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High ECG Risk-Scores Predict Late Gadolinium Enhancement on Magnetic Resonance Imaging in HCM in the Young

Österberg, Anna Wålinder ; Östman-Smith, Ingegerd ; Jablonowski, Robert LU ; Carlsson, Marcus LU ; Green, Henrik ; Gunnarsson, Cecilia ; Liuba, Petru LU and Fernlund, Eva LU orcid (2021) In Pediatric Cardiology 42(3). p.492-500
Abstract

An ECG risk-score has been described that predicts high risk of subsequent cardiac arrest in young patients with hypertrophic cardiomyopathy (HCM). Myocardial fibrosis measured by cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE) also affects prognosis. We assessed whether an ECG risk-score could be used as an indicator of myocardial fibrosis or perfusion deficit on CMR in HCM. In total 42 individuals (7–31 years); 26 HCM patients, seven genotype-positive, phenotype-negative individuals at risk of HCM (first-degree relatives) and nine healthy volunteers, underwent CMR to identify, and grade extent of, myocardial fibrosis and perfusion defect. 12-lead ECG was used for calculating the ECG risk-score (grading 0–14p).... (More)

An ECG risk-score has been described that predicts high risk of subsequent cardiac arrest in young patients with hypertrophic cardiomyopathy (HCM). Myocardial fibrosis measured by cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE) also affects prognosis. We assessed whether an ECG risk-score could be used as an indicator of myocardial fibrosis or perfusion deficit on CMR in HCM. In total 42 individuals (7–31 years); 26 HCM patients, seven genotype-positive, phenotype-negative individuals at risk of HCM (first-degree relatives) and nine healthy volunteers, underwent CMR to identify, and grade extent of, myocardial fibrosis and perfusion defect. 12-lead ECG was used for calculating the ECG risk-score (grading 0–14p). High-risk ECG (risk-score > 5p) occurred only in the HCM group (9/26), and the proportion was significantly higher vs mutation carriers combined with healthy volunteers (0/16, p = 0.008). Extent of LGE correlated to the ECG-score (R2 = 0.47, p = 0.001) in sarcomeric mutations. In low-risk ECG-score patients (0–2p), median percent of myocardium showing LGE (LGE%LVM) were: 0% [interquartile range, IQR, 0–0%], in intermediate-risk (3–5p): 5.4% [IQR 0–13.5%] and in high-risk (6–14p): 10.9% [IQR 4.2–12.3%]. ECG-score > 2p had a sensitivity and specificity of 79% and 84% to detect positive LGE on CMR and 77% vs. 75% to detect perfusion defects in sarcomeric mutations carriers. In patients with myocardial fibrosis as identified by LGE, median ECG risk-score was 8p [range 3–10p]. In conclusions, ECG risk-score > 2 p could be used as a cut-off for screening of myocardial fibrosis. Thus ECG risk-score is an inexpensive complementary tool in risk stratification of HCM in the young.

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author
; ; ; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
CMR, ECG risk-score, HCM, Myocardial fibrosis, Myocardial perfusion defect
in
Pediatric Cardiology
volume
42
issue
3
pages
492 - 500
publisher
Springer
external identifiers
  • scopus:85099915603
  • pmid:33515326
ISSN
0172-0643
DOI
10.1007/s00246-020-02506-9
language
English
LU publication?
yes
id
8c5a3a52-6314-487f-b5f4-84895415ec94
date added to LUP
2021-02-08 13:06:08
date last changed
2024-05-02 02:45:56
@article{8c5a3a52-6314-487f-b5f4-84895415ec94,
  abstract     = {{<p>An ECG risk-score has been described that predicts high risk of subsequent cardiac arrest in young patients with hypertrophic cardiomyopathy (HCM). Myocardial fibrosis measured by cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE) also affects prognosis. We assessed whether an ECG risk-score could be used as an indicator of myocardial fibrosis or perfusion deficit on CMR in HCM. In total 42 individuals (7–31 years); 26 HCM patients, seven genotype-positive, phenotype-negative individuals at risk of HCM (first-degree relatives) and nine healthy volunteers, underwent CMR to identify, and grade extent of, myocardial fibrosis and perfusion defect. 12-lead ECG was used for calculating the ECG risk-score (grading 0–14p). High-risk ECG (risk-score &gt; 5p) occurred only in the HCM group (9/26), and the proportion was significantly higher vs mutation carriers combined with healthy volunteers (0/16, p = 0.008). Extent of LGE correlated to the ECG-score (R<sup>2</sup> = 0.47, p = 0.001) in sarcomeric mutations. In low-risk ECG-score patients (0–2p), median percent of myocardium showing LGE (LGE%LVM) were: 0% [interquartile range, IQR, 0–0%], in intermediate-risk (3–5p): 5.4% [IQR 0–13.5%] and in high-risk (6–14p): 10.9% [IQR 4.2–12.3%]. ECG-score &gt; 2p had a sensitivity and specificity of 79% and 84% to detect positive LGE on CMR and 77% vs. 75% to detect perfusion defects in sarcomeric mutations carriers. In patients with myocardial fibrosis as identified by LGE, median ECG risk-score was 8p [range 3–10p]. In conclusions, ECG risk-score &gt; 2 p could be used as a cut-off for screening of myocardial fibrosis. Thus ECG risk-score is an inexpensive complementary tool in risk stratification of HCM in the young.</p>}},
  author       = {{Österberg, Anna Wålinder and Östman-Smith, Ingegerd and Jablonowski, Robert and Carlsson, Marcus and Green, Henrik and Gunnarsson, Cecilia and Liuba, Petru and Fernlund, Eva}},
  issn         = {{0172-0643}},
  keywords     = {{CMR; ECG risk-score; HCM; Myocardial fibrosis; Myocardial perfusion defect}},
  language     = {{eng}},
  month        = {{01}},
  number       = {{3}},
  pages        = {{492--500}},
  publisher    = {{Springer}},
  series       = {{Pediatric Cardiology}},
  title        = {{High ECG Risk-Scores Predict Late Gadolinium Enhancement on Magnetic Resonance Imaging in HCM in the Young}},
  url          = {{http://dx.doi.org/10.1007/s00246-020-02506-9}},
  doi          = {{10.1007/s00246-020-02506-9}},
  volume       = {{42}},
  year         = {{2021}},
}