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Update on arrhythmogenic ­cardiomyopathies

The last two decades have substantially improved our understanding of ACM from a purely RV dysplasia to an inherited polygenic disease with a broad phenotypic spectrum potentially affecting both ventricles, being aggravated by environmental factors such as endurance sports. Emerging technologies in imaging, genetics, catheter ablation and device therapy have facilitated diagnosis, risk stratification and therapy of ACM, and prevention of SCD. Future challenges will involve early identification of asymptomatic patients and family members, improved risk stratification, and developing causal therapies to cure this challenging disease.

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 What is arrhythmogenic cardiomyopathy?

Arrhythmogenic cardiomyopathy, abbreviated as ACM, is a heart muscle disease with an increased risk of life-threatening arrhythmias (heart rhythm abnormalities) and a progressively decreasing pump function of the heart. Patients with ACM are often young and physically active, but this isn’t essential for disease development. The medical terminology is very descriptive: ‘arrhythmogenic’ means ‘abnormal rhythm’ and ‘cardio-myo-pathy’ means heart muscle disease. ACM is also known as ARVC (arrhythmogenic right ventricular cardiomyopathy): in the classical disease presentation of ACM the disease starts on the right side of the heart (the right ventricle).

Articles of Interest

Arrhythmogenic Cardiomyopathy as a Late Complication of COVID-19-Induced Myocarditis

As myocarditis induced by SARS-CoV-2 infection can evolve to chronic cardiomyopathy, all efforts must be made for accurate diagnosis.

Arrhythmogenic Cardiomyopathy: A Disease or Merely a Phenotype?

Arrhythmogenic cardiomyopathy (AC) is a clinical entity that has evolved conceptually over the past 30 years. Advances in cardiac imaging and the introduction of genetics into everyday practice have revealed that AC comprises multiple phenotypes that are dependent on genetic or acquired factors.

Arrhythmogenic Cardiomyopathy—Current Treatment and Future Options

Arrhythmogenic cardiomyopathy (ACM) is an inheritable heart muscle disease characterised pathologically by fibrofatty myocardial replacement and clinically by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). Although, in its original description, the disease was believed to predominantly involve the right ventricle, biventricular and left-dominant variants, in which the myocardial lesions affect in parallel or even mostly the left ventricle, are nowadays commonly observed. The clinical management of these patients has two main purposes: the prevention of SCD and the control of arrhythmic and heart failure (HF) events. An implantable cardioverter defibrillator (ICD) is the only proven lifesaving treatment...

Arrhythmogenic Right Ventricular Cardiomyopathy in Pediatric Patients: An Important but Underrecognized Clinical Entity

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by fibrofatty infiltration of predominantly the right ventricular (RV) myocardium. Affected patients typically present as young adults with hemodynamically stable ventricular tachycardia, although pediatric cases are increasingly recognized.

Arrhythmogenic Right Ventricular Cardiomyopathy – end stage

Epsilon wave ECG...

Arrhythmogenic Right Ventricular Dysplasia (ARVD)

Second most common cause of sudden cardiac death in young people (after HOCM), accounting for up to 10% of sudden cardiac deaths in patients < 65 yrs of age. Prevalence ~ 1 in 5000. Diagnosis is difficult and relies on a combination of clinical, electrocardiographic and radiological features, as defined by the (horribly complicated) 2010 Task Force Criteria.

Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria

The original designation of “Arrhythmogenic right ventricular (dysplasia/) cardiomyopathy”(ARVC) was used by the scientists who first discovered the disease, in the pre-genetic and pre-cardiac magnetic resonance era, to describe a new heart muscle disease predominantly affecting the right ventricle, whose cardinal clinical manifestation was the occurrence of malignant ventricular arrhythmias.

Resources

Netherlands ACM Registry

The ‘Netherlands ACM Registry’ is a project of the Netherlands Heart Institute. This organization, which was founded in 1972, aims to conduct scientific research on heart diseases by combining the strength of all 8 teaching hospitals in the Netherlands.

Cardiomyopathy UK

ACM has been referred to in the past as Arrhythmogenic Right Ventricular Cardiomyopathy as it was initially thought that it exclusively affected this pumping chamber of the heart.

Children's Cardiomyopathy Foundation

Arrhythmogenic cardiomyopathy (AC), also known as arrhythmogenic right ventricular cardiomyopathy, arrhythmogenic right ventricular dysplasia, right ventricular dysplasia, and right ventricular cardiomyopathy, is the least common type of cardiomyopathy in children.

SADS Foundation

ARVC is caused by a defect in the tiny proteins that hold the heart muscle cells together. These complex proteins, or “desmosomes,” can be disrupted in ARVC, leading to areas of scar and fat deposits. The right ventricle is more susceptible to this damage, though the left ventricle can also be involved. These areas of scarring can lead to abnormal heart rhythms which can cause you to become symptomatic or cause sudden cardiac arrest or death.