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N/A N=33

Novel Cardiac Magnetic Resonance Imaging to Define a Unique Restrictive Cardiomyopathy in Sickle Cell Disease

Sickle Cell Disease

Enrolled (actual)
33
Serious AEs
0.0%
Results posted
Nov 2020
Primary outcome: Primary: Frequency of the Diffuse Myocardial Fibrosis Phenotype — 5; 10; 10; 1 Participants

Study Design & Population

Study type
Observational
Phase
N/A
Interventions
Cardiac magnetic resonance imaging (CMR) (Other)
Age
Pediatric, Adult, Older Adult · 6+ yrs
Sex
All
Sponsor
Children's Hospital Medical Center, Cincinnati
Primary completion
Jan 2018

Outcome Measures

OutcomeResultp-value
PRIMARY
Frequency of the Diffuse Myocardial Fibrosis Phenotype
5; 10; 10; 1

Summary

The purpose of this study is to use cardiac magnetic resonance imaging (CMR) and echocardiographic tissue Doppler imaging to demonstrate a unique restrictive cardiomyopathy of sickle cell disease. The investigators will characterize its frequency and how it might change (e.g., presence/absence and severity) over a 2-year period.

Eligibility Criteria

Inclusion Criteria

  • Sickle cell anemia (HbSS) or sickle-β°-thalassemia (HbSβ°) confirmed by hemoglobin separation and identification techniques
  • Ability to cooperate with and undergo CMR without sedation or anesthesia.
  • Ability to cooperate with and undergo echocardiogram
  • Written informed consent in accordance with the institutional policies and federal guidelines must be provided by the participant (if ≥18 years of age) or parent or legally authorized guardian (if the participant is 6 consecutive months since the initiation of disease-modifying therapy.

Exclusion Criteria

  • Any contraindication to MRI or physical or behavioral factor that could degrade the quality of MRI data or interfere with a participant's tolerance of the MRI, such as permanent or semi-permanent metallic implants, including pacemakers and defibrillators, or severe claustrophobia
  • Known ventricular septal defect (VSD) documented in medical record
  • Estimated Glomerular Filtration Rate (eGFR) <60 mL/min/1.73 m2 (estimated by serum creatinine or cystatin-C)
  • Pregnancy (documented by serum or urine pregnancy test)

The following additional inclusion criterion applies to strata A, B and C only:

  • Current chronic transfusion therapy (defined as regular, approximately monthly, transfusions of packed red blood cells given for at least 6 consecutive months for the treatment of prevention of SCD-related complications with the plan to continue this therapy at the time of potential enrollment).
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT02410811). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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