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3 years ago
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterised by progressive replacement of the ventricular myocardium by fibrofatty tissue.1 Patients with the disease are predisposed to ventricular arrhythmias, heart failure and sudden cardiac death.
Pathophysiology
ARVC has a strong genetic basis with most disease variants displaying an autosomal dominant…
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3 years ago
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a rare heritable cardiomyopathy characterised by fibro-fatty replacement of the myocardium, which predisposes patients to frequent lifethreatening ventricular arrhythmias and slowly progressive ventricular dysfunction.1,2 Structural involvement of the right ventricle (RV) generally predominates,3,4 although left dominant forms…
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3 years ago
Arrhythmogenic cardiomyopathy (ACM) is usually referred to as arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D).1A first historical description was made in 1736, whereas its first modern description dates back to 1982.2 Initially, ACM was thought to be an embryological malformation.3 Yet in recent years it became evident that the pathophysiology of an ongoing genetically…
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3 years ago
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3 years ago
With an ageing population and widespread use of implantable cardioverter-defibrillators, physicians are confronted with an increasing number of patients with symptomatic, drug-refractory ventricular tachycardia (VT). Catheter ablation is an important treatment option in the management of patients with structural heart disease and VT.1,2 In many patients, VT can be successfully ablated from the…
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3 years ago
Ventricular arrhythmias remain a major contributor to cardiac morbidity and mortality worldwide, despite ongoing research and implementation of novel therapeutic interventions. Modern management of patients with ventricular arrhythmias requires a multidisciplinary team approach, especially in complex presentations with a background of multiple medical comorbidities.1,2 Such teams may include…
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3 years ago
Monomorphic ventricular arrhythmias (MMVA) are not uncommon in athletes,1,2 yet their presence appropriately raises concern among practitioners for possible increased risk of sudden cardiac death (SCD) during sports activity and competition. While all MMVA detected in athletes warrant further evaluation,1 a majority of MMVA in this population are likely to be benign. In some instances of so…
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3 years ago
Early repolarisation (ER) is defined as J-point elevation of ≥0.1 mV in two adjacent leads with either a slurred or notched morphology (Figures 1 to 4).1,2 Numerous studies have established an association with ER and increased risk of death and idiopathic ventricular fibrillation (VF).1–5 Clinicians face questions such as patient and family counselling, quantitifying the risk of sudden cardiac…
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3 years ago
An estimated 180,000–300,000 sudden cardiac deaths (SCD) occur in the US annually.1,2 Worldwide, sudden and unexpected cardiac death is the most common cause of death,2 accounting for 17 million deaths every year with SCD accounting for 25% of these. The accepted definition of SCD is death that occurs within one hour of onset of symptoms in witnessed cases, and within 24 hours of last being seen…
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3 years ago
Catheter ablation has been increasingly used as a treatment for refractory ventricular tachycardia (VT) in patients with non-ischaemic cardiomyopathy (NICM). However, ablation outcomes tend to be quite variable because of the heterogeneity of the aetiology for the NICM and associated VT substrate in these patients.1–3 Patients with NICM can be sub-classified based on specific genotypic and…
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