Natural history

There is a variable but typically long asymptomatic lead-time in individuals with ARVC. While this is a genetically transmitted disease, individuals in their teens may not have any characteristics of ARVC on screening tests. ARVC is reported in the childhood years, but is uncommon.

Many individuals have symptoms associated with ventricular tachycardia, such as palpitations, light-headedness, or syncope. Others may have symptoms and signs related to right ventricular failure, such as lower extremity edema, liver congestion with elevated hepatic enzymes. Unfortunately, sudden death may be the first manifestation of disease.

ARVC is a progressive disease. Because moderate and even severe right ventricular dysfunction is minimally symptomatic, arrhythmias are often the only symptomatic presentation in ARVC patients. Over time, the right ventricle becomes more involved, variably leading to right ventricular failure. Typically, the right ventricle will fail before there is left ventricular dysfunction. However, by the time the individual has signs of overt right ventricular failure, there is often histological involvement of the left ventricle. Eventually, the left ventricle may also become involved, leading to bi-ventricular failure. Signs and symptoms of left ventricular failure may become evident, including congestive heart failure, atrial fibrillation, and an increased incidence of thromboembolic events.

The dominant goal of management of ARVC is to decrease the incidence of sudden cardiac death. This raises a clinical dilemma: How to prophylactically treat the asymptomatic patient who was diagnosed during family screening.

A certain subgroup of individuals with ARVC are considered at high risk for sudden cardiac death. Characteristics associated with high risk of sudden cardiac death include: