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MARON AND MCKENNA et al., ACC/ESC Expert Consensus Document on Hypertrophic Cardiomyopathy
JACC 2003; 42
: 000-000

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

General Considerations and Perspectives
This clinical scientific statement represents the consensus of a panel of experts appointed by the ACC and ESC. The writing group is comprised of cardiovascular specialists and molecular biologists, each having extensive experience with HCM. The panel focused largely on the management of this complex disease and derived prudent, practical, and contemporary treatment strategies for the many subgroups of patients comprising the broad HCM disease spectrum. Because of the relatively low prevalence of HCM in general cardiologic practice 50, its diverse presentation, and mechanisms of death and disability and skewed patterns of patient referral 7,11,13,36-38,42,51-59, the level of evidence governing management decisions for drugs or devices has often been derived from non-randomized and retrospective investigations. Large-scale controlled and randomized study designs, such as those that have provided important answers regarding the management of coronary artery disease (CAD) and congestive heart failure 60-62, have generally not been available in HCM as a result of these factors. Therefore, treatment strategies have necessarily evolved based on available data that have frequently been observational in design, sometimes obtained in relatively small patient groups, or derived from the accumulated clinical experience of individual investigators, and reasonable inferences drawn from other cardiac diseases. Consequently, the construction of strict clinical algorithms designed to assess prognosis and dictate treatment decisions for all patients has been challenging and has not yet achieved general agreement. In some clinical situations, management decisions and strategies unavoidably must be individualized to the particular patient.

Understanding of the molecular basis, clinical course, and treatment of HCM has increased substantially in the last decade. In particular, there has been a growing awareness of the clinical and molecular heterogeneity characteristic of this disorder and the many patient subgroups that inevitably influence considerations for treatment. Some of these management strategies are novel and evolving, and this document cannot, in all instances, convey definitive assessments of their role in the treatment armamentarium. Also, for some uncommon subsets within the broad disease spectrum, there are little data currently available to definitively guide therapy. With these considerations in mind, the panel has aspired to create a document that is not only current and pertinent but also has the potential to remain relevant for many years.

 

© 2003 by the American College of Cardiology and the European Society of Cardiology

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