Pulmonary Vein Stenosis
Conditions
Keywords
Cardiac Magnetic Resonance Imaging, Wall Shear Stress
Brief summary
The purpose of this study is to better understand pediatric pulmonary vein stenosis (PVS), which is the narrowing of blood vessels that connect the lungs to the heart. PVS is a life-threatening disease without a clear cause. The investigators think patients who develop PVS have an increased Wall Shear Stress (WSS) level in the pulmonary veins, which is the force placed on the walls of the veins. This study will determine if WSS can be calculated in the pulmonary veins of infants using Ferumoxytol enhanced Cardiac Magnetic Resonance Imaging (FcMRI). If possible, the investigators aim to use FcMRI to better screen patients at risk of PVS and to help guide therapy in patients with PVS.
Detailed description
Background: The mechanism of pediatric intraluminal pulmonary vein stenosis (PVS) remains unknown. It is hypothesized that elevated wall shear stress (WSS) as a result of excessive pulmonary blood flow (left to right shunts) and/or pulmonary vein distortion from surrounding anatomy contributes to the neo-intimal proliferation. Calculating WSS in pediatric pulmonary veins using ferumoxytol enhanced cardiac magnetic resonance (FcMRI) has not been reported and would represent a novel method of evaluation. Objectives: The primary objective is to determine the feasibility of calculating WSS in infant pulmonary veins using FcMRI. The secondary objective is to determine the magnitude and variability of WSS in pulmonary veins among high-risk patients and normal controls. Study Design: Prospective, interventional, single center, feasibility study Setting/Participants: Single center study at The Children's Hospital of Philadelphia. High-risk infants (n = 10) will include two groups of patients; (1) infants with moderate to severe bronchopulmonary dysplasia (BPD) and (2) infants with postoperative repair of total anomalous pulmonary venous connection (TAPVC). Group 1 participants will be infants who are undergoing an MRI as part of clinical care for other issues (i.e., MRI brain for hypoxic ischemia encephalopathy), with the research FcMRI being performed following the clinical care MRI. Group 2 participants will be infants who are undergoing FcMRI as part of clinical care. Controls (n = 10) will be pediatric patients without intracardiac defects who are undergoing FcMRI as part of clinical care (i.e., evaluation of anomalous coronary, aortopathy, vascular ring). Study Procedures, Interventions and Measures: Participants will undergo FcMRI and have the WSS calculated in each pulmonary vein (right upper, right lower, left upper, left lower) using several methodologies. Patients will be followed for 12 months following cMRI monitoring for a new diagnosis of PVS.
Interventions
DRUGFerumoxytol
Ferumoxytol will be used as a contrast agent for the cMRI
Sponsors
Children's Hospital of Philadelphia
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
No minimum to 18 Years
Healthy volunteers
No
Inclusion criteria
Normal (Controls) Subjects Inclusion Criteria 1. Males or Females less than 18 years of age. 2. Weight \> 3 kg. 3. Undergoing cMRI with ferumoxytol as part of clinical care. 4. Structurally normal heart (by echocardiography) with exception of small left to right shunts, isolated valve pathology, anomalous coronary arteries, extracardiac vascular anomalies such as arch anomalies. 5. Parental/guardian permission (informed consent). Normal (Controls) Subjects
Exclusion criteria
1. Congenital heart disease (except small left to right shunts, isolated valve pathology, anomalous coronary arteries, extracardiac vascular anomalies such as arch anomalies). 2. Parents/guardians or subjects who, in the opinion of the Investigator, may be non-compliant with study schedules or procedures. 3. Patient not receiving ferumoxytol as part of their cMRI due to a known hypersensitivity to the drug, or a known diagnosis of iron overload. High-Risk Subject Inclusion Criteria 1. Males or Females less than 12 months of age. 2. Diagnosis of severe BPD (group 1) or TAPVC s/p repair (group 2). 3. Weight \> 3 kg. 4. Receiving respiratory support with an invasive airway (tracheostomy or endotracheal tube) (group 1 only). 5. Undergoing cMRI with ferumoxytol as part of clinical care (group 2 only). 6. Parental/guardian permission (informed consent). High-Risk Subject
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Wall Shear Stress level in infant pulmonary veins using cMRI | 12 months | WSS (dyn/cm2) will be reported in each pulmonary vein using multiple modalities with data from the cMRI. This includes computational fluid dynamics, 4D-Flow and 4\*Mu\*v/r, where Mu is the viscosity of blood, r is the vessel radius, and v is the average velocity of blood. |
Countries
United States
Contacts
CONTACTRyan Callahan, MD
PRINCIPAL_INVESTIGATORRyan Callahan, MD
Children's Hospital of Philadelphia
Outcome results
None listed