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Arrhythmogenic right ventricular cardiomyopathy

What's new

Added 2023 ESC, 2022 AHA/ACC, and 2017 CCS guidelines for the diagnosis and management of arrhythmogenic right ventricular cardiomyopathy.

Background

Overview

Definition
ARVC is a genetically inherited cardiomyopathy characterized by the replacement of normal heart muscle with fibrofatty tissue, predominantly in the right ventricle.
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Pathophysiology
ARVC is caused by the fibrofatty replacement of the myocardium predisposing the heart to electrical instability, leading to ventricular arrhythmias.
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Epidemiology
The prevalence of ARVC worldwide is estimated at 20 per 100,000 population.
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Disease course
Clinically, ARVC can present with a variety of symptoms. The most common presenting symptoms include palpitations, syncope, and SCD. Some patients may also develop signs of HF.
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Prognosis and risk of recurrence
The prognosis of ARVC is variable. It is associated with an increased risk of SCD, particularly in young individuals, and may be the first manifestation of the disease.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of arrhythmogenic right ventricular cardiomyopathy are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2023,2022,2018), the Heart Failure Society of America (HFSA/AHA/ACC 2022), the Canadian Cardiovascular Society (CCS/CHRS 2019), the American Heart Association (AHA/HRS/ACC 2018,2017), and the Canadian Cardiovascular Society (CCS 2017). ...
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Screening and diagnosis

Screening of family relatives, clinical and genetic testing: as per ESC 2023 guidelines, obtain cascade genetic testing, with pre- and post-test counseling, in adult at-risk relatives of a patient with cardiomyopathy with a confident genetic diagnosis (a pathogenic/likely pathogenic variant) in the family (starting with first-degree relatives, if available, and cascading out sequentially).
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  • Screening of family relatives (cardiac MRI)

  • Screening of family relatives (follow-up)

Diagnostic investigations

General principles: as per ESC 2023 guidelines, obtain systematic evaluation in all patients with suspected or established cardiomyopathy using a multiparametric approach, including clinical evaluation, pedigree analysis, ECG, Holter monitoring, laboratory tests, and multimodality imaging.
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  • History and physical examination

  • ECG

  • TTE

  • Cardiac MRI

  • Cardiac CT

  • Nuclear imaging

  • Laboratory tests

  • Genetic testing

Diagnostic procedures

Electrophysiological study: as per ESC 2022 guidelines, consider performing programmed electrical stimulation for risk stratification in patients with ARVC and symptoms highly suspicious for ventricular arrhythmia.
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  • Endomyocardial biopsy

Medical management

General principles
As per ESC 2023 guidelines:
Ensure that all patients with cardiomyopathy and their relatives have access to multidisciplinary teams with expertise in the diagnosis and management of cardiomyopathies.
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Identify and manage risk factors and concomitant diseases as an integral part of the management of patients with cardiomyopathy.
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  • Antiarrhythmic drugs

  • Management of AF (anticoagulation)

  • Management of AF (catheter ablation)

Nonpharmacologic interventions

Physical activity restrictions: as per ESC 2023 guidelines, advise practicing regular low-to-moderate-intensity exercise in all able patients with cardiomyopathy.
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  • Psychological support

Therapeutic procedures

ICD, indications: as per ESC 2023 guidelines, obtain comprehensive SCD risk stratification in all patients with cardiomyopathy not suffered a previous cardiac arrest/sustained ventricular arrhythmia at initial evaluation and at 1-2 year intervals or whenever a change in clinical status occurs.
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  • ICD (technical considerations)

  • Catheter ablation (ventricular arrhythmia)

  • Catheter ablation (AF)

Perioperative care

Perioperative assessment: as per ESC 2023 guidelines, obtain perioperative ECG monitoring in all patients with cardiomyopathy undergoing noncardiac surgery.
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Surgical interventions

LV assist device
As per ESC 2023 guidelines:
Consider initiating mechanical circulatory support therapy in selected patients with cardiomyopathy with advanced HF (NYHA class III-IV) despite optimal pharmacological and device treatment, otherwise suitable for heart transplantation, to improve symptoms and reduce the risk of HF hospitalization from worsening HF and premature death while awaiting a transplant.
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Consider initiating mechanical circulatory support therapy in selected patients with cardiomyopathy with advanced HF (NYHA class III-IV) despite optimal pharmacological and device therapy, ineligible for cardiac transplantation or other surgical options, and without severe RV dysfunction, to reduce the risk of death and improve symptoms.
C

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  • Heart transplantation

Specific circumstances

Pregnant patients: as per ESC 2023 guidelines, obtain prenatal diagnostic testing early in pregnancy, if it is to be pursued by the family, to allow making decisions regarding continuation or coordination of pregnancy.
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Patient education

Genetic counseling
As per ESC 2023 guidelines:
Provide genetic counseling by an appropriately trained healthcare professional, including genetic education, to inform decision-making and psychosocial support in families with an inherited or suspected inherited cardiomyopathy, regardless of whether genetic testing is being considered.
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Provide pre- and post-test genetic counseling in all individuals undergoing genetic testing for cardiomyopathy.
B

Follow-up and surveillance

Follow-up: as per ESC 2023 guidelines, obtain routine follow-up using a multiparametric approach with ECG and echocardiography every 1-2 years in all clinically stable patients with cardiomyopathy.
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  • Transition to adult care