Cor Vasa 2003, 44(10):507-512
Current concepts regarding hypertrophic cardiomyopathy
- I. interní-kardioangiologická klinika, Fakultní nemocnice u sv. Anny, Brno, Česká republika
Hypertrophic cardiomyopathy is an autosomal dominant hereditary disease with a prevalence of about 0.2%. Ten genes for sarcomere proteins whose mutations result in the development of this disease have been identified to date. The diagnosis is based primarily on echocardiography allowing to recognize (mainly non-obstructive and obstructive) subtypes of the disease.
Therapy differs depending on individual disease subtypes, with drug therapy being the mainstay of treatment (Table I). The agents used for drug therapy include beta-blockers and verapamil (or, less frequently, disopyramide in this country). Patients with the obstructive type may be referred, besides drug therapy, to non-pharmacological procedures. The oldest of these is surgical myotomy/myectomy, still regarded as the "gold standard". The most frequently performed procedure today is percutaneous transluminal septal myocardial ablation, which becomes the method of choice in many cases. Cardiac pacing is associated with some questions; in most patients, it reduces the left ventricular outflow tract gradient and improves symptoms. The most common cause of death, particularly in young patients, is sudden death. The agents administered for sudden death prevention include amiodarone and beta-blockers, with controversial results. The most effective procedure is cardioverter-defibrillator implantation in patients at highest risk.
Hypertrophic cardiomyopathy is a variable disease, with genetics believed to play an increasingly important role in its diagnosis and management. As it is, current pharmacological options and, when appropriate, non-pharmacological therapeutic procedures, should be properly used.
Keywords: Hypertrophic cardiomyopathy; Diagnosis; Drug therapy; Non-pharmacological treatment
Published: October 1, 2003 Show citation