Prevalence of right ventricular dysplasia-cardiomyopathy in a non-referral hospital
Introduction
Arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) is a pathologically and histologically well-defined cardiac entity [1]. The frequency of familial disease is estimated to be 30% [2]. In referral centers, the prevalence is about 1 in 5000–10,000 inhabitants, although in regions with intensive family screening such as the Veneto region this disease appears to be much more common [3].
Ventricular tachyarrhythmias, syncope and sudden cardiac death are leading symptoms and in a younger population sustained ventricular tachycardia in ARVD/C is one of the main reasons for electrophysiological studies [4]. The problem of sudden arrhythmic death is often seen in young adolescents and predominantly described in competitive athletes [5]. Little is known about the prevalence, clinical symptoms and prognosis of ARVD/C in non-referral hospitals.
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Methods
Since 1997, the Cardiological Department of the Academic Teaching Hospital in Quedlinburg is responsible for 80,000 inhabitants as the only hospital in the county with 2500 in-hospital patients per year and 1500 out-hospital patients seen by only one cardiologist (S.P.) with the possibility of extensive non-invasive examinations and a catheter laboratory for invasive and interventional cardiology.
The prevalence of patients with ARVD/C living in the county of Quedlinburg was retrospectively
Results
From 1997 to 2002, ARVD/C was diagnosed in 35 female and 45 male non-familial cases (overall prevalence 1 in 1000 inhabitants) with a mean age of 45.6 years (range: 22–91 years) in the Academic Teaching Hospital of Quedlinburg.
First degree relatives were examined in 12 cases with familial predisposition in all cases with up to 3 additional affected family members. In three screened families, there was a history of unexplained sudden cardiac death at younger age.
Symptoms in patients with ARVD/C
Discussion
Very little is known about the prevalence, symptoms and prognosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy in non-referral hospitals. From centers that perform systematic family screening, one can suppose that the prevalence of ARVD/C is higher than expected. From the Veneto region in Italy, the expected prevalence is higher than 6 in 10,000 inhabitants [1], [3]. In general, difficulties in defining the true prevalence are due to familial cases without clinical symptoms or
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