Elsevier

American Heart Journal

Volume 150, Issue 5, November 2005, Pages 947-954
American Heart Journal

Clinical Investigation
Congestive Heart Failure
The Italian registry for hypertrophic cardiomyopathy: A nationwide survey

https://doi.org/10.1016/j.ahj.2005.01.005Get rights and content

Background

National registries are advocated as instrumental to the solution of rarity-related problems for patients with hypertrophic cardiomyopathy (HCM), including limited access to advanced treatment options. Thus, an Italian Registry for HCM was created to assess the clinical profile and the level of care nationwide of patients with HCM.

Methods

Cardiology centers over the national territory were recruited to provide clinical data of all patients with HCM ever seen at each institution. The enrollment period was from May 2000 to May 2002.

Results

The registry enrolled 1677 patients from 40 institutions. Most (69%) were followed at referral centers, whereas 31% were from community centers with intermediate-low patient flow. Patients diagnosed after routine medical examinations or familial screenings were 39%. Most patients were male (62%), in their fourth to sixth decade of life, and in New York Heart Association class I to II (89%); 24% had resting left ventricular obstruction and 18% had atrial fibrillation. During a 9.7-year average follow-up, cardiovascular mortality was 1%/y, mostly because of heart failure, with no significant change over the last 3 decades; sudden death was less common (0.4%/y). Only 4% of patients received a defibrillator; 14% of the 401 patients with LV outflow obstruction underwent invasive relief of obstruction; and <1% were offered genetic analyses or counseling.

Conclusions

The Italian Registry represents the first comprehensive attempt to evaluate the clinical impact and management of HCM at a national level. Findings underscore the role of screening strategies for an early diagnosis and suggest limited use of the advanced therapeutic options for HCM.

Section snippets

The Progetto Cuore

The Italian Registry for HCM is a research initiative implemented as part of the Progetto Cuore (“Heart Project”), a large epidemiologic survey aimed at cardiovascular prevention funded by the Italian Ministry of Health and the National Institute of Health (Istituto Superiore di Sanità, www.cuore.iss.it/eng/index_en.htm). The enrollment period was from May 2000 to May 2002. A direct invitation to participate was made to a large number of cardiology centers nationwide, ranging from tertiary

Participating institutions

Overall, the registry enrolled 1677 patients from 67 Italian provinces, followed at 40 institutions. Of those who were alive at last contact, 93% had been seen within the last 2 years, including 89% within the last 12 months. Most patients (1157, or 69%) were followed at 7 referral institutions with specific interest in HCM (Table I), each following >50 patients, including 4 high-volume centers contributing >150 patients each. At the other end of the spectrum, 13% of the patients were followed

A nationwide survey

The present survey, unprecedented in scope and number of participating institutions, is the result of a nationwide initiative involving 40 cardiological centers and 1677 patients over the entire Italian territory.2 Recent epidemiologic evidence6, 7, 8, 9, 10, 11, 12 suggests a prevalence of HCM of about 1:500 HCM in the general population, that is, about 100 000 patients in Italy. Based on such estimate, our cohort may represent between 1% and 2% of affected individuals in the country. To our

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    This study was funded by a grant from the Italian Ministry of Health and the Istituto Superiore di Sanità.

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