Prediction of the estimated 5-year risk of sudden cardiac death and syncope or non-sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy using late gadolinium enhancement and extracellular volume CMR

Avanesov, Maxim; Münch, Julia; Weinrich, Julius Matthias; Well, Lennart; Säring, Dennis; Stehning, Christian; Tahir, Enver; Bohnen, Sebastian; Radunski, Ulf K; Muellerleile, Kai; Adam, Gerhard; Patten, Monica; Lund, Gunnar

doi:10.1007/s00330-017-4869-x

Cited by 60 publications

(38 citation statements)

References 37 publications

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“…In early stages of hypertrophic cardiomyopathies, replacement scars are absent and only microscopic intramyocardial fibrosis is present, and its extent can be assessed by CMR using T1-mapping ( Dass et al, 2012 ) or extracellular volume (ECV) measurements with gadolinium contrast, both altered even before the onset of hypertrophy in HCM-mutation carriers ( Ho et al, 2013 ). However, the link between the degree of ECV expansion and the risk of arrhythmias in early stage HCM is still uncertain ( Avanesov et al, 2017 ). Therefore, arrhythmias in HCM cannot be the sole consequence of the substrate for re-entry circuits at tissue level.…”

Section: Introduction: Arrhythmic Substrate In Hcm From Tissue To Thmentioning

confidence: 99%

Altered Ca2+ and Na+ Homeostasis in Human Hypertrophic Cardiomyopathy: Implications for Arrhythmogenesis

et al. 2018

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Hypertrophic cardiomyopathy (HCM) is the most common mendelian heart disease, with a prevalence of 1/500. HCM is a primary cause of sudden death, due to an heightened risk of ventricular tachyarrhythmias that often occur in young asymptomatic patients. HCM can slowly progress toward heart failure, either with preserved or reduced ejection fraction, due to worsening of diastolic function. Accumulation of intra-myocardial fibrosis and replacement scars underlies heart failure progression and represents a substrate for sustained arrhythmias in end-stage patients. However, arrhythmias and mechanical abnormalities may occur in hearts with little or no fibrosis, prompting toward functional pathomechanisms. By studying viable cardiomyocytes and trabeculae isolated from inter-ventricular septum samples of non-failing HCM patients with symptomatic obstruction who underwent myectomy operations, we identified that specific abnormalities of intracellular Ca2+ handling are associated with increased cellular arrhytmogenesis and diastolic dysfunction. In HCM cardiomyocytes, diastolic Ca2+ concentration is increased both in the cytosol and in the sarcoplasmic reticulum and the rate of Ca2+ transient decay is slower, while the amplitude of Ca2+-release is preserved. Ca2+ overload is the consequence of an increased Ca2+ entry via L-type Ca2+-current [due to prolongation the action potential (AP) plateau], combined with a reduced rate of Ca2+-extrusion through the Na+/Ca2+ exchanger [due to increased cytosolic (Na+)] and a lower expression of SERCA. Increased late Na+ current (INaL) plays a major role, as it causes both AP prolongation and Na+ overload. Intracellular Ca2+ overload determines an higher frequency of Ca2+ waves leading to delayed-afterdepolarizations (DADs) and premature contractions, but is also linked with the increased diastolic tension and slower relaxation of HCM myocardium. Sustained increase of intracellular [Ca2+] goes hand-in-hand with the increased activation of Ca2+/calmodulin-dependent protein-kinase-II (CaMKII) and augmented phosphorylation of its targets, including Ca2+ handling proteins. In transgenic HCM mouse models, we found that Ca2+ overload, CaMKII and increased INaL drive myocardial remodeling since the earliest stages of disease and underlie the development of hypertrophy, diastolic dysfunction and the arrhythmogenic substrate. In conclusion, diastolic dysfunction and arrhythmogenesis in human HCM myocardium are driven by functional alterations at cellular and molecular level that may be targets of innovative therapies.

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Section: Introduction: Arrhythmic Substrate In Hcm From Tissue To Thmentioning

confidence: 99%

Altered Ca2+ and Na+ Homeostasis in Human Hypertrophic Cardiomyopathy: Implications for Arrhythmogenesis

et al. 2018

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“…Although the current HCM risk‐SCD calculator is a practical scoring model developed to identify high‐risk patients and has been validated in adults, the prediction of SCD in HCM still remains challenging due to the heterogeneity of clinical expressions and the relatively low event rate observed in this disease. Apart from traditional risk factors, some novel imaging parameters on CMR have been recently studied for prognostic values as risk stratifiers of HCM 42,43 . However, cardiac evaluations by CMR are relatively high in cost, and are not so widely available in common medical institutions.…”

Section: Discussionmentioning

confidence: 99%

Correlations between cardiac troponin I and nonsustained ventricular tachycardia in hypertrophic obstructive cardiomyopathy

Liu

Shen

et al. 2020

Clinical Cardiology

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Background: Nonsustained ventricular tachycardia (NSVT) is an independent risk factor for sudden cardiac death (SCD) in patients with hypertrophic obstructive cardiomyopathy (HOCM). However, data concerning the correlations of cardiac biomarkers and NSVT in HOCM are rather limited. Hypothesis: Our study aimed to investigate the associations between the occurrence of NSVT and circulating biomarkers representing myocardial injury (cardiac troponin I, cTnI), cardiac function (N-terminal pro-brain natriuretic peptide, NT-pro BNP), and inflammation (high-sensitivity C-reactive protein) in a large Chinese HOCM cohort. Methods: A total of 755 consecutive HOCM patients were recruited. Systematic cardiac evaluations and circulating biomarkers were examined routinely in all subjects under the clinically stable status. According to the results of 24-hour Holter monitoring, patients were divided into the NSVT group (n = 138) and the nonventricular tachycardia (non-VT) group (n = 617). Results: Compared with the non-VT group, circulating levels of both cTnI and NT-pro BNP elevated significantly in patients with positive NSVT episodes (P < .001). Multivariable analyses demonstrated that cTnI was independently associated with the presence of NSVT (OR = 1.675, 95% CI: 1.406-1.994, P < .001). Concentrations of cTnI increased progressively not only with the aggravation of ventricular arrhythmic events (P < .001), but also with the growing risk of SCD in HOCM patients (P < .001). Serum cTnI ≥ 0.0265 ng/mL indicated predictive value for the occurrence of NSVT in the HOCM cohort (area under the curve = 0.707, 95% CI: 0.660-0.754, P < .001). Conclusions: Elevated cTnI was an independent determinant of NSVT, and it seemed to be valuable for assessing the clinical status of ventricular arrhythmias and the risk of SCD in patients with HOCM.

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“…Avanesov et al studied the ability of ECV to predict prognosis in HCM patients, and then compared it to other CMR and conventional risk markers. In 73 subjects affected by HCM followed-up for 5 years, ECV was proved to be a better predictor of SCD than LGE and performed similarly to HCM Risk-SCD score and better than LGE and post-contrast T1 mapping in predicting syncope or NSVT [ 54 ]. Moreover, an incremental value of combined analysis of HCM Risk-SCD score and ECV for prediction of syncope and NSVT was shown [ 54 ], underlining a possible role of routine ECV analysis in HCM patients to improve the patient selection for implantable cardioverter-defibrillator (ICD) implantation.…”

Section: Hypertrophic Cardiomyopathymentioning

confidence: 99%

Role of CMR Mapping Techniques in Cardiac Hypertrophic Phenotype

et al. 2020

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Non-ischemic cardiomyopathies represent a heterogeneous group of myocardial diseases potentially leading to heart failure, life-threatening arrhythmias, and eventually death. Myocardial dysfunction is associated with different underlying pathological processes, ultimately inducing changes in morphological appearance. Thus, classification based on presenting morphological phenotypes has been proposed, i.e., dilated, hypertrophic, restrictive, and right ventricular cardiomyopathies. In light of the key diagnostic and prognostic role of morphological and functional features, cardiovascular imaging has emerged as key element in the clinical workflow of suspected cardiomyopathies, and above all, cardiovascular magnetic resonance (CMR) represents the ideal technique to be used: thanks to its physical principles, besides optimal spatial and temporal resolutions, incomparable contrast resolution allows to assess myocardial tissue abnormalities in detail. Traditionally, weighted images and late enhancement images after gadolinium-based contrast agent administration have been used to perform tissue characterization, but in the last decade quantitative assessment of pre-contrast longitudinal relaxation time (native T1), post-contrast longitudinal relaxation time (post-contrast T1) and transversal relaxation time (T2), all displayed with dedicated pixel-wise color-coded maps (mapping), has contributed to give precious knowledge insight, with positive influence of diagnostic accuracy and prognosis assessment, mostly in the setting of the hypertrophic phenotype. This review aims to describe the available evidence of the role of mapping techniques in the assessment of hypertrophic phenotype, and to suggest their integration in the routine CMR evaluation of newly diagnosed cardiomyopathies with increased wall thickness.

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Prediction of the estimated 5-year risk of sudden cardiac death and syncope or non-sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy using late gadolinium enhancement and extracellular volume CMR

Cited by 60 publications

References 37 publications

Altered Ca2+ and Na+ Homeostasis in Human Hypertrophic Cardiomyopathy: Implications for Arrhythmogenesis

Altered Ca2+ and Na+ Homeostasis in Human Hypertrophic Cardiomyopathy: Implications for Arrhythmogenesis

Correlations between cardiac troponin I and nonsustained ventricular tachycardia in hypertrophic obstructive cardiomyopathy

Role of CMR Mapping Techniques in Cardiac Hypertrophic Phenotype

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