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


METHODS
Retrospective and partly prospective observational study.


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


METHODS
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.


METHODS
Diagnosis, management, morbidity and mortality were analysed.


RESULTS
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.


CONCLUSIONS
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.
Munclinger
M J
MJ
Cardiac Unit, Wentworth Hospital, Durban.
Patel
J J
JJ
Mitha
A S
AS
D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D003937:Diagnosis, Differential; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies