Survival is good in diagnosed and treated ARVC

19th June 2015

A seven year study of people with arrhythmogenic right ventricular cardiomyopathy (ARVC) and their families has shown that prognosis is good in those who have been diagnosed and treated.

The study, led by Dr AJ Groenweg, from the University Medical Centre in Utrecht, Netherlands, looked at over 1,000 people - 439 people with ARVC and 562 family members.

During follow up 72 per cent of the initial patients had potentially dangerous heart rhythms but researchers reported that “overall cardiac mortality and the need for heart transplant was low (six per cent and four per cent)”.

Those who died suddenly were much less likely to have an ICD (implantable cardioverter defibrillator).

ARVC, like most of the cardiomyopathies, usually runs in families.

Gene mutations potentially causing the disease was identified in 276 of initial patients but the symptoms and progress of patients and family members were similar in those with and without identified mutations.

One third of the family members developed ARVC. Family members with identified gene mutations were more likely to have symptoms than family members without recognised mutations.

The study, reported in Circulation: Cardiovascular Genetics, concluded that “Long-term outcome was favourable in diagnosed and treated patients and family members. Outcome in index-patients was modulated by implantable cardioverter-defibrillator implantation, but not by mutation status and familial background of disease. Outcome in [affected] family members was determined by symptoms at first evaluation and mutations.”

Ref: Clinical Presentation, Long-Term Follow-Up, and Outcomes of 1001 Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Patients and Family Members.

For more information about ARVC.