Evaluation of Mavacamten in Symptomatic Patients With Nonobstructive Hypertrophic Cardiomyopathy
Carolyn Y. Ho,Matthew E Mealiffe,Richard G. Bach,Mondira Bhattacharya,Lubna Choudhury,Jay M. Edelberg,Sheila M. Hegde,Daniel Jacoby,Neal K. Lakdawala,Steven J. Lester,Yanfei Ma,Ali J. Marian,Sherif F. Nagueh,Anjali T. Owens,Florian Rader,Sara Saberi,Amy J. Sehnert,Mark V. Sherrid,Scott D. Solomon,Andrew Wang,Omar Wever-Pinzon,Timothy C. Wong,Stephen B. Heitner +22 more
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TLDR
Mavacamten, a novel myosin inhibitor, was well tolerated in most subjects with symptomatic nHCM and treatment was associated with a significant reduction in NT-proBNP and cTnI, suggesting improvement in myocardial wall stress.About:
This article is published in Journal of the American College of Cardiology.The article was published on 2020-06-02 and is currently open access. It has received 149 citations till now. The article focuses on the topics: Hypertrophic cardiomyopathy.read more
Citations
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Journal ArticleDOI
2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy
Steve R. Ommen,Seema Mital,Michael A. Burke,Sharlene M. Day,Anita Deswal,Perry M. Elliott,Lauren L. Evanovich,Judy Hung,Jose A. Joglar,Paul F. Kantor,Carey Kimmelstiel,Michelle M. Kittleson,Mark S. Link,Martin S. Maron,Matthew W. Martinez,Christina Y. Miyake,Hartzell V. Schaff,Christopher Semsarian,Paul Sorajja +18 more
Journal ArticleDOI
2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines
Steve R. Ommen,Seema Mital,Michael A. Burke,Sharlene M. Day,Anita Deswal,Perry M. Elliott,Lauren L. Evanovich,Judy Hung,Jose A. Joglar,Paul F. Kantor,Carey Kimmelstiel,Michelle M. Kittleson,Mark S. Link,Martin S. Maron,Matthew W. Martinez,Christina Y. Miyake,Hartzell V. Schaff,Christopher Semsarian,Paul Sorajja +18 more
TL;DR: This summary operationalizes the recommendations from the full guideline and presents a combination of diagnostic work-up, genetic and family screening, risk stratification approaches, lifestyle modifications, surgical and catheter interventions, and medications that constitute components of guideline directed medical therapy.
Journal ArticleDOI
Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): a randomised, double-blind, placebo-controlled, phase 3 trial
Iacopo Olivotto,Artur Oreziak,Roberto Barriales-Villa,Theodore P. Abraham,Ahmad Masri,Pablo García-Pavía,Pablo García-Pavía,Sara Saberi,Neal K. Lakdawala,Matthew T. Wheeler,Anjali T. Owens,Milos Kubanek,Wojciech Wojakowski,Morten Kvistholm Jensen,Juan Ramón Gimeno-Blanes,Kia Afshar,Jonathan Myers,Jonathan Myers,Sheila M. Hegde,Scott D. Solomon,Amy J. Sehnert,David Zhang,Wanying Li,Mondira Bhattacharya,Jay M. Edelberg,Cynthia Burstein Waldman,Steven J. Lester,Andrew Wang,Carolyn Y. Ho,Daniel Jacoby,Jozef Bartunek,Antoine Bondue,Emeline Van Craenenbroeck,David Zemanek,Morten Søndergaard Jensen,Jens Mogensen,Jens Jakob Thune,Philippe Charron,Albert Hagège,Olivier Lairez,Jean Noël Trochu,Christoph Axthelm,Hans Dirk Duengen,Norbert Frey,Veselin Mitrovic,Michael Preusch,Jeanette Schulz-Menger,Tim Seidler,Michael Arad,Majdi Halabi,Amos Katz,Daniel Monakier,Offir Paz,Samuel Viskin,Donna R. Zwas,Hans-Peter Brunner-La Rocca,Michelle Michels,Dariusz Dudek,Zofia Oko-Sarnowska,Nuno Cardim,Helder Pereira,Pablo García Pavia,Juan Ramon Gimeno Blanes,Rafael Hidalgo Urbano,Luis Miguel Rincón Diaz,Perry M. Elliott,Zaheer Yousef,Theodore Abraham,Paulino Alvarez,Richard G. Bach,Richard C. Becker,Lubna Choudhury,David Fermin,John L. Jefferies,Christopher M. Kramer,Neal K. Lakdawala,Steven Lester,Ali J. Marian,Mathew S. Maurer,Sherif F. Nagueh,David R. Owens,Florian Rader,Mark V. Sherrid,Jamshid Shirani,John Symanski,Aslan T. Turer,Omar Wever-Pinzon,Timothy C. Wong,Mohamad H. Yamani +88 more
TL;DR: Treatment with mavacamten improved exercise capacity, LVOT obstruction, NYHA functional class, and health status in patients with obstructive hypertrophic cardiomyopathy and highlights the benefits of disease-specific treatment for this condition.
Journal ArticleDOI
2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.
Steve R. Ommen,Seema Mital,Michael A. Burke,Sharlene M. Day,Anita Deswal,Perry M. Elliott,Lauren L. Evanovich,Judy Hung,Jose A. Joglar,Paul F. Kantor,Carey Kimmelstiel,Michelle M. Kittleson,Mark S. Link,Martin S. Maron,Matthew W. Martinez,Christina Y. Miyake,Hartzell V. Schaff,Christopher Semsarian,Paul Sorajja,Patrick T. O'Gara,Joshua A. Beckman,Glenn N. Levine,Sana M. Al-Khatib,Anastasia Armbruster,Kim K. Birtcher,Joaquin Ciggaroa,Dave L. Dixon,Lisa de las Fuentes,Lee A. Fleisher,Federico Gentile,Zachary D. Goldberger,Bulent Gorenek,Norrisa Haynes,Adrian F. Hernandez,Mark A. Hlatky,W. Schuyler Jones,Joseph E. Marine,Daniel B. Mark,Latha Palaniappan,Mariann R. Piano,Jacqueline E. Tamis-Holland,Duminda N. Wijeysundera,Y. Joseph Woo +42 more
Journal ArticleDOI
Small Molecules acting on Myofilaments as Treatments for Heart and Skeletal Muscle Diseases.
TL;DR: The contractile apparatus is a promising target for new drug development according to an extensive literature review of 21 small molecules directed to five different targets, which showed a potential therapeutic value in HCM, DCM or heart failure (HF).
References
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Journal ArticleDOI
2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy The Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC)
Perry M. Elliott,Aris Anastasakis,Michael A. Borger,Martin Borggrefe,Franco Cecchi,Philippe Charron,Albert Hagège,Antoine Lafont,Giuseppe Limongelli,Heiko Mahrholdt,William J. McKenna,Jens Mogensen,Petros Nihoyannopoulos,Stefano Nistri,Petronella G. Pieper,Burkert Pieske,Claudio Rapezzi,Frans H. Rutten,Christoph Tillmanns,Hugh Watkins +19 more
TL;DR: The objective of this study was to establish a baseline level of confidence that the once-in-a-lifetime implantation trial—Reduce Inappropriate Therapy protocol can be trusted to provide safe and effective treatment for cardiac arrhythmia and stroke-like episodes.
Journal ArticleDOI
Hypertrophic Cardiomyopathy: Genetics, Pathogenesis, Clinical Manifestations, Diagnosis, and Therapy.
Ali J. Marian,Eugene Braunwald +1 more
TL;DR: The routine applications of genetic testing and preclinical identification of family members represents an important advance and enhanced understanding of the molecular pathogenesis of HCM and have stimulated efforts designed to identify new therapeutic agents.
Journal ArticleDOI
The histologic basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy.
James C. Moon,Emma Reed,Mary N. Sheppard,Andrew G. Elkington,Siew Yen Ho,Margaret Burke,Mario Petrou,Dudley J. Pennell +7 more
TL;DR: In this patient with HCM and heart failure, regions of myocardial late gadolinium enhancement by CMR represented regions of increased myocardia collagen but not disarray.
Journal ArticleDOI
Occurrence and Frequency of Arrhythmias in Hypertrophic Cardiomyopathy in Relation to Delayed Enhancement on Cardiovascular Magnetic Resonance
A. Selcuk Adabag,Barry J. Maron,Evan Appelbaum,Evan Appelbaum,Caitlin J. Harrigan,Jacqueline L. Buros,C. Michael Gibson,C. Michael Gibson,John R. Lesser,Constance A. Hanna,James E. Udelson,Warren J. Manning,Warren J. Manning,Martin S. Maron +13 more
TL;DR: In this large HCM cohort with no or only mild symptoms, myocardial fibrosis detected by CMR was associated with greater likelihood and increased frequency of ventricular tachyarrhythmias (including NSVT) on ambulatory Holter ECG.
Journal ArticleDOI
Myocardial Fibrosis as an Early Manifestation of Hypertrophic Cardiomyopathy
Carolyn Y. Ho,Begoña López,Otavio R. Coelho-Filho,Neal K. Lakdawala,Allison L. Cirino,Petr Jarolim,Raymond Y. Kwong,Arantxa González,Steven D. Colan,Jonathan G. Seidman,Javier Díez,Christine E. Seidman +11 more
TL;DR: Elevated levels of serum PICP indicated increased myocardial collagen synthesis in sarcomere-mutation carriers without overt disease, suggesting that collagen synthesis exceeds degradation.
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