40 Participants Needed

PET Imaging for Heart Function in Sickle Cell Disease

PR
Overseen ByParul Rai, MD
No Placebo GroupAll trial participants will receive the active study treatment (no placebo)
Prior Safety DataThis treatment has passed at least one previous human trial

Trial Summary

What is the purpose of this trial?

There is limited information on what causes injury to the heart in individuals with Sickle Cell Disease (SCD). Researchers in this study want to see if decreased blood flow to the heart during stress could be causing the heart damage seen in SCD patients. They also want to test people who don't have SCD to see if their hearts react the same way under stress. Primary Objective * To estimate the coronary flow reserve (CFR) (also referred to as myocardial perfusion reserve), as measured by PET stress-rest myocardial perfusion imaging, in SCD patients with and without diastolic dysfunction, and healthy controls. Secondary Objectives * To investigate the relationship between decreased CFR (quantified with PET stress- rest myocardial perfusion imaging) and presence of abnormal diastolic parameters

Will I have to stop taking my current medications?

The trial information does not specify whether you need to stop taking your current medications. However, if you have a seizure disorder and are on anti-epileptic drugs (AED), you may be excluded from participating.

What data supports the effectiveness of the treatment involving PET Imaging for Heart Function in Sickle Cell Disease?

The research suggests that PET imaging can be useful in detecting impaired metabolism in areas around major vessel infarcts in sickle cell disease, which may help in understanding heart function. However, it is not generally effective for small vessel disease, and more studies are needed to confirm its usefulness.12345

How does PET imaging for heart function in sickle cell disease differ from other treatments?

PET imaging with 13N-ammonia is unique because it allows for noninvasive, detailed measurement of blood flow in the heart, which can help assess heart function in sickle cell disease. This approach is different from standard treatments as it provides a precise evaluation of myocardial perfusion (blood flow in the heart muscle) and can detect subtle changes in heart function that other methods might miss.678910

Research Team

PR

Parul Rai, MD

Principal Investigator

St. Jude Children's Research Hospital

Eligibility Criteria

This trial is for adults aged 18-21 with Sickle Cell Disease (SCD) and heart dysfunction, specifically those with abnormal diastolic parameters. It includes three groups: SCD patients with diastolic dysfunction, those without it, and healthy black individuals as controls.

Inclusion Criteria

I am 18-21, Black, with SCD (HbSS or HbSβ0thalassemia), and have minimal heart issues.
I am a healthy Black individual aged 18-21 with two or fewer abnormal diastolic parameters.
I am 18-21, Black, with sickle cell disease and heart issues.

Exclusion Criteria

Stratum C: All genotypes of SCD, Diagnosed with three or more abnormal diastolic parameters (based on guidelines by American Society of echocardiography), Individual with hemoglobin level below the normal range for that age and sex, Individuals with signs, symptoms or EKG findings of acute myocardial ischemia, infarction or unstable angina, Individuals with history of VT/VF or SVT, Previous cardiac surgery, Known congenital heart disease (other than patent ductus arteriosus or Atrial septal defect), Stenotic valvular disease or left main coronary artery stenosis, History of myo/pericarditis, Left ventricle systolic dysfunction, Cardiovascular instability/uncontrolled hypertension (h/o hypertensive urgency or emergency), History of sinus node dysfunction or high-grade AV nodal block, History of aborted sudden cardiac death or cardiac arrest, Current seizure disorder on AED, Pregnant/Breast-feeding, Any medical or social reason, which, in the opinion of the principal investigators would make the participation of the subject ill-advised
Stratum A: Recent hospitalization for vaso-occlusive pain crisis or acute chest syndrome in last 4 weeks, Blood transfusion in the last 3 months, Individuals with signs, symptoms or EKG findings of acute myocardial ischemia, infarction or unstable angina, Individuals with history of VT/VF or SVT, Previous cardiac surgery, Known congenital heart disease (other than patent ductus arteriosus or Atrial septal defect), Stenotic valvular disease or left main coronary artery stenosis, History of myo/pericarditis, Left ventricle systolic dysfunction, Cardiovascular instability/uncontrolled hypertension (h/o hypertensive urgency or emergency), History of sinus node dysfunction or high grade AV nodal block, History of aborted sudden cardiac death or cardiac arrest, Current seizure disorder on AED, Pregnant/Breast-feeding, Any medical or social reason, which, in the opinion of the principal investigators would make the participation of the subject ill-advised
I have a complex heart condition or recent severe health issues.

Timeline

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Initial Assessment

First visit includes blood tests, an electrocardiogram (EKG), and an echocardiogram

1 day
1 visit (in-person)

PET Imaging and Stress Test

Second visit includes a positron emission tomography (PET) stress test and a blood test

1 day
1 visit (in-person)

Follow-up

Participants are monitored for safety and effectiveness after PET imaging study tests with next day and one-week follow-up phone calls

1 week

Treatment Details

Interventions

  • [13N]NH3
  • Lexiscan
  • Positron emission tomography
Trial OverviewThe study aims to measure blood flow reserve in the heart using PET scans during rest and stress conditions. Researchers will compare results between SCD patients with varying levels of heart function and healthy people to understand the cause of heart damage in SCD.
Participant Groups
3Treatment groups
Experimental Treatment
Group I: Stratum CExperimental Treatment3 Interventions
Healthy controls
Group II: Stratum BExperimental Treatment3 Interventions
Sickle cell patients without diastolic dysfunction
Group III: Stratum AExperimental Treatment3 Interventions
Sickle Cell patients with diastolic dysfunction

Find a Clinic Near You

Who Is Running the Clinical Trial?

St. Jude Children's Research Hospital

Lead Sponsor

Trials
451
Recruited
5,326,000+

Findings from Research

In a study involving 12 children with sickle cell disease and 5 controls, arterial spin labeling (ASL) showed that cerebral blood flow (CBF) was higher in patients compared to controls, indicating potential alterations in blood flow due to the disease.
The study highlighted the importance of correcting CBF measurements for hematocrit and blood velocity, as these adjustments improved the accuracy of flow estimates and revealed clinically relevant asymmetries in some patients, although no direct link was found between CBF asymmetries and silent infarcts.
Arterial spin labeling measurement of cerebral perfusion in children with sickle cell disease.Gevers, S., Nederveen, AJ., Fijnvandraat, K., et al.[2019]
Doppler tissue imaging (DTI) revealed that children with sickle cell anemia (SCA) have significantly higher right and left ventricular Tei indexes, indicating impaired myocardial function compared to healthy children, with 85 SCA patients studied.
There is a strong negative correlation between mean hemoglobin concentration and the Tei indexes, suggesting that lower hemoglobin levels are associated with worse cardiac function in SCA children, highlighting the importance of early screening for potential cardiac issues.
Assessment of cardiac functions in children with sickle cell anemia: doppler tissue imaging study.AboHadeed, HM., Zolaly, MA., Khoshhal, SQ., et al.[2018]
In a study involving 14 children with sickle cell disease and 7 control subjects, cerebral blood flow (CBF) was found to be significantly higher in the sickle cell patients across all cerebral artery territories, indicating altered blood flow dynamics in these patients.
Despite higher overall CBF, some sickle cell patients exhibited decreased blood flow in specific areas of the brain, suggesting that conventional imaging may not fully capture the extent of cerebral blood flow abnormalities in this condition.
Sickle cell disease: continuous arterial spin-labeling perfusion MR imaging in children.Oguz, KK., Golay, X., Pizzini, FB., et al.[2016]

References

Arterial spin labeling measurement of cerebral perfusion in children with sickle cell disease. [2019]
Assessment of cardiac functions in children with sickle cell anemia: doppler tissue imaging study. [2018]
Sickle cell disease: continuous arterial spin-labeling perfusion MR imaging in children. [2016]
Noninvasive diagnostic imaging in hemoglobinopathies. [2007]
Role of positron emission tomography in determining the extent of CNS ischemia in patients with sickle cell disease. [2019]
Noninvasive evaluation of regional myocardial perfusion with positron emission computed tomography. [2019]
Tomographic images of blood pool and perfusion in brain and heart. [2015]
Reproducibility of measurements of regional resting and hyperemic myocardial blood flow assessed with PET. [2022]
Quantitative evaluation of myocardial blood flow with [13N]ammonia. [2018]
10.United Statespubmed.ncbi.nlm.nih.gov
Nitrogen-13 ammonia perfusion imaging: relation to metabolic imaging. [2019]