Dosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA‐HCM

Background Aficamten, a novel cardiac myosin inhibitor, reversibly reduces cardiac hypercontractility in obstructive hypertrophic cardiomyopathy. We present a prespecified analysis of the pharmacokinetics, pharmacodynamics, and safety of aficamten in SEQUOIA‐HCM (Safety, Efficacy, and Quantitative U...

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Main Authors: Caroline J. Coats, Ahmad Masri, Michael E. Nassif, Roberto Barriales‐Villa, Michael Arad, Nuno Cardim, Lubna Choudhury, Brian Claggett, Hans‐Dirk Düngen, Pablo Garcia‐Pavia, Albert A. Hagège, James L. Januzzi, Matthew M. Y. Lee, Gregory D. Lewis, Chang‐Sheng Ma, Martin S. Maron, Zi Michael Miao, Michelle Michels, Iacopo Olivotto, Artur Oreziak, Anjali T. Owens, John A. Spertus, Scott D. Solomon, Jacob Tfelt‐Hansen, Marion van Sinttruije, Josef Veselka, Hugh Watkins, Daniel L. Jacoby, Polina German, Stephen B. Heitner, Stuart Kupfer, Justin D. Lutz, Fady I. Malik, Lisa Meng, Amy Wohltman, Theodore P. Abraham
Format: Article
Language:English
Published: Wiley 2024-08-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
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Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.124.035993
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author Caroline J. Coats
Ahmad Masri
Michael E. Nassif
Roberto Barriales‐Villa
Michael Arad
Nuno Cardim
Lubna Choudhury
Brian Claggett
Hans‐Dirk Düngen
Pablo Garcia‐Pavia
Albert A. Hagège
James L. Januzzi
Matthew M. Y. Lee
Gregory D. Lewis
Chang‐Sheng Ma
Martin S. Maron
Zi Michael Miao
Michelle Michels
Iacopo Olivotto
Artur Oreziak
Anjali T. Owens
John A. Spertus
Scott D. Solomon
Jacob Tfelt‐Hansen
Marion van Sinttruije
Josef Veselka
Hugh Watkins
Daniel L. Jacoby
Polina German
Stephen B. Heitner
Stuart Kupfer
Justin D. Lutz
Fady I. Malik
Lisa Meng
Amy Wohltman
Theodore P. Abraham
author_facet Caroline J. Coats
Ahmad Masri
Michael E. Nassif
Roberto Barriales‐Villa
Michael Arad
Nuno Cardim
Lubna Choudhury
Brian Claggett
Hans‐Dirk Düngen
Pablo Garcia‐Pavia
Albert A. Hagège
James L. Januzzi
Matthew M. Y. Lee
Gregory D. Lewis
Chang‐Sheng Ma
Martin S. Maron
Zi Michael Miao
Michelle Michels
Iacopo Olivotto
Artur Oreziak
Anjali T. Owens
John A. Spertus
Scott D. Solomon
Jacob Tfelt‐Hansen
Marion van Sinttruije
Josef Veselka
Hugh Watkins
Daniel L. Jacoby
Polina German
Stephen B. Heitner
Stuart Kupfer
Justin D. Lutz
Fady I. Malik
Lisa Meng
Amy Wohltman
Theodore P. Abraham
author_sort Caroline J. Coats
collection DOAJ
description Background Aficamten, a novel cardiac myosin inhibitor, reversibly reduces cardiac hypercontractility in obstructive hypertrophic cardiomyopathy. We present a prespecified analysis of the pharmacokinetics, pharmacodynamics, and safety of aficamten in SEQUOIA‐HCM (Safety, Efficacy, and Quantitative Understanding of Obstruction Impact of Aficamten in HCM). Methods and Results A total of 282 patients with obstructive hypertrophic cardiomyopathy were randomized 1:1 to daily aficamten (5–20 mg) or placebo between February 1, 2022, and May 15, 2023. Aficamten dosing targeted the lowest effective dose for achieving site‐interpreted Valsalva left ventricular outflow tract gradient <30 mm Hg with left ventricular ejection fraction (LVEF) ≥50%. End points were evaluated during titration (day 1 to week 8), maintenance (weeks 8–24), and washout (weeks 24–28), and included major adverse cardiac events, new‐onset atrial fibrillation, implantable cardioverter‐defibrillator discharges, LVEF <50%, and treatment‐emergent adverse events. At week 8, 3.6%, 12.9%, 35%, and 48.6% of patients achieved 5‐, 10‐, 15‐, and 20‐mg doses, respectively. Baseline characteristics were similar across groups. Aficamten concentration increased by dose and remained stable during maintenance. During the treatment period, LVEF decreased by −0.9% (95% CI, −1.3 to −0.6) per 100 ng/mL aficamten exposure. Seven (4.9%) patients taking aficamten underwent per‐protocol dose reduction for site‐interpreted LVEF <50%. There were no treatment interruptions or heart failure worsening for LVEF <50%. No major adverse cardiovascular events were associated with aficamten, and treatment‐emergent adverse events were similar between treatment groups, including atrial fibrillation. Conclusions A site‐based dosing algorithm targeting the lowest effective aficamten dose reduced left ventricular outflow tract gradient with a favorable safety profile throughout SEQUOIA‐HCM. Registration URL: https://www.clinicaltrials.gov; Unique Identifier: NCT05186818.
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spelling doaj-art-ecd7f5b3949f4a8ca276c42cd7292a3f2025-08-20T02:12:53ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802024-08-01131510.1161/JAHA.124.035993Dosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA‐HCMCaroline J. Coats0Ahmad Masri1Michael E. Nassif2Roberto Barriales‐Villa3Michael Arad4Nuno Cardim5Lubna Choudhury6Brian Claggett7Hans‐Dirk Düngen8Pablo Garcia‐Pavia9Albert A. Hagège10James L. Januzzi11Matthew M. Y. Lee12Gregory D. Lewis13Chang‐Sheng Ma14Martin S. Maron15Zi Michael Miao16Michelle Michels17Iacopo Olivotto18Artur Oreziak19Anjali T. Owens20John A. Spertus21Scott D. Solomon22Jacob Tfelt‐Hansen23Marion van Sinttruije24Josef Veselka25Hugh Watkins26Daniel L. Jacoby27Polina German28Stephen B. Heitner29Stuart Kupfer30Justin D. Lutz31Fady I. Malik32Lisa Meng33Amy Wohltman34Theodore P. Abraham35School of Cardiovascular and Metabolic Health University of Glasgow United KingdomOregon Health and Science University Portland ORUniversity of Missouri Kansas City Healthcare Institute for Innovations in Quality and Saint Luke’s Mid America Heart Institute Kansas City MOComplexo Hospitalario Universitario A Coruña, INIBIC, CIBERCV‐ISCIII A Coruña SpainLeviev Heart Center, Sheba Medical Center Ramat‐Gan and Tel Aviv University Ramat‐Gan IsraelHospital CUF Descobertas Lisbon PortugalNorthwestern University Feinberg School of Medicine Chicago ILCardiovascular Division Brigham and Women’s Hospital, Harvard Medical School Boston MACharité Campus Virchow‐Klinikum Berlin GermanyHospital Universitario Puerta de Hierro de Majadahonda, IDIPHISA, CIBERCV, and Centro Nacional de Investigaciones Cardiovasculares (CNIC) Madrid SpainAssistance Publique Hôpitaux de Paris, Département de Cardiologie, Hôpital Européen Georges‐Pompidou Paris FranceDivision of Cardiology, Department of Medicine Massachusetts General Hospital, Harvard Medical School Boston MASchool of Cardiovascular and Metabolic Health University of Glasgow United KingdomDivision of Cardiology, Department of Medicine Massachusetts General Hospital, Harvard Medical School Boston MABeijing Anzhen Hospital, Capital Medical University Beijing ChinaLahey Hospital and Medical Center Burlington MACardiovascular Division Brigham and Women’s Hospital, Harvard Medical School Boston MADepartment of Cardiology Erasmus Medical Center, Cardiovascular Institute, Thoraxcenter Rotterdam The NetherlandsMeyer Children’s Hospital, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Florence ItalyNational Institute of Cardiololgy Warsaw PolandUniversity of Pennsylvania Perelman School of Medicine Philadelphia PAUniversity of Missouri Kansas City Healthcare Institute for Innovations in Quality and Saint Luke’s Mid America Heart Institute Kansas City MOCardiovascular Division Brigham and Women’s Hospital, Harvard Medical School Boston MASection of Forensic Genetics, Department of Forensic Medicine, Faculty of Health and Medical Sciences University of Copenhagen DenmarkHypertrophic Cardiomyopathy Patient Author Zwolle The NetherlandsJV Cardiology Prague Czech RepublicRadcliffe Department of Medicine University of Oxford United KingdomCytokinetics, Incorporated South San Francisco CACytokinetics, Incorporated South San Francisco CACytokinetics, Incorporated South San Francisco CACytokinetics, Incorporated South San Francisco CACytokinetics, Incorporated South San Francisco CACytokinetics, Incorporated South San Francisco CACytokinetics, Incorporated South San Francisco CACytokinetics, Incorporated South San Francisco CAUniversity of California San Francisco San Francisco CABackground Aficamten, a novel cardiac myosin inhibitor, reversibly reduces cardiac hypercontractility in obstructive hypertrophic cardiomyopathy. We present a prespecified analysis of the pharmacokinetics, pharmacodynamics, and safety of aficamten in SEQUOIA‐HCM (Safety, Efficacy, and Quantitative Understanding of Obstruction Impact of Aficamten in HCM). Methods and Results A total of 282 patients with obstructive hypertrophic cardiomyopathy were randomized 1:1 to daily aficamten (5–20 mg) or placebo between February 1, 2022, and May 15, 2023. Aficamten dosing targeted the lowest effective dose for achieving site‐interpreted Valsalva left ventricular outflow tract gradient <30 mm Hg with left ventricular ejection fraction (LVEF) ≥50%. End points were evaluated during titration (day 1 to week 8), maintenance (weeks 8–24), and washout (weeks 24–28), and included major adverse cardiac events, new‐onset atrial fibrillation, implantable cardioverter‐defibrillator discharges, LVEF <50%, and treatment‐emergent adverse events. At week 8, 3.6%, 12.9%, 35%, and 48.6% of patients achieved 5‐, 10‐, 15‐, and 20‐mg doses, respectively. Baseline characteristics were similar across groups. Aficamten concentration increased by dose and remained stable during maintenance. During the treatment period, LVEF decreased by −0.9% (95% CI, −1.3 to −0.6) per 100 ng/mL aficamten exposure. Seven (4.9%) patients taking aficamten underwent per‐protocol dose reduction for site‐interpreted LVEF <50%. There were no treatment interruptions or heart failure worsening for LVEF <50%. No major adverse cardiovascular events were associated with aficamten, and treatment‐emergent adverse events were similar between treatment groups, including atrial fibrillation. Conclusions A site‐based dosing algorithm targeting the lowest effective aficamten dose reduced left ventricular outflow tract gradient with a favorable safety profile throughout SEQUOIA‐HCM. Registration URL: https://www.clinicaltrials.gov; Unique Identifier: NCT05186818.https://www.ahajournals.org/doi/10.1161/JAHA.124.035993aficamtencardiac myosin inhibitorhypertrophic cardiomyopathy
spellingShingle Caroline J. Coats
Ahmad Masri
Michael E. Nassif
Roberto Barriales‐Villa
Michael Arad
Nuno Cardim
Lubna Choudhury
Brian Claggett
Hans‐Dirk Düngen
Pablo Garcia‐Pavia
Albert A. Hagège
James L. Januzzi
Matthew M. Y. Lee
Gregory D. Lewis
Chang‐Sheng Ma
Martin S. Maron
Zi Michael Miao
Michelle Michels
Iacopo Olivotto
Artur Oreziak
Anjali T. Owens
John A. Spertus
Scott D. Solomon
Jacob Tfelt‐Hansen
Marion van Sinttruije
Josef Veselka
Hugh Watkins
Daniel L. Jacoby
Polina German
Stephen B. Heitner
Stuart Kupfer
Justin D. Lutz
Fady I. Malik
Lisa Meng
Amy Wohltman
Theodore P. Abraham
Dosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA‐HCM
Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
aficamten
cardiac myosin inhibitor
hypertrophic cardiomyopathy
title Dosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA‐HCM
title_full Dosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA‐HCM
title_fullStr Dosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA‐HCM
title_full_unstemmed Dosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA‐HCM
title_short Dosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA‐HCM
title_sort dosing and safety profile of aficamten in symptomatic obstructive hypertrophic cardiomyopathy results from sequoia hcm
topic aficamten
cardiac myosin inhibitor
hypertrophic cardiomyopathy
url https://www.ahajournals.org/doi/10.1161/JAHA.124.035993
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