Evidence map›Paper›PMID 29622585›Full record

Observational studyCirculation2018

Prevalence and Progression of Late Gadolinium Enhancement in Children and Adolescents With Hypertrophic Cardiomyopathy.

Anna Axelsson Raja, Hoshang Farhad, Anne Marie Valente, John-Paul Couce, John Lynn Jefferies, Henning Bundgaard, Kenneth Zahka, Harry Lever, Anne M Murphy, Euan Ashley and 7 more

Abstract readMulticenter StudyObservational Study
PubMed Full text at PMC Publisher
In one paragraph

Observational study in Circulation, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 49 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
49citing papers in PubMed, 2 pooled it
–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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

49 citing papers in PubMed, 2 syntheses or guidelines pooled it.

  1. Guideline
  2. Pooled it
  3. Article
  4. Advanced myocardial characterization and function with cardiac CT.The international journal of cardiovascular imaging · 2026
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  14. Reduction in myofilament CaFrontiers in physiology · 2025
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4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

17 authors.

Anna Axelsson RajaRigshospitalet, University of Copenhagen, Denmark (A.A., H.B.).
Hoshang FarhadBrigham and Women's Hospital, Boston, MA (A.A., H.F., C.Y.H.).
Anne Marie ValenteBoston Children's Hospital, MA (A.M.V., J.-P.C., S.D.C.).
John-Paul CouceBoston Children's Hospital, MA (A.M.V., J.-P.C., S.D.C.).
John Lynn JefferiesCincinnati Children's Hospital Medical Center, OH (J.L.J.).
Henning BundgaardRigshospitalet, University of Copenhagen, Denmark (A.A., H.B.).
Kenneth ZahkaCleveland Clinic, OH (K.Z., H.L.).
Harry LeverCleveland Clinic, OH (K.Z., H.L.).
Anne M MurphyJohns Hopkins University, Baltimore, MD (A.M.M.).
Euan AshleyStanford University School of Medicine, Palo Alto, CA (E.A.).
Sharlene M DayUniversity of Michigan, Ann Arbor (S.M.D.).
Mark V SherridNew York University Langone Medical Center, NY (M.V.S.).
Ling ShiNew England Research Institutes, Watertown, MA (L.S.).
David A BluemkeUniversity of Wisconsin, Madison (D.A.B.).
Charles E CanterWashington University School of Medicine, St. Louis, MO (C.E.C.).
Steven D ColanBoston Children's Hospital, MA (A.M.V., J.-P.C., S.D.C.).
Carolyn Y HoBrigham and Women's Hospital, Boston, MA (A.A., H.F., C.Y.H.).

Funding

Using Genetics For Early Phenotyping & Prevention of Hypertrophic CardiomyopathyP50HL112349 · NHLBI · BRIGHAM AND WOMEN'S HOSPITAL · PI HO, CAROLYN Y, SEIDMAN, CHRISTINE E · 2012 to 2017
$11.2M
Using Genetics for Early Phenotyping & Prevention of Hypertrophic CardiomyopathyP20HL101408 · NHLBI · BRIGHAM AND WOMEN'S HOSPITAL · PI HO, CAROLYN Y, SEIDMAN, CHRISTINE E · 2010 to 2011
$1.5M
NHLBI NIH HHS P20 HL101408NHLBI NIH HHS P50 HL112349
6 · The paper itself

Abstract

backgroundLate gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) is believed to represent dense replacement fibrosis. It is seen in ≈60% of adult patients with hypertrophic cardiomyopathy (HCM). However, the prevalence of LGE in children and adolescents with HCM is not well established. In addition, longitudinal studies describing the development and evolution of LGE in pediatric HCM are lacking. This study assesses the prevalence, progression, and clinical correlations of LGE in children and adolescents with, or genetically predisposed to, HCM.

methodsCMR scans from 195 patients ≤21 years of age were analyzed in an observational, retrospective study, including 155 patients with overt HCM and 40 sarcomere mutation carriers without left ventricular (LV) hypertrophy. The extent of LGE was quantified by measuring regions with signal intensity >6 SD above nulled remote myocardium.

resultsPatients were 14.3±4.5 years of age at baseline and 68% were male. LGE was present in 70 (46%) patients with overt HCM (median extent, 3.3%; interquartile range, 0.8-7.1%), but absent in mutation carriers without LV hypertrophy. Thirty-one patients had >1 CMR (median interval between studies, 2.4 years; interquartile range, 1.5-3.2 years). LGE was detected in 13 patients (42%) at baseline and in 16 patients (52%) at follow-up CMR. The median extent of LGE increased by 2.4 g/y (range, 0-13.2 g/y) from 2.9% (interquartile range, 0.8-3.2%) of LV mass to 4.3% (interquartile range, 2.9-6.8%) ( P=0.02). In addition to LGE, LV mass and left atrial volume, indexed to body surface area, and z score for LV mass, as well, increased significantly from first to most recent CMR.

conclusionsLGE was present in 46% of children and adolescents with overt HCM, in contrast to ≈60% typically reported in adult HCM. In the subset of patients with serial imaging, statistically significant increases in LGE, LV mass, and left atrial size were detected over 2.5 years, indicating disease progression over time. Further prospective studies are required to confirm these findings and to better understand the clinical implications of LGE in pediatric HCM.

Indexed as

Magnetic Resonance ImagingAdolescentAge FactorsCardiomyopathy, HypertrophicChildContrast MediaDisease ProgressionFemaleFibrosisGenetic Predisposition to DiseaseHumansMalePhenotypePredictive Value of TestsPrevalenceRetrospective StudiesContrast Mediacardiomyopathy, hypertrophicfibrosismagnetic resonance imagingpediatricssarcomeres

Identifiers

PMID29622585
PMCPMC6173664

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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.