Follow-up of patients with arrhythmogenic right ventricular cardiomyopathy dysplasia

The enlargement of data on the natural course and management of patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D).

Retrospective and partly prospective observational study.

Cardiac Unit, Wentworth Hospital, Durban--the only unit in KwaZulu-Natal providing an arrhythmia and electrophysiology service.

Those included were: (i) patients referred for palpitations, unexplained syncope, or ventricular tachycardia and in whom ARVC/D was diagnosed according to multiple criteria; and (ii) family members of patients with ARVC/D in whom the disease was documented using the same criteria.

Diagnosis, management, morbidity and mortality were analysed.

Twelve patients were diagnosed with ARVC/D over a period or 6 years. At the end of follow-up for 3.4 +/- 3.2 years, 7 of them were well and alive on anti-arrhythmic medication, 2 were asymptomatic, and 3 had died. One death was sudden, 1 patient died due to left ventricular failure, and 1 patient died due to a low cardiac output syndrome 3 months after right ventricular isolation, i.e. the mortality rate was 25%. ARVC/D was found in all racial groups and was familial in 5 patients (42%). In all but one patient the correct diagnosis was not suspected by the referring institution, physician or cardiologist.

ARVC/D needs to be included into a differential diagnosis of unexplained syncope, palpitations, or ventricular tachycardia by all health service providers. Its management remains a complex challenge with varying results.