The Impact of Mavacamten on the Pathophysiology of Hypertrophic Cardiomyopathy: A Narrative Review
| العنوان: | The Impact of Mavacamten on the Pathophysiology of Hypertrophic Cardiomyopathy: A Narrative Review |
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| المؤلفون: | Jay M. Edelberg, Amy J. Sehnert, Matthew E. Mealiffe, Carlos L. del Rio, Robert McDowell |
| المصدر: | American Journal of Cardiovascular Drugs. 22:497-510 |
| بيانات النشر: | Springer Science and Business Media LLC, 2022. |
| سنة النشر: | 2022 |
| مصطلحات موضوعية: | Sarcomeres, Benzylamines, Humans, Pharmacology (medical), General Medicine, Cardiomyopathy, Hypertrophic, Uracil, Cardiology and Cardiovascular Medicine |
| الوصف: | Hypertrophic cardiomyopathy (HCM) is a chronic, progressive disease of the cardiomyocyte with a diverse and heterogeneous clinical presentation and course. This diversity and heterogeneity have added to the complexity of modeling the pathophysiological pathways that contribute to the disease burden. The development of novel therapeutic approaches targeting precise mechanisms within the underlying biology of HCM provides a tool to model and test these pathways. Here, we integrate the results of clinical observations with mavacamten, an allosteric, selective, and reversible inhibitor of cardiac myosin, the motor unit of the sarcomere, to develop an integrated pathophysiological pathway model of HCM, confirming the key role of excess sarcomeric activity. This model may serve as a foundation to understand the role of HCM pathophysiological pathways in the clinical presentation of the disease, and how a targeted therapeutic intervention capable of normalizing sarcomeric activity and repopulating low-energy utilization states may reduce the impact of these pathways in HCM and potentially related disease states. |
| تدمد: | 1179-187X 1175-3277 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::544fae7d80c1c110aaea7e72b0c23f5f https://doi.org/10.1007/s40256-022-00532-x |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....544fae7d80c1c110aaea7e72b0c23f5f |
| قاعدة البيانات: | OpenAIRE |
Find this article in full text from Springer Verlag. | تدمد: | 1179187X 11753277 |
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