Evidence map›Paper›PMID 34352074›Full record

ArticleEuropace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology2022

Clinical characteristics and risk stratification of desmoplakin cardiomyopathy.

Weijia Wang, Brittney Murray, Crystal Tichnell, Nisha A Gilotra, Stefan L Zimmerman, Alessio Gasperetti, Paul Scheel, Harikrishna Tandri, Hugh Calkins, Cynthia A James

Open access · hybridAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 57 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
57citing papers in PubMed, 1 pooled it
9.6field-weighted citation impact, top 1% of its field
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

57 citing papers in PubMed, 1 synthesis or guideline pooled it, 90 citations in OpenAlex.

  1. Pooled it
  2. Review
  3. Article
  4. Article
  5. Homozygous NonsenseJournal of clinical medicine · 2026
    Article
  6. Review
  7. Review
  8. A Cardiomyopathy to Remember: A Case Report of Desmoplakin Cardiomyopathy.European journal of case reports in internal medicine · 2026
    Article
  9. Article
  10. Article
  11. Article
  12. Observational
  13. Review
  14. Tailored therapeutics for cardiomyopathies.Nature reviews. Cardiology · 2025
    Review
  15. Article
  16. Article
  17. Article
  18. Diagnostic Criteria and Disease Staging for Desmoplakin Cardiomyopathy.medRxiv : the preprint server for health sciences · 2025
    Article
  19. Review
  20. Article
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

10 authors at 1 institution in 1 country.

Weijia WangDivision of Cardiology, Department of Medicine, Johns Hopkins University, Johns Hopkins Hospital, Blalock 545, 600 North Wolfe Street, Baltimore, MD 21287, USA.ORCID 0000-0002-9435-0145
Brittney MurrayDivision of Cardiology, Department of Medicine, Johns Hopkins University, Johns Hopkins Hospital, Blalock 545, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Crystal TichnellDivision of Cardiology, Department of Medicine, Johns Hopkins University, Johns Hopkins Hospital, Blalock 545, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Nisha A GilotraDivision of Cardiology, Department of Medicine, Johns Hopkins University, Johns Hopkins Hospital, Blalock 545, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Stefan L ZimmermanDivision of Cardiology, Department of Medicine, Johns Hopkins University, Johns Hopkins Hospital, Blalock 545, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Alessio GasperettiDivision of Cardiology, Department of Medicine, Johns Hopkins University, Johns Hopkins Hospital, Blalock 545, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Paul ScheelDivision of Cardiology, Department of Medicine, Johns Hopkins University, Johns Hopkins Hospital, Blalock 545, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Harikrishna TandriDivision of Cardiology, Department of Medicine, Johns Hopkins University, Johns Hopkins Hospital, Blalock 545, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Hugh CalkinsORCID 0000-0002-9262-9433
Cynthia A JamesDivision of Cardiology, Department of Medicine, Johns Hopkins University, Johns Hopkins Hospital, Blalock 545, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Johns Hopkins University · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

aimsDesmoplakin (DSP) cardiomyopathy is an increasingly recognized form of arrhythmogenic cardiomyopathy. With a genotype-specific approach, we characterized the diagnosis, natural history, and risk for ventricular arrhythmia and heart failure in DSP cardiomyopathy. METHODS AND

resultsWe followed 91 individuals [45 probands, 34% male, median age 27.5 years (interquartile interval 20.0-43.9)] with pathogenic or likely pathogenic DSP variants for a median of 4.3 years. Regarding the ventricular involvement, left predominance was most common (n = 22, 28%) followed by bi-ventricular in 12 (15%) and right predominance in 5 (6%). Myocardial injury (chest pain, elevated troponin, normal coronary angiogram) occurred in 20 (22%) individuals. Incidence rates of sustained ventricular arrhythmia and heart failure (ventricular dysfunction ± symptoms) were 5.9 [95% confidence interval (CI): 3.9-9.1] and 6.7 (95% CI: 4.5-9.8) per 100 person-years, respectively. In univariate regression, myocardial injury was associated with sustained ventricular arrhythmia [hazard ratio (HR) 2.53, 95% CI: 1.05-6.11] and heart failure (HR 7.53, 95% CI: 3.10-18.26). After adjustment, left ventricular ejection fraction <35% and right ventricular dysfunction were prognostic for sustained ventricular arrhythmia while proband status and myocardial injury were prognostic for heart failure (all P < 0.05). The sensitivity of the arrhythmogenic right ventricular cardiomyopathy Task Force Criteria in diagnosing left dominant disease was 0.73; 5/22 (23%) of patients with sustained ventricular arrhythmias did not meet these criteria.

conclusionDSP cardiomyopathy affects both ventricles and carries high risk for ventricular arrhythmia and heart failure. Myocardial injury is associated with worse disease outcomes. Both diagnosis and risk stratification of DSP cardiomyopathy need refinement.

Indexed as

Arrhythmogenic Right Ventricular DysplasiaCardiomyopathiesAdultDesmoplakinsFemaleHumansMaleRisk AssessmentStroke VolumeVentricular Function, LeftDesmoplakinsArrhythmogenic cardiomyopathyDesmoplakinHeart failureMyocardial injuryVentricular arrhythmia

Identifiers

PMID34352074
PMCPMC8824516
OpenAlexW3191581306

What OpenQuestion holds

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LicenceCC BY-NC
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Registered trials

None linked

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.