How is ARVD/C diagnosed?

ARVD/C is diagnosed through a scoring system consisting of major and minor criteria.3 Factors taken into account include ventricular dysfunction and structural alterations, tissue characterization, electrocardiographic repolarization/depolarization abnormalities, ventricular arrhythmias, family history, and genetic testing.3 A thorough initial evaluation should be conducted (see Table 2).

Individuals presenting without known heart disease but with specific arrhythmias should be screened for ARVD/C (see Table 3) and other potential diagnoses should be ruled out (see Table 4).1

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What is the next step following diagnosis?

Preventing SCD is the most important goal of management, so once the diagnosis has been established, the next step is to stratify patients at risk for SCD and determine if they would benefit from an ICD (Table 5).3 Since ICDs carry risks and side effects, we target their use to high-risk individuals. So if the risk of SCD is, for example, 1:1000 per year or less, it is hard to justify the use of an ICD, because the risks outweigh the benefits. Once the risk of SCE gets to 1:100 or higher, the risk of ICD-related side effects becomes justified.

Are medical alternatives available to patients who do not receive an ICD?

The most commonly used and recommended agents arebeta-blockers, which reduce risk of SCD in patients with ARVD/C. Isoproterenol can be proarrhythmic in these patients and should be avoided.1 Angiotensin-converting enzyme (ACE) inhibitors are sometimes used as afterload reducers.1,3

Does catheter ablation play a role in management of ARVD/C?

Catheter ablation can ameliorate symptoms and improve quality of life, but is not curative. It is recommended for patients with incessant ventricular tachycardia (VT) or with frequent appropriate ICD interventions, despite maximal pharmacologic therapies.3

What role does exercise restriction play in ARVD management?

Exercise restriction is a critical intervention. Exercise increases the pressure in both the LV and RV, but especially in the RV. When the RV dilates, the radius expands, placing further stress on an already weakened myocardial wall. Exercise is the most important environmental trigger of the clinical manifestations of ARVD/C.3

A person with the ARVD/C mutation who does not engage in vigorous exercise, such as competitive athletics or marathons, is unlikely to suffer SCD or to show other clinical signs of the disease. The American Heart Association and American College of Cardiology (AHA/ACC) recommends avoidance of all intense physical activities for patients with probable, borderline, or definite diagnosis of ARVD/C, except for sports with low cardiovascular demand.5