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      Non-sustained Ventricular Tachycardia as a Presentation of Arrhythmogenic Right Ventricular Cardiomyopathy

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          Abstract

          Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare disorder with familial (autosomal dominant) predisposition and can be challenging to diagnose. Non-sustained ventricular tachycardia (NSVT) is a relatively uncommon and short-lived arrhythmia when seen in the general, healthy population. NSVT with a left bundle branch block morphology is usually idiopathic but may also be seen in ARVC. It can also be associated with poorer prognosis and increased mortality. Repetitive monomorphic ventricular ectopic beats may suggest ARVC, but could also be idiopathic. Timely diagnosis is vital due to the unpredictability and progressive nature of ARVC. We present a case of a 40-year-old Caucasian female with heart palpitations and NSVT found on an outpatient Holter monitor, and later found to have clinical and radiological features consistent with ARVC.

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          2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy

          Jeffrey A. Towbin, William J. McKenna, Dominic J. Abrams … (2019)
          Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
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            Right ventricular dysplasia: a report of 24 adult cases.

            F Marcus, G H Fontaine, G. Guiraudon … (1982)
            Right ventricular dysplasia is characterized by an abnormality in the development of part of the right ventricular musculature. Patients with right ventricular dysplasia may present with ventricular tachycardia, supraventricular arrhythmias, right-heart failure or asymptomatic cardiomegaly. Twenty-two adult patients with right ventricular dysplasia who had recurrent ventricular tachycardia were seen during a 7-year period. The male/female ratio was 2.7:1. The mean age at the time of hospitalization was 39 years. All but one of the patients had ventricular tachycardia of a left bundle branch block configuration. With few exceptions, the T waves were inverted over the right precordial leads. The heart was usually enlarged and the pulmonary vasculature was usually normal. In six patients who had two-dimensional echocardiograms, all showed increased right ventricular diastolic dimensions. All patients had right ventricular angiography; the diagnosis of right ventricular dysplasia was substantiated during surgery in 12 patients and at autopsy in another. Two other patients who did not have arrhythmias had right ventricular dysplasia diagnosed by right- and left-heart angiography. Our unique experience, when combined with a literature review of 34 adult cases, permits a composite clinical profile of this condition in the adult.
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              Evolving Diagnostic Criteria for Arrhythmogenic Cardiomyopathy

              Domenico Corrado, Alessandro Zorzi, Alberto Cipriani … (2021)
              Criteria for diagnosis of arrhythmogenic cardiomyopathy (ACM) were first proposed in 1994 and revised in 2010 by a Task Force. Although the Task Force criteria demonstrated a good accuracy for diagnosis of the original right ventricular phenotype (arrhythmogenic right ventricular cardiomyopathy), they lacked sensitivity for identification of the expanding phenotypic spectrum of ACM, which includes left‐sided variants and did not incorporate late‐gadolinium enhancement findings by cardiac magnetic resonance. The 2020 International criteria (“Padua criteria”) have been developed by International experts with the aim to improve the diagnosis of ACM by providing new criteria for the diagnosis of left ventricular phenotypic features. The key upgrade was the incorporation of tissue characterization findings by cardiac magnetic resonance for noninvasive detection of late‐gadolinium enhancement/myocardial fibrosis that are determinants for characterization of arrhythmogenic biventricular and left ventricular cardiomyopathy. The 2020 International criteria are heavily dependent on cardiac magnetic resonance, which has become mandatory to characterize the ACM phenotype and to exclude other diagnoses. New criteria regarding left ventricular depolarization and repolarization ECG abnormalities and ventricular arrhythmias of left ventricular origin were also provided. This article reviews the evolving approach to diagnosis of ACM, going back to the 1994 and 2010 International Task Force criteria and then grapple with the modern 2020 International criteria.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Cureus
                Journal ID (iso-abbrev): Cureus
                Journal ID (issn): 2168-8184
                Title: Cureus
                Publisher: Cureus (Palo Alto (CA) )
                ISSN (Electronic): 2168-8184
                Publication date (Electronic, pub): 6 May 2023
                Publication date (Electronic, collection): May 2023
                Volume: 15
                Issue: 5
                Electronic Location Identifier: e38620
                Affiliations
                [1 ] Internal Medicine, NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, USA
                [2 ] Cardiac Electrophysiology, NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, USA
                Author notes
                Article
                DOI: 10.7759/cureus.38620
                PMC ID: 10240442
                SO-VID: c064e8ad-1d6b-418a-93f4-aba40e9b5bda
                Copyright © Copyright © 2023, Amin et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date accepted : 5 May 2023
                Categories
                Subject: Cardiology
                Subject: Internal Medicine

                Keywords: arvd,nsvt,atrial tachyarrhythmia,non-sustained ventricular tachycardia,idiopathic dilated cardiomyopathy,arrhythmogenic right ventricular dysplasia
                Data availability:
                Keywords: arvd, nsvt, atrial tachyarrhythmia, non-sustained ventricular tachycardia, idiopathic dilated cardiomyopathy, arrhythmogenic right ventricular dysplasia

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