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G Ital Cardiol. 1997 Nov;27(11):1133-43.

[Clinical course of hypertrophic cardiomyopathy in a non selected population. The Experience of the Italian Multicenter Cardiomyopathy Study].

[Article in Italian]

Author information

  • 1Unità di Cardiologia, S. Luca Azienda Ospedaliera Careggi, Firenze.

Abstract

BACKGROUND:

Most of the information available on the clinical course and prognosis of hypertrophic cardiomyopathy (HCM) is based on data generated from international referral centres and as a result, it constitutes a potentially biased perspective of the disease process in this complex and diverse condition. A multicentric study was therefore set up with the aim of providing information on unselected patient populations with HCM.

METHODS:

The study group comprised 330 patients from 5 non-referral hospitals (mean age 42 +/- 16 years, M/F 226/104, 74-22%-obstructive, 299-91%-in NYHA class I-II) who were followed up regularly for 9.5 +/- 5.6 years.

RESULTS:

The vast majority of patients (n = 272, 82%) remained asymptomatic or mildly symptomatic during the follow-up period, whereas the remaining patients (n = 58, 18%) experienced clinical deterioration or died. Of the 18 patients (5%) who died of cardiovascular causes related to hypertrophic cardiomyopathy, 14 had progressive congestive heart failure and only 4 died suddenly. The annual mortality rate for cardiovascular disease was 0.57%, while the mortality rate due to sudden cardiac death was only 0.1%. The cumulative survival rate was 98, 95 and 93%, at 5, 10 and 15 years of follow-up respectively. Atrial fibrillation proved to be a relatively common (n = 81, 24%) and particularly unfavourable clinical feature, with higher mortality rate for cardiovascular causes related to hypertrophic cardiomyopathy. Syncope occurred in 47 patients (14%) but did not appear to have prognostic significance.

CONCLUSIONS:

In an unselected population, hypertrophic cardiomyopathy had a relatively benign prognosis that was inconsistent with its prior characterization as a generally progressive disorder, based primarily on the experience of selected referral institutions. Sudden unexpected cardiac death was distinctly uncommon, although a sizable proportion of patients, particularly the subset prone to atrial fibrillation, did experience clinical deterioration.

PMID:
9463057
[PubMed - indexed for MEDLINE]
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