Usefulness of Inducible Ventricular Tachycardia to Predict Long-Term Adverse Outcomes in Arrhythmogenic Right Ventricular Cardiomyopathy

Saguner, Ardan M.; Medeiros-Domingo, Argelia; Schwyzer, Moritz A; On, Chol-Jun; Haegeli, Laurent; Wölber, Thomas; Hürlimann, David; Steffel, Jan; Krasniqi, Nazmi; Rüeger, Sina; Held, Leonhard; Lüscher, Thomas F.; Brunckhorst, Corinna; Duru, Fırat

doi:10.1016/j.amjcard.2012.09.025

Cited by 63 publications

(49 citation statements)

References 30 publications

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“…8,9,[12][13][14]28,36 Yet previous studies have yielded conflicting evidence regarding the usefulness of RV function and dimension for risk prediction in ARVC/D. [7][8][9][10][11][12][13][14][15]37 Controversial findings by others may be related to different study methodologies, divergent imaging modalities, and heterogeneous patient cohorts.…”

Section: Predictive Role Of Echocardiographic Variablesmentioning

confidence: 81%

Different Prognostic Value of Functional Right Ventricular Parameters in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Saguner

Vecchiati

Baldinger

et al. 2014

Circ: Cardiovascular Imaging

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A rrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited disease characterized by a progressive fibrofatty replacement mainly involving the RV myocardium. It is associated with ventricular arrhythmias, heart failure, and sudden cardiac death (SCD). 1-6Clinical Perspective on p 239 RV dilation, reduced RV systolic function, and diffuse RV involvement have previously been studied as predictors of an adverse outcome in patients with ARVC/D but have yielded conflicting results possibly because of divergent imaging modalities, varying parameters measured, and heterogeneous patient cohorts. [7][8][9][10][11][12][13][14][15] Data about the predictive role of RV fractional area change (FAC) in ARVC/D as measured by 2-dimensional transthoracic echocardiography (2D TTE) are scarce and controversial. 9,[14][15][16][17][18][19][20] Tricuspid annulus plane systolic excursion (TAPSE) can easily be determined by M-mode TTE. It is a robust parameter for

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Section: Predictive Role Of Echocardiographic Variablesmentioning

confidence: 81%

Different Prognostic Value of Functional Right Ventricular Parameters in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Saguner

Vecchiati

Baldinger

et al. 2014

Circ: Cardiovascular Imaging

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“…23 Inappropriate ICD therapy was defined as previously described. 10 For the purpose of this study, atrial arrhythmia was defined as AF and/or AFl that lasted ≥30 s. Episodes lasting <30 s were only included if they were terminated by an ICD shock.…”

Section: Outcome Measuresmentioning

confidence: 99%

Clinical Role of Atrial Arrhythmias in Patients With Arrhythmogenic Right Ventricular Dysplasia

Saguner

Ganahl

Kraus

et al. 2014

Circ J

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“…1,5 Several prognostic parameters have been associated with adverse outcomes in this entity, but risk stratification in patients with ARVC/ D needs further improvement. [6][7][8][9] It has been shown that increased exercise levels may raise the risk of ventricular arrhythmias in this disease. 10,11 Furthermore, gender has been suggested to play an important role.…”

Section: Introductionmentioning

confidence: 99%

Sex hormones affect outcome in arrhythmogenic right ventricular cardiomyopathy/dysplasia: from a stem cell derived cardiomyocyte-based model to clinical biomarkers of disease outcome

Akdiş¹,

Saguner²,

Shah

et al. 2017

European Heart Journal

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122

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Aims Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is characterized by fibrofatty infiltration of the myocardium and ventricular arrhythmias that may lead to sudden cardiac death. It has been observed that male patients develop the disease earlier and present with more severe phenotypes as compared to females. Thus, we hypothesized that serum levels of sex hormones may contribute to major arrhythmic cardiovascular events (MACE) in patients with ARVC/D. Methods and results The serum levels of five sex hormones, sex hormone-binding globulin, high sensitivity troponin T, pro-brain natriuretic peptide, cholesterol, triglycerides, insulin, and glucose were measured in 54 ARVC/D patients (72% male). Twenty-six patients (48%) experienced MACE. Total and free testosterone levels were significantly increased in males with MACE as compared to males with a favourable outcome, whereas estradiol was significantly lower in females with MACE as compared to females with a favourable outcome. Increased testosterone levels remained independently associated with MACE in males after adjusting for age, body mass index, Task Force criteria, ventricular function, and desmosomal mutation status. Furthermore, an induced pluripotent stem cell-derived ARVC/D cardiomyocyte model was used to investigate the effects of sex hormones. In this model, testosterone worsened and estradiol improved ARVC/D-related pathologies such as cardiomyocyte apoptosis and lipogenesis, strongly supporting our clinical findings. Conclusions Elevated serum testosterone levels in males and decreased estradiol levels in females are independently associated with MACE in ARVC/D, and directly influence disease pathology. Therefore, determining the levels of sex hormones may be useful for risk stratification and may open a new window for preventive interventions.

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Usefulness of Inducible Ventricular Tachycardia to Predict Long-Term Adverse Outcomes in Arrhythmogenic Right Ventricular Cardiomyopathy

Cited by 63 publications

References 30 publications

Different Prognostic Value of Functional Right Ventricular Parameters in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Different Prognostic Value of Functional Right Ventricular Parameters in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Clinical Role of Atrial Arrhythmias in Patients With Arrhythmogenic Right Ventricular Dysplasia

Sex hormones affect outcome in arrhythmogenic right ventricular cardiomyopathy/dysplasia: from a stem cell derived cardiomyocyte-based model to clinical biomarkers of disease outcome

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