Maximilian N Möbius-Winkler, Ulrich Laufs, Karsten Lenk
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引用次数: 0
Abstract
Background: Hypertrophic cardiomyopathy (HCM) with or without left ventricular outflow tract (LVOT) obstruction is a common primary myocardial disease, with a prevalence of 1:500. It is characterized by thickening of the myocardium. Its diagnostic evaluation includes history-taking and physical examination, genetic studies, transthoracic echocardiography, and cardiac MRI. When optimally treated, it carries a mortality of less than 1% per year.
Methods: This review is based on pertinent publications retrieved by a selective literature search, including the current guidelines.
Results: In symptomatic patients with high LVOT gradients (≥ 50 mm Hg), the treatment of first choice is pharmacotherapy with non-vasodilating beta-blockers or non-dihydropyridine-type calcium channel antagonists. Common side effects include bradycardia and hypotension, and there is a risk of AV nodal blockade. Both substance classes lower the LVOT gradient. Beta-blockers alleviate dyspnea and improve patients' quality of life. Verapamil can increase physical resilience. A further option is mavacamten, a myosin inhibitor that gained approval in Germany in mid-2023: it, too, lowers the LVOT gradient and improves quality of life. In 7-10% of patients, there is a reversible reduction of the left ventricular ejection fraction to less than 50%. Septal reduction treatments can be considered if drug therapy fails. Attention must also be paid to the management of sequelae such as atrial fibrillation, malignant arrhythmias, and mitral valve insufficiency.
Conclusion: Patients with HCM have a near-normal life expectancy if the disease is diagnosed early and treated according to the guidelines. The treatment of HCM and HOCM (hypertrophic obstructive cardiomyopathy) have been studied in no more than a few clinical trials, and randomized studies with clinical endpoints are needed.
期刊介绍:
Deutsches Ärzteblatt International is a bilingual (German and English) weekly online journal that focuses on clinical medicine and public health. It serves as the official publication for both the German Medical Association and the National Association of Statutory Health Insurance Physicians. The journal is dedicated to publishing independent, peer-reviewed articles that cover a wide range of clinical medicine disciplines. It also features editorials and a dedicated section for scientific discussion, known as correspondence.
The journal aims to provide valuable medical information to its international readership and offers insights into the German medical landscape. Since its launch in January 2008, Deutsches Ärzteblatt International has been recognized and included in several prestigious databases, which helps to ensure its content is accessible and credible to the global medical community. These databases include:
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