Long‐term prognostic value of ultrastructural features in dilated cardiomyopathy: comparison with cardiac magnetic resonance

Saito, Tsunenori; Asai, Kuniya; Tachi, Masaki; Satō, Shigeru; Mozawa, Kosuke; Adachi, Akiko; Sasaki, Yoshihiro; Amano, Yasuo; Mizuno, Kyoichi; Kumita, Shin‐ichiro; Shimizu, Wataru

doi:10.1002/ehf2.12662

Cited by 6 publications

(7 citation statements)

References 30 publications

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“…In the present study, however, the presence of LGE was not a predictor for cardiac outcomes. A similar result has been obtained in other studies 31,32 . The reason of discordant results was unclear.…”

Section: Discussionsupporting

confidence: 87%

Clinical significance of left atrial geometry in dilated cardiomyopathy patients: A cardiovascular magnetic resonance study

Yazaki

Nabeta

Inomata

et al. 2020

Clinical Cardiology

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Background Clinical significance of left atrial (LA) function and geometry in patients with dilated cardiomyopathy (DCM) remains uncertain. Hypothesis LA geometric parameters assessed by cardiac magnetic resonance (CMR) predict the prognosis in patients with DCM. Methods The present study included patients with DCM and sinus rhythm who underwent CMR between December 2007 and April 2018. LA volume was measured using CMR. LA sphericity index was computed as the ratio of the measured maximum LA volume by the volume of a sphere with maximum LA length diameter. Results We included 255 patients in this study. During the mean follow‐up of 3.92 years, hospitalization for HF occurred in 37 patients. The LA sphericity index was significantly higher in patients with hospitalization for HF than in those without (0.78 ± 0.35 vs. 0.58 ± 0.18, p < .001). Multivariable Cox regression analysis identified a higher LA sphericity index as an independent predictor of hospitalization for HF. Patients were categorized based on the median of LA sphericity index. The Kaplan–Meier curve showed that patients with a high LA sphericity index (≥0.57) had a significantly higher risk of hospitalization for HF than those with a low LA sphericity index (<0.57). Conclusion LA sphericity index was an independent predictor of hospitalization for HF. Assessment of LA geometric parameters might be useful for risk stratification in patients with DCM.

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Section: Discussionsupporting

confidence: 87%

Clinical significance of left atrial geometry in dilated cardiomyopathy patients: A cardiovascular magnetic resonance study

Yazaki

Nabeta

Inomata

et al. 2020

Clinical Cardiology

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“…Lipid droplets (L) are a finding suggestive of acute myocardial damage. 16 Li, lipofuscin; m, mitochondria. (G) In cardiomyocytes of P8 with a TMEM43 variant (c.271A>G), diffuse myofilament lysis (ML) spreads near the abnormal‐shaped nucleus (N).…”

Section: Resultsmentioning

confidence: 99%

“…The boundary is unclear and includes fewer glycogen granules (g) and mitochondria (m) than surrounding areas of cardiomyocytes. Lipid droplets (L) are a finding suggestive of acute myocardial damage 16 . Li, lipofuscin; m, mitochondria.…”

Section: Resultsmentioning

confidence: 99%

“…The limitations of EMB are well known, 26 particularly considering the complexity of ultrastructural interpretation and the small size of the electron microscopy field. 7 , 9 , 16 Because molecular analysis with DNA/RNA extraction was not performed in all patients, it cannot be completely ruled out that cardiomyopathy secondary to viral myocarditis was included. We think that further studies with increased numbers will elucidate the specific ultrastructural features for each gene variant.…”

Section: Discussionmentioning

confidence: 99%

“…The small number of patients in this study prevented the statistical analysis for associations with prognosis or cardiac function. The limitations of EMB are well known, 26 particularly considering the complexity of ultrastructural interpretation and the small size of the electron microscopy field 7,9,16 . Because molecular analysis with DNA/RNA extraction was not performed in all patients, it cannot be completely ruled out that cardiomyopathy secondary to viral myocarditis was included.…”

Section: Discussionmentioning

confidence: 99%

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Myocardial ultrastructure can augment genetic testing for sporadic dilated cardiomyopathy with initial heart failure

et al. 2021

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Aims The aim of the present study was to consider whether the ultrastructural features of cardiomyocytes in dilated cardiomyopathy can be used to guide genetic testing. Methods and results Endomyocardial biopsy and whole‐exome sequencing were performed in 32 consecutive sporadic dilated cardiomyopathy patients [51.0 (40.0–64.0) years, 75% men] in initial phases of decompensated heart failure. The predicted pathogenicity of ultrarare (minor allele frequency ≤0.0005), non‐synonymous variants was determined using the American College of Medical Genetics guidelines. Focusing on 75 cardiomyopathy‐susceptibility and 41 arrhythmia‐susceptibility genes, we identified 404 gene variants, of which 15 were considered pathogenic or likely pathogenic in 14 patients (44% of 32). There were five sarcomeric gene variants (29% of 17 variants) found in five patients (16% of 32), involving a variant of MYBPC3 and four variants of TTN. A patient with an MYBPC3 variant showed disorganized sarcomeres, three patients with TTN variants located in the region encoding the A‐band domain showed sparse sarcomeres, and a patient with a TTN variant in encoding the I‐band domain showed disrupted sarcomeres. The distribution of diffuse myofilament lysis depended on the causal genes; three patients with the same TMEM43 variant had diffuse myofilament lysis near nuclei (P = 0.011), while two patients with different DSP variants had lysis in the peripheral areas of cardiomyocytes (P = 0.033). Conclusions Derangement patterns of myofilament and subcellular distribution of myofilament lysis might implicate causal genes. Large‐scale studies are required to confirm whether these ultrastructural findings are related to the causative genes.

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Risk Stratification in Nonischemic Dilated Cardiomyopathy Using CMR Imaging

Eichhorn,

Koeckerling,

Reddy

et al. 2024

JAMA

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ImportanceAccurate risk stratification of nonischemic dilated cardiomyopathy (NIDCM) remains challenging.ObjectiveTo evaluate the association of cardiac magnetic resonance (CMR) imaging–derived measurements with clinical outcomes in NIDCM.Data SourcesMEDLINE, Embase, Cochrane Library, and Web of Science Core Collection databases were systematically searched for articles from January 2005 to April 2023.Study SelectionProspective and retrospective nonrandomized diagnostic studies reporting on the association between CMR imaging–derived measurements and adverse clinical outcomes in NIDCM were deemed eligible.Data Extraction and SynthesisPrespecified items related to patient population, CMR imaging measurements, and clinical outcomes were extracted at the study level by 2 independent reviewers. Random-effects models were fitted using restricted maximum likelihood estimation and the method of Hartung, Knapp, Sidik, and Jonkman.Main Outcomes and MeasuresAll-cause mortality, cardiovascular mortality, arrhythmic events, heart failure events, and major adverse cardiac events (MACE).ResultsA total of 103 studies including 29 687 patients with NIDCM were analyzed. Late gadolinium enhancement (LGE) presence and extent (per 1%) were associated with higher all-cause mortality (hazard ratio [HR], 1.81 [95% CI, 1.60-2.04]; P &lt; .001 and HR, 1.07 [95% CI, 1.02-1.12]; P = .02, respectively), cardiovascular mortality (HR, 2.43 [95% CI, 2.13-2.78]; P &lt; .001 and HR, 1.15 [95% CI, 1.07-1.24]; P = .01), arrhythmic events (HR, 2.69 [95% CI, 2.20-3.30]; P &lt; .001 and HR, 1.07 [95% CI, 1.03-1.12]; P = .004) and heart failure events (HR, 1.98 [95% CI, 1.73-2.27]; P &lt; .001 and HR, 1.06 [95% CI, 1.01-1.10]; P = .02). Left ventricular ejection fraction (LVEF) (per 1%) was not associated with all-cause mortality (HR, 0.99 [95% CI, 0.97-1.02]; P = .47), cardiovascular mortality (HR, 0.97 [95% CI, 0.94-1.00]; P = .05), or arrhythmic outcomes (HR, 0.99 [95% CI, 0.97-1.01]; P = .34). Lower risks for heart failure events (HR, 0.97 [95% CI, 0.95-0.98]; P = .002) and MACE (HR, 0.98 [95% CI, 0.96-0.99]; P &lt; .001) were observed with higher LVEF. Higher native T1 relaxation times (per 10 ms) were associated with arrhythmic events (HR, 1.07 [95% CI, 1.01-1.14]; P = .04) and MACE (HR, 1.06 [95% CI, 1.01-1.11]; P = .03). Global longitudinal strain (GLS) (per 1%) was not associated with heart failure events (HR, 1.06 [95% CI, 0.95-1.18]; P = .15) or MACE (HR, 1.03 [95% CI, 0.94-1.14]; P = .43). Limited data precluded definitive analysis for native T1 relaxation times, GLS, and extracellular volume fraction (ECV) with respect to mortality outcomes.ConclusionThe presence and extent of LGE were associated with various adverse clinical outcomes, whereas LVEF was not significantly associated with mortality and arrhythmic end points in NIDCM. Risk stratification using native T1 relaxation times, extracellular volume fraction, and global longitudinal strain requires further evaluation.

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Long‐term prognostic value of ultrastructural features in dilated cardiomyopathy: comparison with cardiac magnetic resonance

Cited by 6 publications

References 30 publications

Clinical significance of left atrial geometry in dilated cardiomyopathy patients: A cardiovascular magnetic resonance study

Clinical significance of left atrial geometry in dilated cardiomyopathy patients: A cardiovascular magnetic resonance study

Myocardial ultrastructure can augment genetic testing for sporadic dilated cardiomyopathy with initial heart failure

Risk Stratification in Nonischemic Dilated Cardiomyopathy Using CMR Imaging

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