Heart Failure in Patients with Arrhythmogenic Cardiomyopathy

Chen, Shi; Chen, Liang; Duru, Fırat; Hu, Shengshou

doi:10.3390/jcm10204782

Cited by 9 publications

(11 citation statements)

References 113 publications

(166 reference statements)

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“…30 Mutations like DSG2, DSP, and PLN often lead to biventricular involvement and accelerate HF course in this disease. 35 In accordance with these studies, our findings also reflected the specific role of gene mutations in predicting adverse HF outcomes. However, to provide a more applicable and easy-to-use tool for physicians, we opted not to include the genotype as a risk factor in our model.…”

Section: Prior Studies On Risk Predictionsupporting

confidence: 88%

Novel Risk Prediction Model to Determine Adverse Heart Failure Outcomes in Arrhythmogenic Right Ventricular Cardiomyopathy

Chen

Saguner

et al. 2022

JAHA

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Background Patients with arrhythmogenic right ventricular cardiomyopathy are at risk for life‐threatening ventricular tachyarrhythmias, but progressive heart failure (HF) may occur in later stages of disease. This study aimed to characterize potential risk predictors and develop a model for individualized assessment of adverse HF outcomes in arrhythmogenic right ventricular cardiomyopathy. Methods and Results Longitudinal and observational cohorts with 290 patients with arrhythmogenic right ventricular cardiomyopathy from the Fuwai Hospital in Beijing, China, and 99 patients from the University Heart Center in Zurich, Switzerland, with follow‐up data were studied. The primary end point of the study was heart transplantation or death attributable to HF. The model was developed by Cox regression analysis for predicting risk and was internally validated. During 4.92±3.03 years of follow‐up, 48 patients reached the primary end point. The determinants of the risk prediction model were left ventricular ejection fraction, serum creatinine levels, moderate‐to‐severe tricuspid regurgitation, and atrial fibrillation. Implantable cardioverter‐defibrillators did not reduce the occurrence of adverse HF outcomes. Conclusions A novel risk prediction model for arrhythmogenic right ventricular cardiomyopathy has been developed using 2 large and well‐established cohorts, incorporating common clinical parameters such as left ventricular ejection fraction, serum creatinine levels, tricuspid regurgitation, and atrial fibrillation, which can identify patients who are at risk for terminal HF events, and may guide physicians to assess individualized HF risk and to optimize management strategies.

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Section: Prior Studies On Risk Predictionsupporting

confidence: 88%

Novel Risk Prediction Model to Determine Adverse Heart Failure Outcomes in Arrhythmogenic Right Ventricular Cardiomyopathy

Chen

Saguner

et al. 2022

JAHA

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“…Arrhythmia is a common manifestation of ARVC. An association between ventricular arrhythmia and heart failure has not been reported [12]. In this study, a history of VF or unstable sVT was more frequent in patients who reached the endpoint with borderline statistical significance.…”

Section: Discussioncontrasting

confidence: 58%

“…According to the current knowledge, changes in the ECG also have a prognostic value in ARVC. First-degree atrioventricular block, low voltage of the QRS complexes, inversion of T waves in leads V4-V6, and the presence of the epsilon wave were associated with the progression of heart failure [12,21,25]. We found a higher incidence of epsilon wave and a longer QRS duration in leads V1-V3 in patients who reached the primary endpoint.…”

Section: Discussionmentioning

confidence: 57%

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NT-proBNP is superior to novel plasma biomarkers for predicting adverse outcome in arrhythmogenic right ventricular cardiomyopathy

Borowiec¹,

Woźniak²,

Wróbel³

et al. 2023

Polish Archives of Internal Medicine

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The study aimed to identify predictors for developing end-stage heart failure and evaluate the role of biomarkers in predicting adverse outcomes in arrhythmogenic right ventricular cardiomyopathy (ARVC). We showed that biomarkers (sST2, MMP-2, NT-proBNP, and hsTnT, but not Gal-3 and MMP-9) have a prognostic value for death or heart transplantation in ARVC, while they have no role in predicting ventricular arrhythmia. Moreover, we propose a prognostic model for end-stage heart failure including three independent predictors: NT-proBNP concentration ≥ 890.3 pg/ml, right ventricular end-diastolic area ≥ 39.0 cm2, and history of atrial tachycardia. Such a model may be helpful for clinicians in the day-to-day management of patients with ARVC.

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“…Arrhythmogenic cardiomyopathy (AC) is a frequent cause of sudden cardiac death in young adults and may lead to heart failure [ 1 , 2 ]. It is characterized by focal fibrofatty replacement of myocardium leading to ventricular dilation and ventricular wall aneurysms [ 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

Autophagy and Endoplasmic Reticulum Stress during Onset and Progression of Arrhythmogenic Cardiomyopathy

Pitsch

Kant

Mytzka

et al. 2021

Cells

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Arrhythmogenic cardiomyopathy (AC) is a heritable, potentially lethal disease without a causal therapy. AC is characterized by focal cardiomyocyte death followed by inflammation and progressive formation of connective tissue. The pathomechanisms leading to structural disease onset and progression, however, are not fully elucidated. Recent studies revealed that dysregulation of autophagy and endoplasmic/sarcoplasmic reticulum (ER/SR) stress plays an important role in cardiac pathophysiology. We therefore examined the temporal and spatial expression patterns of autophagy and ER/SR stress indicators in murine AC models by qRT-PCR, immunohistochemistry, in situ hybridization and electron microscopy. Cardiomyocytes overexpressing the autophagy markers LC3 and SQSTM1/p62 and containing prominent autophagic vacuoles were detected next to regions of inflammation and fibrosis during onset and chronic disease progression. mRNAs of the ER stress markers Chop and sXbp1 were elevated in both ventricles at disease onset. During chronic disease progression Chop mRNA was upregulated in right ventricles. In addition, reduced Ryr2 mRNA expression together with often drastically enlarged ER/SR cisternae further indicated SR dysfunction during this disease phase. Our observations support the hypothesis that locally altered autophagy and enhanced ER/SR stress play a role in AC pathogenesis both at the onset and during chronic progression.

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Heart Failure in Patients with Arrhythmogenic Cardiomyopathy

Cited by 9 publications

References 113 publications

Novel Risk Prediction Model to Determine Adverse Heart Failure Outcomes in Arrhythmogenic Right Ventricular Cardiomyopathy

Novel Risk Prediction Model to Determine Adverse Heart Failure Outcomes in Arrhythmogenic Right Ventricular Cardiomyopathy

NT-proBNP is superior to novel plasma biomarkers for predicting adverse outcome in arrhythmogenic right ventricular cardiomyopathy

Autophagy and Endoplasmic Reticulum Stress during Onset and Progression of Arrhythmogenic Cardiomyopathy

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