Evidence map›Paper›PMID 41312339›Full record

ArticleBlood red cells & iron2025

Longitudinal changes and predictors of cardiac extracellular volume fraction in sickle cell anemia.

Omar Niss, Cara E Morin, Sassan Hashemi, Tarek Alsaied, Sean M Lang, Michael D Taylor, MacKenzie Tasset, Punam Malik, Charles T Quinn

Registry-linked trialAbstract read
In one paragraph

Article in Blood red cells & iron, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02410811 (Novel Cardiac Magnetic Resonance Imaging to Define a Unique Restrictive Cardiomyopathy in Sickle Cell Disease), which is not on this map. Not yet cited in PubMed.

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1 · What the graph read from it

What it found

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT02410811 completednot on this map

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

TypeobservationalSponsorChildren's Hospital Medical Center, CincinnatiRan2014 to 2019Enrolled33ConditionsSickle Cell DiseaseArmsCardiac magnetic resonance imaging (CMR)
3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors.

Omar NissDivision of Hematology, Cincinnati Children's Hospital Medical Center.
Cara E MorinDepartment of Radiology, Cincinnati Children's Hospital Medical Center.
Sassan HashemiDivision of Cardiothoracic Surgery, Cincinnati Children's Hospital Medical Center.
Tarek AlsaiedDepartment of Cardiology, UPMC Children's Hospital of Pittsburgh.
Sean M LangDivision of Cardiology, Cincinnati Children's Hospital Medical Center.
Michael D TaylorDepartment of Pediatrics, University of Texas at Austin.
MacKenzie TassetDivision of Hematology, Cincinnati Children's Hospital Medical Center.
Punam MalikDivision of Hematology, Cincinnati Children's Hospital Medical Center.
Charles T QuinnDivision of Hematology, Cincinnati Children's Hospital Medical Center.

Funding

Cincinnati Center of Excellence in Hemoglobinopathies ResearchU01HL117709 · NHLBI · CINCINNATI CHILDRENS HOSP MED CTR · PI MALIK, PUNAM · 2013 to 2017
$8.9M
NHLBI NIH HHS U01 HL117709
6 · The paper itself

Abstract

Diffuse myocardial fibrosis is a key feature of the cardiomyopathy of sickle cell anemia (SCA) that is linked to diastolic dysfunction. Cardiac MRI (CMR)-derived extracellular volume fraction (ECV) is a quantitative biomarker of myocardial fibrosis that is elevated in SCA. The stability of ECV in SCA and its clinical associations over time are unknown. To measure longitudinal changes in ECV, without specific intervention, and identify correlates of ECV change in patients with SCA, we conducted a prospective, 2-year study involving annual CMR, echocardiography, and laboratory assessments in 24 patients with SCA (mean age 21.4±10 years). ECV was calculated using T1 mapping pre- and post-gadolinium, and extracellular matrix and cellular volumes were calculated to account for relative changes. Diastolic function was classified by echocardiography. Longitudinal associations were assessed using linear mixed-effects models. ECV was abnormally increased at baseline and remained stable over time (mean change -1.1±2.7% per year). Intra-subject ECV variability was low (mean 2.4%), with no differences by age or sex. ECV correlated with diastolic dysfunction at all timepoints (P=0.01). Changes in ECV were more closely associated with changes in extracellular matrix volume (P=0.01) than with myocardial cell volume. In a longitudinal multivariable analysis, ECV was independently associated with diastolic dysfunction, hemoglobin, NT-proBNP, left atrial volume, and peak pulmonary artery velocity. In summary, ECV is a stable and reliable metric of myocardial extracellular matrix expansion in SCA. Its strong associations with diastolic dysfunction and biomarkers of cardiac stress support its use as an imaging biomarker for antifibrotic therapies in SCA. This trial was registered at www.ClinicalTrials.gov as #NCT02410811.

Identifiers

PMID41312339
PMCPMC12652408

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