the procedure involves inserting tools into the body, it is designed to be thorough and help doctors understand the condition of the veins without major risks. No widespread reports of serious side effects have emerged from this test, suggesting it is well-tolerated by patients. However, discussing any concerns with a healthcare provider is always advisable, as they can offer advice based on specific health needs. ^{1 2 3 4 5}

Why are researchers excited about this trial?

Researchers are excited about the standardized catheterization assessment because it offers a comprehensive approach to evaluating pulmonary vein stenosis. Unlike traditional methods that might rely solely on angiography, this assessment includes a combination of angiography, intravascular ultrasound, pressure assessment, and compliance testing. This multifaceted approach allows for a more detailed understanding of the vein's condition, potentially leading to more precise and effective treatment strategies. By integrating these advanced techniques, the assessment could significantly improve the diagnosis and management of pulmonary vein stenosis, offering hope for better patient outcomes.

What evidence suggests that this standardized catheterization assessment is effective for pulmonary vein stenosis?

Research has shown that a standard test, which participants in this trial will undergo, can help predict outcomes for children with pulmonary vein stenosis (PVS). One study found that 92% of children avoided a lung transplant within a year after the test, indicating that most did not need a transplant during that period. The test uses tools like special X-rays and ultrasound to provide clear images of the veins, aiding doctors in understanding the condition better. While no direct evidence suggests this test can cure PVS, it appears promising for managing and predicting the disease. ^{2 3 4 5 6}

Who Is on the Research Team?

Jesse Esch, MD

Principal Investigator

Boston Children's Hospital/Harvard Medical School

Are You a Good Fit for This Trial?

This trial is for pediatric patients who are suspected to have or are known to have pulmonary vein stenosis (PVS) and are stable enough to undergo a cardiac catheterization at Boston Children's Hospital.

Inclusion Criteria

My child is having a heart procedure to check for narrowed lung veins.

Exclusion Criteria

Hemodynamically unstable patients as determined by the patient care team.

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Catheterization Assessment

Participants undergo a comprehensive catheterization assessment including angiography, intravascular ultrasound, pressure assessment, and compliance testing

1 day

1 visit (in-person)

Follow-up

Participants are monitored for pulmonary vein status and transplant-free survival

12 months

Periodic assessments

What Are the Treatments Tested in This Trial?

Interventions

• Standardized catheterization assessment

Trial Overview The study tests a comprehensive catheterization assessment during cardiac catheterization, including angiography, intravascular ultrasound, pressure measurement, and compliance testing. The goal is to predict PVS outcomes in children.

How Is the Trial Designed?

Group I: Standardized catheterization assessment Experimental Treatment 1 Intervention

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

Standardized catheterization assessment is already approved in United States for the following indications:

Approved in United States as Standardized catheterization assessment for:

• Diagnosis and assessment of pediatric pulmonary vein stenosis

Who Is Running the Clinical Trial?

Boston Children's Hospital

Lead Sponsor

Trials

801

Recruited

5,584,000+

Children's Hospital of Philadelphia

Collaborator

Trials

749

Recruited

11,400,000+

Published Research Related to This Trial

Quality assurance (QA) and quality improvement (QI) initiatives in cardiac catheterization laboratories are essential for enhancing patient safety and optimizing interactions between patients and healthcare teams.

National registries and appropriate use criteria (AUC) for revascularization are key tools for benchmarking performance and ensuring that medical procedures are used effectively, especially in the context of value-based healthcare.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Quality management in the cardiac catheterization laboratory. Henien, S., Aronow, HD., Abbott, JD.[2020]

In a review of 4454 pediatric cardiac catheterizations over 9 years, the overall incidence of adverse events was found to be 9.3%, indicating a higher risk compared to general pediatric anesthesia.

Infants under 1 year old experienced a significantly higher event rate of 13.9%, particularly during therapeutic interventions, highlighting the need for careful monitoring in this vulnerable age group.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Incidents and complications during pediatric cardiac catheterization. Bennett, D., Marcus, R., Stokes, M.[2012]

In a study of 240 patients with pulmonary vein stenosis who underwent 841 catheterizations, serious adverse events occurred in 12% of cases, with pulmonary hemorrhage and arrhythmia being the most common, while severe events like stroke and death were rare.

Younger patients (under 6 months) and those with low oxygen saturation or severely elevated pulmonary artery pressure were more likely to experience serious adverse events and require high-level cardiorespiratory support after the procedure.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Predictors of Serious Adverse Events and High-Level Cardiorespiratory Support in Patients Undergoing Transcatheter Pulmonary Vein Interventions. Barreto, JA., Gauvreau, K., Porras, D., et al.[2023]

In a study of 513 patients with acute lung injury, physical examination findings like cool extremities and knee mottling were found to have high specificity but low sensitivity for predicting low cardiac index and mixed venous oxygen saturation, indicating they are not reliable indicators of ineffective circulation.

Central venous oxygen saturation levels below 70% were associated with low mixed venous oxygen saturation, but a level of 70% or higher could effectively rule out the risk of low mixed venous oxygen saturation, suggesting it may be a useful measure in clinical assessments.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Association of physical examination with pulmonary artery catheter parameters in acute lung injury. Grissom, CK., Morris, AH., Lanken, PN., et al.[2021]

In a study of 1043 patients with cardiogenic shock, those who received pulmonary artery catheters (PACs) had a lower in-hospital mortality rate (29.3%) compared to those who did not receive PACs (36.2%), particularly benefiting patients in more severe stages of shock (SCAI stages D and E).

Patients with PACs were more likely to receive advanced heart failure therapies, such as temporary mechanical circulatory support and durable ventricular assist devices, indicating that PACs may enhance treatment options and outcomes in severe cases of cardiogenic shock.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Pulmonary Artery Catheter Usage and Impact on Mortality in Patients with Cardiogenic Shock: Results from a Canadian, Single Centre Registry. Réa, ABBAC., Mihajlovic, V., Vishram-Nielsen, JKK., et al.[2023]

In a review of 126 heart catheterization procedures performed on children, adverse events occurred in 13% of cases, with serious complications in 5.5%, indicating that while the procedure is generally safe, there are risks involved.

Factors such as being under one year old, having an unscheduled admission, and the use of inotropic drugs were associated with a higher likelihood of experiencing adverse events, highlighting the importance of thorough pre-procedure assessments.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

[Adverse events during diagnostic and therapeutic pediatric cardiac catheterization]. Zepeda-Arámbula, A., Gutiérrez-Cobian, L., Villatoro-Fernández, JL., et al.[2019]

In a study of 4,401 children and young adults with pulmonary hypertension undergoing cardiac catheterization, the observed risk of serious adverse outcomes was 3.5%, indicating a significant risk associated with this invasive procedure.

Factors such as younger age, concurrent cardiac operations, and pre-procedural systemic vasodilator infusion increased the risk of adverse outcomes, while the use of pulmonary vasodilators before the procedure was linked to a reduced risk.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Predictors of Catastrophic Adverse Outcomes in Children With Pulmonary Hypertension Undergoing Cardiac Catheterization: A Multi-Institutional Analysis From the Pediatric Health Information Systems Database. O'Byrne, ML., Glatz, AC., Hanna, BD., et al.[2018]

In a study of 8111 cardiac catheterization procedures in children and young adults with pulmonary hypertension, the overall risk of catastrophic adverse events was found to be 1.4%, with a 5.2% risk of death before discharge, highlighting the high-risk nature of this population.

Factors such as being a prematurely born neonate, receiving inotropic treatment before the procedure, and having lower systemic arterial saturation were linked to increased risks of adverse events, while higher hospital experience with pulmonary hypertension procedures was associated with a reduced risk of complications.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Risk Factors for Major Early Adverse Events Related to Cardiac Catheterization in Children and Young Adults With Pulmonary Hypertension: An Analysis of Data From the IMPACT (Improving Adult and Congenital Treatment) Registry. O'Byrne, ML., Kennedy, KF., Kanter, JP., et al.[2020]

Pulmonary artery catheterization is a valuable diagnostic tool that helps measure various heart pressures and cardiac output, providing insights into conditions like heart failure and pulmonary hypertension.

The review highlights the challenges in accurately assessing hemodynamic measurements due to varying techniques, which can affect clinical decisions and management strategies.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Right heart catheterization in clinical practice: a review of basic physiology and important issues relevant to interpretation. Del Rio-Pertuz, G., Nugent, K., Argueta-Sosa, E.[2023]

The Society for Cardiac Angiography and Interventions has established a structured quality improvement program for cardiac catheterization labs, emphasizing the importance of identifying risk-adjusted quality indicators to ensure valid comparisons of outcomes.

The program includes a comprehensive data collection and evaluation process to monitor patient outcomes and adverse events, followed by corrective actions and reassessment to enhance the quality of care in these medical settings.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Guidelines for continuous quality improvement in the cardiac catheterization laboratory. Laboratory Performance Standards Committee of the Society for Cardiac Angiography & Interventions. Heupler, FA., al-Hani, AJ., Dear, WE.[2019]

The pulmonary artery catheter has been linked to increased morbidity and mortality, prompting a workshop by the NHLBI and FDA to improve its safety and utility in clinical settings.

There is a strong need for standardized education for healthcare professionals on the use and interpretation of pulmonary artery catheters, with a focus on specific clinical scenarios like heart failure and sepsis, to enhance patient outcomes.

NCBI (Pubmed)

pubmed.ncbi.nlm.nih.gov

Pulmonary artery catheterization and clinical outcomes: National Heart, Lung, and Blood Institute and Food and Drug Administration Workshop Report. Consensus Statement. Bernard, GR., Sopko, G., Cerra, F., et al.[2022]

Citations

1. clinicaltrials.gov clinicaltrials.gov/study/NCT04696289

Predicting Pediatric Pulmonary Vein Stenosis Outcomes ... Predicting Outcomes in Pediatric Intraluminal Pulmonary Vein Stenosis Using a Comprehensive Standardized Catheterization Assessment: A Prospective Study.

2. pubmed.ncbi.nlm.nih.gov pubmed.ncbi.nlm.nih.gov/40654078/

Predicting Outcomes in Pediatric Intraluminal Pulmonary ... Transplant-free survival was 92% at 12 months. Conclusions: A multimodal pulmonary vein catheterization assessment determined angiographic and ...

3. withpower.com withpower.com/trial/phase-stenosis-pulmonary-vein-1-2021-75a92

Standardized Catheterization Assessment for Pulmonary ... The available research does not provide specific data supporting the effectiveness of Standardized Catheterization Assessment for Pulmonary Vein Stenosis. The ...

4. researchgate.net researchgate.net/publication/393669894_Predicting_Outcomes_in_Pediatric_Intraluminal_Pulmonary_Vein_Stenosis_Using_a_Comprehensive_Standardized_Catheterization_Assessment_A_Prospective_Study

Predicting Outcomes in Pediatric Intraluminal Pulmonary ... Predicting Outcomes in Pediatric Intraluminal Pulmonary Vein Stenosis Using a Comprehensive Standardized Catheterization Assessment: A Prospective Study.

5. clinicalradiologyonline.net clinicalradiologyonline.net/article/S0009-9260(23)00235-0/fulltext

Evaluation of paediatric pulmonary vein stenosis by ... CCTA plays an important role in detecting paediatric PV stenosis and identifies additional findings compared to TTE that have direct surgical/interventional ...

6. ahajournals.org ahajournals.org/doi/10.1161/CIRCINTERVENTIONS.124.015002?doi=10.1161/CIRCINTERVENTIONS.124.015002

Predicting Outcomes in Pediatric Intraluminal Pulmonary Vein ... The study is a prospective trial applying a standardized catheterization assessment to patients under 13 years of age who are undergoing cardiac catheterization ...