Journal Information
Vol. 30. Issue 11.
Pages 829-835 (November 2011)
Share
Share
Download PDF
More article options
Vol. 30. Issue 11.
Pages 829-835 (November 2011)
Original article
Open Access
From hypertrophic cardiomyopathy centers to inherited cardiovascular disease centers in Europe. A small or a major step? A position paper from the Nucleus of the Working Group on Myocardial and Pericardial Diseases of the Portuguese Society of Cardiology
Dos centros de miocardiopatia hipertrófica até às unidades de doenças cardíacas hereditárias na Europa: um pequeno grande passo?
Visits
7430
Nuno Cardima,
Corresponding author
ncardim@hospitaldaluz.pt

Corresponding author.
, António Freitasb, Dulce Britoc
a Serviço de Cardiologia, Hospital da Luz, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisboa, Portugal
b Serviço de Cardiologia, Hospital Fernando da Fonseca, Amadora, Portugal
c Serviço de Cardiologia, Hospital de Santa Maria, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal
This item has received

Under a Creative Commons license
Article information
Abstract

The prevalence, complexity, clinical importance, heterogeneity and unpredictability of inherited cardiovascular diseases make the development of inherited cardiovascular disease centers an inevitability, with the ultimate goal of reducing the morbidity and mortality associated with these conditions. An inherited cardiovascular disease center may be seen as a subunit of a cardiology department, with health professionals specializing in these types of disorders, organized to provide excellence in all related areas, including diagnosis, treatment, follow-up, prevention, risk stratification and prognosis. Among its objectives are the development of action protocols and the creation of databases that enable patients to be included in national and international research networks. To achieve these objectives these centers should include functional units of clinical and basic sciences, research, training and education, acting in harmony in a holistic approach to patients and their families. As most experience on inherited cardiovascular diseases is based on hypertrophic cardiomyopathy and on “hypertrophic cardiomyopathy centers”, these centers represent an excellent opportunity to learn how to set up inherited cardiovascular disease centers. European centers will differ from country to country, reflecting the heterogeneity of national health systems, but will share a common core, presented in this document. Though we are aware that this ambitious project is not at all easy and may be difficult to implement in its entirety – in fact we consider it a major step – our position is that all the efforts to achieve it are worthwhile, considering that the main goal will always be the well-being of those affected by these particular disorders.

Keywords:
Inherited cardiovascular diseases
Inherited cardiovascular disease centers
Hypertrophic cardiomyopathy
Hypertrophic cardiomyopathy centers
Resumo

A prevalência, complexidade, importância clínica, heterogeneidade e imprevisibilidade das doenças cardíacas heredofamiliares torna a criação de Unidades de Doenças Cardíacas Hereditárias uma inevitabilidade, cujo objectivo final é reduzir a morbilidade e a mortalidade relacionadas com estas patologias. Uma Unidade de Doenças Cardíacas Hereditárias é uma subunidade de um departamento de Cardiologia constituída por profissionais de saúde especializados neste tipo de doenças, organizado de forma a proporcionar a excelência em todos as suas áreas, incluindo diagnóstico, tratamento, seguimento, prevenção, estratificação de risco e determinação de prognóstico. Alguns dos seus objectivos são o desenvolvimento de protocolos de actuação e a criação de bases de dados que permitam a inclusão de doentes em registos e redes de investigação nacionais e internacionais. Para atingir estes objectivos estes centros devem integrar Unidades Funcionais (clínica, ciências básicas, investigação, treino e educação), actuando harmonicamente numa abordagem holística de doentes e suas famílias. Como grande parte do conhecimento referente às doenças cardíacas hereditárias se baseia na miocardiopatia hipertrófica e na experiência adquirida com os “centros de miocardiopatia hipertrófica”, estes representam um excelente modelo para aprender como criar e desenvolver as Unidades de Doenças Cardíacas Hereditárias. A nível Europeu, as características destas Unidades serão diferentes de um país para outro, reflectindo a heterogeneidade dos diferentes sistemas e serviços nacionais de saúde, compartilhando no entanto um core comum, apresentado neste documento. Embora conscientes de que este projecto é ambicioso e de que a sua concretização como um todo não será uma tarefa fácil, consideramos a aplicação e divulgação deste conceito a nível nacional e Europeu um passo de grande importância. Assim, a nossa posição é que todos os esforços para atingir esta meta valem a pena, considerando que o principal objectivo será sempre o bem-estar dos doentes afectados por estas patologias.

Palavras-chave:
Doenças cardíacas hereditárias
Unidades de doenças cardíacas hereditárias
Miocardiopatia hipertrófica
Centros de miocardiopatia hipertrófica
Full text is only aviable in PDF
References
[1]
H. Burton, C. Alberg, Stewart A.
Heart to heart: Inherited Cardiovascular Conditions Services.
A Needs Assessment and Service Review, (2009),
[2]
B.J. Maron, W.J. McKenna, G.K. Danielson, et al.
American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines.
Eur Heart J, 24 (2003 Nov), pp. 1965-1991
[3]
P. Lebecque, A. Leonard, K. De Boeck, et al.
Early referral to cystic fibrosis specialist centre impacts on respiratory outcome.
J Cystic Fibrosis, (2009), pp. 26-30
[4]
National Pulmonary Hypertension Centres of the UK Ireland.
Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland.
Heart, 94 (2008), pp. i1-i41
[5]
B. Maron.
Hypertrophic cardiomyopathy centers.
Am J Cardiol, (2009), pp. 1158-1159
[6]
B. Maron, P. Spirito, Win-Kuang Shen, et al.
Implantable Cardioverter-Defibrillators and Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy.
JAMA, 298 (2007), pp. 405-412
[7]
B. Maron, I. Olivotto, P. Spirito, et al.
Epidemiology of Hypertrophic Cardiomyopathy–Related Death: Revisited in a Large Non–Referral-Based Patient Population.
Circulation, 102 (2000), pp. 858-864
[8]
World Population Prospects: The 2006 Revision Population Database. UN – Department of Economic and Social Affairs. http://esa.un.org/unpp. Retrieved 2008-06-10.
[9]
European countries according to the EU. European Commission. http://europa.eu/abc/european_countries. Retrieved 2008-06-13.
[10]
Rare Diseases Task Force: European reference networks in the field of rare diseases: state of the art and future directions. Third report 2008. http://www.orpha.net/actor/EuropaNews/2008/doc/CE.pdf.
[11]
Centres of Reference for rare diseases in Europe: State-of-the-art in 2006 and recommendations of the Rare Diseases Task Force. http://ec.europa.eu/health/ph_threats/non_com/docs/contribution_policy.pdf.
[12]
S. Wordsworth, J. Leal, E. Blair, et al.
DNA testing for hypertrophic cardiomyopathy: a cost-effectiveness model.
Eur Heart J, 31 (2010), pp. 926-935
Copyright © 2011. Sociedade Portuguesa de Cardiologia
Download PDF
Idiomas
Revista Portuguesa de Cardiologia (English edition)
Article options
Tools
en pt

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos

By checking that you are a health professional, you are stating that you are aware and accept that the Portuguese Journal of Cardiology (RPC) is the Data Controller that processes the personal information of users of its website, with its registered office at Campo Grande, n.º 28, 13.º, 1700-093 Lisbon, telephone 217 970 685 and 217 817 630, fax 217 931 095, and email revista@spc.pt. I declare for all purposes that the information provided herein is accurate and correct.