Observational studyCirculation. Cardiovascular imaging2019
Quantitative Myocardial Perfusion in Fabry Disease.
Observational study in Circulation. Cardiovascular imaging, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers, 2 of them syntheses that pooled 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.
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
39 citing papers in PubMed, 2 syntheses or guidelines pooled it, 56 citations in OpenAlex.
- Cardiovascular magnetic resonance native TJournal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance · 2020Pooled it
- Society for cardiovascular magnetic resonance expert consensus statement on quantitative myocardial perfusion cardiovascular magnetic resonance imaging.Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic ResonanceGuideline
- The Prognostic Significance of Quantitative Myocardial Perfusion: An Artificial Intelligence-Based Approach Using Perfusion Mapping.Circulation · 2020Trial
- ECG parameters to detect cardiac involvement in Fabry disease.BMC cardiovascular disorders · 2026Article
- Infiltrative and Inflammatory Cardiomyopathies: Using Imaging to Probe Mechanisms of Myocardial Dysfunction.Journal of cardiac failure · 2026Review
- Coronary Microvascular Dysfunction in Cardiomyopathies: Insights on Clinical and Prognostic Roles.Reviews in cardiovascular medicine · 2026Review
- AI-assisted longitudinal cardiac phenotyping identifies domain-specific remodeling patterns in Fabry cardiomyopathy.Frontiers in artificial intelligence · 2026Article
- Left ventricular myocardial work combined with stress echocardiography assessment of cardiac function in patients with Fabry disease.Scientific reports · 2025Article
- Cardiac Magnetic Resonance in the Evaluation and Management of Nonischemic Cardiomyopathies.JACC. Heart failure · 2025Review
- Cardiac manifestations of Fabry disease.NPJ cardiovascular health · 2025Review
- Myocardial Perfusion Imaging with Cardiovascular Magnetic Resonance in Nonischemic Cardiomyopathies: An In-Depth Review of Techniques and Clinical Applications.Medicina (Kaunas, Lithuania) · 2025Review
- MRI T2 Mapping of Dorsal Root Ganglia Reveals Increased T2 Relaxation Time in Classical Fabry Disease.Biomedicines · 2025Article
- Role of Cardiac Magnetic Resonance in Inflammatory and Infiltrative Cardiomyopathies: A Narrative Review.Journal of clinical medicine · 2024Review
- Arrhythmogenesis in Fabry Disease.Current cardiology reports · 2024Review
- Impairment of sympathetic activity in Fabry disease cardiomyopathy: A further challenge for cardiac imaging.Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology · 2023Article
- Inflammation across the spectrum of hypertrophic cardiac phenotypes.Heart failure reviews · 2023Review
- Hypertrophic Cardiomyopathy versus Storage Diseases with Myocardial Involvement.International journal of molecular sciences · 2023Review
- Coronary microvascular disease in hypertrophic and infiltrative cardiomyopathies.Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology · 2023Article
- Increased cardiac involvement in Fabry disease using blood-corrected native T1 mapping.Scientific reports · 2023Article
- Apical Ischemia Is a Universal Feature of Apical Hypertrophic Cardiomyopathy.Circulation. Cardiovascular imaging · 2023Article
Corrections and comments
- Commented on by
Authors and funding
14 authors at 5 institutions in 4 countries.
Funding
Abstract
backgroundFabry disease (FD) is an X-linked lysosomal storage disease resulting in tissue accumulation of sphingolipids. Key myocardial processes that lead to adverse outcomes in FD include storage, hypertrophy, inflammation, and fibrosis. These are quantifiable by multiparametric cardiovascular magnetic resonance. Recent developments in cardiovascular magnetic resonance perfusion mapping allow rapid in-line perfusion quantification permitting broader clinical application, including the assessment of microvascular dysfunction. We hypothesized that microvascular dysfunction in FD would be associated with storage, fibrosis, and edema.
methodsA prospective, observational study of 44 FD patients (49 years, 43% male, 24 [55%] with left ventricular hypertrophy [LVH]) and 27 healthy controls with multiparametric cardiovascular magnetic resonance including vasodilator stress perfusion mapping. Myocardial blood flow (MBF) was measured and its associations with other processes investigated.
resultsCompared with LVH- FD, LVH+ FD had higher left ventricular ejection fraction (73% versus 68%), more late gadolinium enhancement (85% versus 15%), and a lower stress MBF (1.76 versus 2.36 mL/g per minute). The reduction in stress MBF was more pronounced in the subendocardium than subepicardium. LVH- FD had lower stress MBF than controls (2.36 versus 3.00 mL/g per minute; P=0.002). Across all FD, late gadolinium enhancement and low native T1 were independently associated with reduced stress MBF. On a per-segment basis, stress MBF was independently associated with wall thickness, T2, extracellular volume fraction, and late gadolinium enhancement.
conclusionsFD patients have reduced perfusion, particularly in the subendocardium with greater reductions with LVH, storage, edema, and scar. Perfusion is reduced even without LVH suggesting it is an early disease marker.
Indexed as
Identifiers
What OpenQuestion holds
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.