Evidence map›Paper›PMID 41176334›Full record

Observational studyOpen heart2025

Left atrial strain tracks abnormal ventricular mechanics in Fabry disease.

Chosita Cheepvasarach, Michael Gribble, Martin Ugander, Ravi Vijapurapu, Sabrina Nordin, Joao Augusto, Richard Paul Steeds, Michel Tchan, James C Moon, Faraz Pathan and 1 more

Registry-linked trialAbstract readMulticenter StudyObservational Study
PubMed Full text at PMC Publisher
In one paragraph

Observational study in Open heart, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03199001 (Native T1 Mapping by Cardiovascular Magnetic Resonance Imaging in Rare Diseases- A New Method to Improve Patient Care), which is not on this map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

NCT03199001 unknown statusnot on this map

Native T1 Mapping by Cardiovascular Magnetic Resonance Imaging in Rare Diseases- A New Method to Improve Patient Care

TypeobservationalSponsorUniversity College, LondonRan2015 to 2019Enrolled400ConditionsFabry Disease
3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Review
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

11 authors.

Chosita CheepvasarachRoyal North Shore Hospital, St Leonards, New South Wales, Australia chositacx@gmail.com.ORCID http://orcid.org/0009-0003-6277-3199
Michael GribbleThe University of Sydney School of Medicine, Sydney, New South Wales, Australia.
Martin UganderThe University of Sydney School of Medicine, Sydney, New South Wales, Australia.
Ravi VijapurapuCardiology, Queen Elizabeth Hospital Birmingham, Birmingham, UK.
Sabrina NordinCardiology, Barts Health NHS Trust, London, UK.
Joao AugustoHospital Prof Doutor Fernando Fonseca, Amadora, Portugal.
Richard Paul SteedsCardiology, Queen Elizabeth Hospital Birmingham, Birmingham, UK.ORCID http://orcid.org/0000-0001-5687-2535
Michel TchanThe University of Sydney Faculty of Medicine and Health, Sydney, New South Wales, Australia.
James C MoonCardiovascular Sciences, Barts Health NHS Trust, London, UK.
Faraz PathanThe University of Sydney, Sydney, New South Wales, Australia.
Rebecca KozorSydney Medical School, The University of Sydney School of Medicine, Sydney, New South Wales, Australia.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundFabry disease (FD) is an X linked lysosomal disorder with ventricular myocardial involvement that drives morbidity and mortality. Early diagnosis of cardiac involvement can be difficult. This study explored whether abnormal left atrial (LA) strain by cardiovascular magnetic resonance (CMR) may be an early sign of ventricular involvement in FD.

methodsA multicentre, multinational cohort of patients with FD was assembled with images centralised for core lab analysis. Adult patients with gene-positive FD and healthy volunteers (HV) underwent CMR. LA strain analyses included manually contouring the left atrium in end-diastole and end-systole to calculate LA volumes and ejection fraction, then semiautomatic analysis for LA reservoir strain.

resultsThere were n=214 patients with FD (mean age 45±15 years, 39% males) and n=76 HV (49±15 years, 53% males). CMR results in FD: left ventricular ejection fraction 73% (IQR=9), left ventricular mass index (LVMi) 89±39 g/m

conclusionsLA strain is abnormal in FD with LVH (overt disease) and correlates with LVMi, native T1 and GLS. LA strain is normal in FD with early disease (LVH negative+low T1) and normal in FD with no myocardial disease (LVH negative+normal T1). These findings indicate that LA strain is a consequence of abnormal LV mechanics such as LVH and abnormal GLS, rather than isolated myocardial sphingolipid deposition. TRIAL REGISTRATION NUMBER: NCT03199001.

Indexed as

Atrial Function, LeftFabry DiseaseHeart AtriaHypertrophy, Left VentricularMagnetic Resonance Imaging, CineAdultFemaleHeart VentriclesHumansMaleMiddle AgedStroke VolumeVentricular Dysfunction, LeftVentricular Function, LeftCardiac Imaging TechniquesMagnetic Resonance AngiographyMagnetic Resonance Imaging

Identifiers

PMID41176334
PMCPMC12581072

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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.