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 |