XXXX in Tuscany: clinical course and outcome in an unselected regional population.

@entity1 @entity1164 @entity712 @entity1163 @entity955 @entity204 @entity1121 @entity65
OBJECTIVES: Our aim was to study a population of @entity1 with @entity1121 from the well defined geographic region of Tuscany in central Italy, a group virtually free of selective referral bias and therefore probably closely representative of the true @entity1 population with this disease. BACKGROUND: Most available information on clinical course, natural history and prognosis of @entity1121 is based on data generated from tertiary referral centers and therefore constitutes a potentially biased perspective of the disease process in this complex and diverse condition. METHODS: The study group comprised 202 @entity1 aged 1 to 74 years (mean +/- SD 41 +/- 17) at initial diagnosis and followed up for 1 to 30 years (mean 10 +/- 5). RESULTS: Largely with the use of single or multiple drug therapy, the vast majority of @entity1 (n = 154 [76%]) were asymptomatic or mildly symptomatic and in stable or improved condition over the period of follow-up, whereas the remaining @entity1 (n = 48 [24%]) experienced deterioration, had substantial functional impairment or died. Of the 13 @entity1 (6%) who died of cardiovascular causes related to @entity1121 , 11 had progressive @entity712 (including 6 in the end-stage phase) and only 2 died suddenly. The annual mortality rate for @entity65 was 0.6% and that due to @entity1164 was only 0.1%; the cumulative survival rate was 97%, 95% and 92%, respectively, at 5, 10 and 15 years of follow-up. @entity955 proved to be a relatively common (n = 57 [28%]) and particularly unfavorable clinical feature, with @entity204 occurring in 9 of the 57 @entity1 . The cumulative survival rate after 15 years was 76% for @entity1 with @entity955 versus 97% for @entity1 with sinus rhythm. @entity1163 occurred in 33 @entity1 (16%) but did not appear to be of prognostic significance. CONCLUSIONS: In an unselected regional population, @entity1121 had a relatively benign prognosis inconsistent with its prior characterization as a generally progressive disorder, based primarily on the experience of selected referral institutions. Sudden unexpected @entity204 was distinctly uncommon, although a sizable proportion of @entity1 (particularly the subset prone to @entity955 ), did experience clinical deterioration.
