VALOR-HCM: Trial in SRT-eligible patients with oHCM



Phase 3, double-blind, randomized, placebo-controlled, multicentre, single country, parallel group trial (112 adult patients) evaluated the efficacy and safety profile of CAMZYOS in septal reduction therapy (SRT)-eligible patients with oHCM1

 

Trial Design

 

Select inclusion criteria1-3

  • ≥18 years old
  • Despite maximally tolerated drug therapy, experienced severe dyspnea or chest pain; symptomatic NYHA Class III/IV* oHCM or Class II with exertional syncope/near syncope
  • LVEF ≥60%
  • Peak LVOT gradient ≥50 mmHg at rest or with provocation
  • Referred or under active consideration for SRT within 12 months of screening

 

Select exclusion criteria2,3

  • Known infiltrative or storage disorder causing cardiac hypertrophy that mimics oHCM
  • Any medical condition that precludes upright exercise stress testing
  • Afib present at screening

 

 

Trial Participants1

 

  • N=112; CAMZYOS n=56, placebo n=56
  • Symptomatic NYHA Class II, III and IV* oHCM
  • LVEF ≥60%
  • LVOT peak gradient ≥50 mmHg at rest or with provocation

 

* CAMZYOS is only indicated for NYHA Class II-III adult patients.

Afib=atrial fibrillation; LVEF=left ventricular ejection fraction; KCCQ-23 CSS=Kansas City Cardiomyopathy Questionnaire-23 Clinical Summary Score; LVEF=left ventricular ejection fraction; LVOT=left ventricular outflow tract; NT-proBNP=Nterminal-proBtype natriuretic peptide; NYHA=New York Heart Association; oHCM=obstructive hypertrophic cardiomyopathy; SRT=septal reduction therapy

Maximally tolerated drug therapy included beta blockers, calcium channel blockers and disopyramide as monotherapy or in combination.


Select patient demographics and disease characteristics1,2

table of patient demographics and disease characteristics

Adapted from the Product Monograph.

Therapy1,2

  • Randomized 1:1 to a starting dose of 5 mg of CAMZYOS or placebo once daily for 16 weeks
  • Randomization was stratified by type of SRT recommended (surgical myectomy or alcohol ablation) and NYHA functional class
  • CAMZYOS dose could be periodically adjusted to maintain a LVEF ≥50% and if warranted by a decrease in LVOT gradient with Valsalva manoeuvre
  • Allowed to continue standard HCM therapy (beta blocker or calcium channel blocker monotherapy)

 

HCM=hypertrophic cardiomyopathy; LAVI=left atrial volume index; LV=left ventricular; LVEF=left ventricular ejection fraction; LVOT=left ventricular outflow tract; NYHA=New York Heart Association; SD=standard deviation; SRT=septal reduction therapy

* CAMZYOS is only indicated for NYHA Class II-III adult patients.
Treatment should be interrupted if LVEF <50%, heart failure symptoms or worsening clinical status occurs.


 

Trial Endpoints

Primary composite endpoint1

Composite endpoint of:

Primary composite endpoint

 

Secondary endpoints:1

Change from baseline to Week 16 in:

  • Post-exercise LVOT peak gradient
  • NYHA functional class
  • NT-proBNP
  • Cardiac troponin I

Patient-reported:

  • Kansas City Cardiomyopathy Questionnaire-23 Clinical Summary Score (KCCQ-23 CSS)

 

LVOT=left ventricular outflow tract; NT-proBNP=Nterminal pro-Btype natriuretic peptide; NYHA=New York Heart Association; SRT=septal reduction therapy

* LVOT gradient of ≥50 mmHg and NYHA Class III-IV, or Class II with exertional syncope or near syncope.

 



PRIMARY COMPOSITE ENDPOINT

With CAMZYOS: Statistically significant reduction vs. placebo in proportion of patients who met the primary composite endpoint: Proceeded with (prior to or at Week 16) or remained guideline-eligible for SRT (at Week 16)1

 
Graph SRT eligibility at Week 16.  With CAMZYOS ~4x more patients no longer met guideline eligibility for SRT or elected not to proceed vs. placebo

Adapted from the Product Monograph.

* Patient decision to proceed with SRT: CAMZYOS treatment group n=2 (4%), placebo group n=2 (4%). SRT status not evaluable (imputed as meeting guideline criteria): CAMZYOS treatment group n=0, placebo group n=2 (4%).1

SRT=septal reduction therapy

 



SECONDARY ENDPOINTS

Post-exercise LVOT peak gradient: Statistically significant improvement observed with CAMZYOS vs. placebo at Week 161

 

With CAMZYOS: ~20x the reduction  in post-exercise LVOT peak gradient shown vs. placebo

Adapted from the Product Monograph.

The guideline-based threshold LVOT gradient for surgery consideration is ≥50 mmHg.4

LVOT=left ventricular outflow tract; SD=standard deviation
* Hierarchical testing of secondary efficacy endpoints showed statistically significant improvement with CAMZYOS vs. placebo.

 

NYHA Class: Statistically significantly more patients showed improvement in symptom control (≥1 NYHA Class) with CAMZYOS vs. placebo1

 

With CAMZYOS 3x as many patients’ NYHA Class improved by ≥1 vs. placebo

Adapted from the Product Monograph.

NYHA=New York Heart Association
* Hierarchical testing of secondary efficacy endpoints showed statistically significant improvement with CAMZYOS vs. placebo.

 

 

NT-proBNP and Cardiac troponin I: Significant results shown with CAMZYOS vs. placebo1

 

NT-proBNP: CAMZYOS significantly reduced this cardiac biomarker from baseline to Week 16 vs. placebo

Treatment difference with CAMZYOS
Geometric mean ratio to baseline difference, 0.33 (95% CI: 0.27-0.42)
p<0.0001*

CAMZYOS -399 ng/L reduction from baseline
vs. placebo 40 ng/L2

Cardiac troponin I: CAMZYOS significantly reduced this cardiac biomarker from baseline to Week 16 vs. placebo

Treatment difference with CAMZYOS
Geometric mean ratio to baseline difference, 0.53 (95% CI: 0.40-0.70)
p<0.0001

CAMZYOS -9.2 ng/L reduction from baseline
vs. placebo 0.07 ng/L2

 

NT-proBNP=N-terminal pro-B-type natriuretic peptide
Geometric mean ratios <1.0 represent an x-fold decrease for CAMZYOS vs. placebo.2
* Hierarchical testing of secondary efficacy endpoints showed statistically significant improvement with CAMZYOS vs. placebo.

 



SECONDARY ENDPOINTS: PATIENT-REPORTED

KCCQ-23 CSS*: Statistically significantly greater improvement shown in patient-reported scores1

 

With CAMZYOS 5x mean improvement in score shown vs. placebo (10 vs. 2) at Week 16

Adapted from the Product Monograph.

 

table of results

Adapted from the Product Monograph.

CSS=Clinical Summary Score; KCCQ-23 CSS=Kansas City Cardiomyopathy Questionnaire 23 Clinical Summary Score; oHCM=obstructive hypertrophic cardiomyopathy
* The KCCQ-23 CSS is a validated patient-reported outcome in oHCM and is composed of the physical limitations (PL) and the total symptom score (TSS) of the KCCQ-23. KCCQ-23 TSS and KCCQ-23 PL were exploratory endpoints.1
† Hierarchical testing of secondary efficacy endpoints showed statistically significant improvement with CAMZYOS vs. placebo.

 



References: 1. CAMZYOS (mavacamten capsules) Product Monograph. Bristol Myers Squibb Canada, February 14, 2024. 2. Desai MY, et al. Myosin inhibition in patients with obstructive hypertrophic cardiomyopathy referred for septal reduction therapy. J Am Coll Cardiol 2022;80(2):95-108. 3. Desai MY, et al. Supplementary appendix. J Am Coll Cardiol 2022;80(2):95-108. 4. Ommen SR, et al. 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy. Circulation 2020;142(25):e588-631.