Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy: Progress and Pitfalls

Oomen, A. W. G. J.; Semsarian, Christopher; Puranik, Rajesh; Sy, Raymond W.

doi:10.1016/j.hlc.2018.03.023

Cited by 19 publications

(24 citation statements)

References 32 publications

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“…ISP helps in normalizing the diminished heartbeat. However, prolonged use of ISP could also lead to change in the left and right ventricular wall function and physiology inducing cardiomyopathy [42]. Therefore, in our present study, we employed ISP for inducing CH in the rat cardiomyocyte (H9C2) cell lines, through the process of oxidative stress-induced damage.…”

Section: Discussionmentioning

confidence: 97%

Application of Zebrafish Model in the Suppression of Drug-Induced Cardiac Hypertrophy by Traditional Indian Medicine Yogendra Ras

et al. 2020

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Zebrafish is an elegant vertebrate employed to model the pathological etiologies of human maladies such as cardiac diseases. Persistent physiological stresses can induce abnormalities in heart functions such as cardiac hypertrophy (CH), which can lead to morbidity and mortality. In the present study, using zebrafish as a study model, efficacy of the traditional Indian Ayurveda medicine “Yogendra Ras” (YDR) was validated in ameliorating drug-induced cardiac hypertrophy. YDR was prepared using traditionally described methods and composed of nano- and micron-sized metal particles. Elemental composition analysis of YDR showed the presence of mainly Au, Sn, and Hg. Cardiac hypertrophy was induced in the zebrafish following a pretreatment with erythromycin (ERY), and the onset and reconciliation of disease by YDR were determined using a treadmill electrocardiogram, heart anatomy analysis, C-reactive protein release, and platelet aggregation time-analysis. YDR treatment of CH-induced zebrafish showed comparable results with the Standard-of-care drug, verapamil, tested in parallel. Under in-vitro conditions, treatment of isoproterenol (ISP)-stimulated murine cardiomyocytes (H9C2) with YDR resulted in the suppression of drug-stimulated biomarkers of oxidative stress: COX-2, NOX-2, NOX-4, ANF, troponin-I, -T, and cardiolipin. Taken together, zebrafish showed a strong disposition as a model for studying the efficacy of Ayurvedic medicines towards drug-induced cardiopathies. YDR provided strong evidence for its capability in modulating drug-induced CH through the restoration of redox homeostasis and exhibited potential as a viable complementary therapy.

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Section: Discussionmentioning

confidence: 97%

Application of Zebrafish Model in the Suppression of Drug-Induced Cardiac Hypertrophy by Traditional Indian Medicine Yogendra Ras

et al. 2020

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“…Rare mutations in TMEM43, transmembrane protein 43, located in nucleus inner membrane, are associated with severe ACM phenotypes, 120 high penetrance and high SCD risk. 111,121,122 This gene contains a response element for PPARγ (an adipogenic transcription factor), which may explain the fibrofatty replacement of the myocardium, a characteristic pathological finding in ACM. 123…”

Section: Arrhythmogenic Ventricular Cardiomyopathymentioning

confidence: 99%

Sudden Cardiac Death in Young Athletes: Literature Review of Molecular Basis

et al. 2020

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Intense athletic training and competition can rarely result in sudden cardiac death (SCD). Despite the introduction of pre-participation cardiovascular screening, especially among young competitive athletes, sport-related SCD remains a debated issue among medical personnel, sports communities and laypersons alike, and generates significant media attention. The most frequent cause of SCD is a hidden inherited cardiomyopathy, the athletes may not even be aware of. Predictive medicine, by searching the presence of pathogenic alterations in cardiac genes, may be an integrative tool, besides the conventional ones used in cardiology (mainly electro and echocardiogram), to reach a definitive diagnosis in athletes showing signs/symptoms, even borderline, of inherited cardiomyopathy/ channelopathy, and in athletes presenting family history of SCD and/or of hereditary cardiac disease. In this review, we revised the molecular basis of the major cardiac diseases associated to sudden cardiac death and the clinical molecular biology approach that can be used to perform risk assessment at DNA level of sudden cardiac death, contributing to the early implementation of adequate therapy. Alterations can occur in ion channel genes, in genes encoding desmosomal and junctional proteins, sarcomeric and Z-disc proteins, proteins for the cytoskeleton and the nuclear envelope. The advent of next generation sequencing (NGS) technology has provided the means to search for mutations in all these genes, at the same time. Therefore, this molecular approach should be the preferred methodology for the aforementioned purpose.

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“…Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by myocardial atrophy with fibro-fatty replacement, eventually resulting in wall thinning and cardiac dilatation. The inflow tract, outflow tract and apex of the right ventricle constitute the so-called “triangle of dysplasia” (Oomen et al, 2018 ). ARVC manifests with myocardial inflammation, right ventricular aneurysms, and ventricular tachyarrhythmias, which represents one of the leading causes of sudden death in the young and in athletes (Basso et al, 2018 ).…”

Section: Arrhythmogenic Right Ventricular Cardiomyopathymentioning

confidence: 99%

SCN5A Variants: Association With Cardiac Disorders

et al. 2018

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The SCN5A gene encodes the alpha subunit of the main cardiac sodium channel Nav1.5. This channel predominates inward sodium current (INa) and plays a critical role in regulation of cardiac electrophysiological function. Since 1995, SCN5A variants have been found to be causatively associated with Brugada syndrome, long QT syndrome, cardiac conduction system dysfunction, dilated cardiomyopathy, etc. Previous genetic, electrophysiological, and molecular studies have identified the arrhythmic and cardiac structural characteristics induced by SCN5A variants. However, due to the variation of disease manifestations and genetic background, impact of environmental factors, as well as the presence of mixed phenotypes, the detailed and individualized physiological mechanisms in various SCN5A-related syndromes are not fully elucidated. This review summarizes the current knowledge of SCN5A genetic variations in different SCN5A-related cardiac disorders and the newly developed therapy strategies potentially useful to prevent and treat these disorders in clinical setting.

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Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy: Progress and Pitfalls

Cited by 19 publications

References 32 publications

Application of Zebrafish Model in the Suppression of Drug-Induced Cardiac Hypertrophy by Traditional Indian Medicine Yogendra Ras

Application of Zebrafish Model in the Suppression of Drug-Induced Cardiac Hypertrophy by Traditional Indian Medicine Yogendra Ras

Sudden Cardiac Death in Young Athletes: Literature Review of Molecular Basis

SCN5A Variants: Association With Cardiac Disorders

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