[Hypertrophic cardiomyopathy: a genetically-carried heart disease].
Hypertrophic cardiomyopathy (HCM) is a cardiac muscle disease with characteristic (mostly asymmetrically distributed) hypertrophy of a non-dilated left ventricle in the absence of another cardiac or systemic disease that can cause left ventricle hypertrophy. The prevalence of HCM in the general population is estimated to be 1 in 500 persons. It is an inheritable disease of the heart with a heterogeneous expression and a great diversity of morphological, functional and clinical features. The genes involved code for components of a large protein complex ('the sarcomere'), which ensures the contraction of the cardiac muscle. Electrocardiography, echocardiography and cardiac MRI play a role in the diagnosis. Medicinal treatment can improve the diastolic filling and the ventricle function. In addition to this there are surgical and non-surgical possibilities for myocardial reduction. For patients with life-threatening arrhythmias and for the primary prevention of sudden death for high-risk patients, an internally implantable cardioverter-defibrillator is indicated. The early detection of patients with a predisposition for HCM is only possible by means of genotyping.
van Dockum
W G
WG
Academisch Ziekenhuis Vrije Universiteit, afd. Cardiologie, Postbus 7057, 1007 MB Amsterdam. wg.vandockum@vumc.nl
Doevendans
P A F M
PA
van Rossum
A C
AC
Wilde
A A M
AA
D024741:Cardiomyopathy, Hypertrophic, Familial / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D005838:Genotype; D006801:Humans; D009426:Netherlands / Q000453:epidemiology; D015995:Prevalence; D011379:Prognosis; D012307:Risk Factors