Hypertrophic Cardiomyopathy with Special Focus on Mavacamten and Its Future in Cardiology
Hypertrophic cardiomyopathy (HCM) is a heterogeneous group of heart muscle disorders that affects millions, with an incidence from 1 in 500 to 1 in 200. Factors such as genetics, age, gender, comorbidities, and environmental factors may contribute to the course of this disease. Diagnosis of HCM has...
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2024-11-01
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| author | Ewelina Młynarska Ewa Radzioch Bartłomiej Dąbek Klaudia Leszto Alicja Witkowska Witold Czarnik Weronika Jędraszak Jacek Rysz Beata Franczyk |
| author_facet | Ewelina Młynarska Ewa Radzioch Bartłomiej Dąbek Klaudia Leszto Alicja Witkowska Witold Czarnik Weronika Jędraszak Jacek Rysz Beata Franczyk |
| author_sort | Ewelina Młynarska |
| collection | DOAJ |
| description | Hypertrophic cardiomyopathy (HCM) is a heterogeneous group of heart muscle disorders that affects millions, with an incidence from 1 in 500 to 1 in 200. Factors such as genetics, age, gender, comorbidities, and environmental factors may contribute to the course of this disease. Diagnosis of HCM has improved significantly in the past few decades from simple echocardiographic evaluations to a more complex, multimodal approach embracing advanced imaging, genetic, and biomarker studies. This review focuses on Mavacamten, a selective allosteric inhibitor of cardiac myosin, as a pharmacological treatment for HCM. Patients with HCM experience pathological actomyosin interactions, leading to impaired relaxation and increased energy expenditure. Mavacamten decreases available myosin heads, reducing actomyosin cross-bridges during systole and diastole. By reducing the number of bridges left ventricular outflow tract pressure is normalized and cardiac cavities are filled. This mechanism enhances patient performance and alleviates symptoms such as chest pain and dyspnea. The results suggest the potential for Mavacamten to transform the treatment of obstructive hypertrophic cardiomyopathy. Studies to date have shown significant improvement in exercise capacity, symptom relief, and a reduction in the need for invasive procedures such as septal myectomy. Further studies are needed to confirm the clinical results. |
| format | Article |
| id | doaj-art-77aa2dfd64d24879a7e3642a763b63e2 |
| institution | DOAJ |
| issn | 2227-9059 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | MDPI AG |
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| series | Biomedicines |
| spelling | doaj-art-77aa2dfd64d24879a7e3642a763b63e22025-08-20T02:50:52ZengMDPI AGBiomedicines2227-90592024-11-011212267510.3390/biomedicines12122675Hypertrophic Cardiomyopathy with Special Focus on Mavacamten and Its Future in CardiologyEwelina Młynarska0Ewa Radzioch1Bartłomiej Dąbek2Klaudia Leszto3Alicja Witkowska4Witold Czarnik5Weronika Jędraszak6Jacek Rysz7Beata Franczyk8Department of Nephrocardiology, Medical University of Lodz, ul. Zeromskiego 113, 90-549 Lodz, PolandDepartment of Nephrocardiology, Medical University of Lodz, ul. Zeromskiego 113, 90-549 Lodz, PolandDepartment of Nephrocardiology, Medical University of Lodz, ul. Zeromskiego 113, 90-549 Lodz, PolandDepartment of Nephrocardiology, Medical University of Lodz, ul. Zeromskiego 113, 90-549 Lodz, PolandDepartment of Nephrocardiology, Medical University of Lodz, ul. Zeromskiego 113, 90-549 Lodz, PolandDepartment of Nephrocardiology, Medical University of Lodz, ul. Zeromskiego 113, 90-549 Lodz, PolandDepartment of Nephrocardiology, Medical University of Lodz, ul. Zeromskiego 113, 90-549 Lodz, PolandDepartment of Nephrology, Hypertension and Family Medicine, Medical University of Lodz, ul. Zeromskiego 113, 90-549 Lodz, PolandDepartment of Nephrocardiology, Medical University of Lodz, ul. Zeromskiego 113, 90-549 Lodz, PolandHypertrophic cardiomyopathy (HCM) is a heterogeneous group of heart muscle disorders that affects millions, with an incidence from 1 in 500 to 1 in 200. Factors such as genetics, age, gender, comorbidities, and environmental factors may contribute to the course of this disease. Diagnosis of HCM has improved significantly in the past few decades from simple echocardiographic evaluations to a more complex, multimodal approach embracing advanced imaging, genetic, and biomarker studies. This review focuses on Mavacamten, a selective allosteric inhibitor of cardiac myosin, as a pharmacological treatment for HCM. Patients with HCM experience pathological actomyosin interactions, leading to impaired relaxation and increased energy expenditure. Mavacamten decreases available myosin heads, reducing actomyosin cross-bridges during systole and diastole. By reducing the number of bridges left ventricular outflow tract pressure is normalized and cardiac cavities are filled. This mechanism enhances patient performance and alleviates symptoms such as chest pain and dyspnea. The results suggest the potential for Mavacamten to transform the treatment of obstructive hypertrophic cardiomyopathy. Studies to date have shown significant improvement in exercise capacity, symptom relief, and a reduction in the need for invasive procedures such as septal myectomy. Further studies are needed to confirm the clinical results.https://www.mdpi.com/2227-9059/12/12/2675cardiomyopathieshypertrophic cardiomyopathymavacamtencardiac therapeutics |
| spellingShingle | Ewelina Młynarska Ewa Radzioch Bartłomiej Dąbek Klaudia Leszto Alicja Witkowska Witold Czarnik Weronika Jędraszak Jacek Rysz Beata Franczyk Hypertrophic Cardiomyopathy with Special Focus on Mavacamten and Its Future in Cardiology Biomedicines cardiomyopathies hypertrophic cardiomyopathy mavacamten cardiac therapeutics |
| title | Hypertrophic Cardiomyopathy with Special Focus on Mavacamten and Its Future in Cardiology |
| title_full | Hypertrophic Cardiomyopathy with Special Focus on Mavacamten and Its Future in Cardiology |
| title_fullStr | Hypertrophic Cardiomyopathy with Special Focus on Mavacamten and Its Future in Cardiology |
| title_full_unstemmed | Hypertrophic Cardiomyopathy with Special Focus on Mavacamten and Its Future in Cardiology |
| title_short | Hypertrophic Cardiomyopathy with Special Focus on Mavacamten and Its Future in Cardiology |
| title_sort | hypertrophic cardiomyopathy with special focus on mavacamten and its future in cardiology |
| topic | cardiomyopathies hypertrophic cardiomyopathy mavacamten cardiac therapeutics |
| url | https://www.mdpi.com/2227-9059/12/12/2675 |
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