| Abstract [eng] |
Introduction. Hypertrophic cardiomyopathy is one of the most common genetically determined heart diseases. About 70% of patients have an obstruction in the left ventricular outflow tract, which is associated with an increased risk of heart failure, arrhythmias and sudden cardiac death. Although some patients remain asymptomatic throughout their lives, the first symptoms may appear at any age and sometimes the disease is diagnosed only after sudden cardiac death. Contemporary pharmacological treatment includes traditional drugs that have been used for many years and selective cardiac myosin inhibitors that have recently entered clinical practice. Controlling symptoms, reducing left ventricular outflow tract obstruction, improving quality of life and halting disease progression are the primary goals of medical therapy. Objective. To review the literature on the efficacy and patient benefit of current medications for hypertrophic cardiomyopathy with left ventricular outflow tract obstruction and to present a clinical case illustrating treatment strategy. Methodology. Narrative literature review. “PubMed”, “ScienceDirect” and “Clinical Key” databases were searched for scientific publications using the following search terms and their combinations: “hypertrophic cardiomyopathy”, “obstructive hypertrophic cardiomyopathy”, “medical treatment”, “beta-blockers”, “calcium channel blockers”, “disopyramide”, “selective cardiac myosin inhibitors”. Priority was given to publications less than 10 years old. Articles and clinical trials data were reviewed. The most recent 2023 European Society of Cardiology and 2024 American Heart Association guidelines for the management of cardiomyopathies were analysed and compared. The aetiology, pathogenesis, diagnosis and treatment of the disease were described, with a focus on medical therapy. With written informed consent from the patient and the institution, the clinical case of a 32-year-old patient with hypertrophic cardiomyopathy with left ventricular outflow tract obstruction was analysed and described. Conclusions. The best results of medical therapy in patients with hypertrophic cardiomyopathy with left ventricular outflow tract obstruction are achieved through individualised treatment strategies, taking into account the patient's symptoms, their severity and haemodynamic parameters. Conventional therapies such as beta-blockers, calcium channel blockers and disopyramide can reduce symptoms and left ventricular outflow tract gradient, but their effect is not sufficient in some cases. The selective cardiac myosin inhibitors mavacamten and aficamten are the newest drugs in the treatment of this disease, with high efficacy in reducing obstruction, symptom severity and improving quality of life, and their tolerability and safety are favourably evaluated in clinical trials. However, continuous monitoring of the patient is necessary to assess possible adverse drug reactions, changes in symptom intensity and haemodynamics, and to modify the drug treatment strategy or switch to interventional therapies if necessary and feasible. Keywords: hypertrophic cardiomyopathy, left ventricular outflow tract obstruction, medical therapy, selective cardiac myosin inhibitors. |
