The Effect of Mavacamten on Myocardial Perfusion in Obstructive Hypertrophic Cardiomyopathy

Preliminary

Kostas Moschonas

2025-10-31

Study Design

Adults with HCM and:

  1. NYHA II or III
  2. \(Max\,LVOT\,gradient\geq 50 mmHg\), at rest or provocation
  3. \(LVEF \geq 55\%\)

Start at \(2.5\,mg\)

Up-titrated according to LVOT gradient and symptoms.

Clinical follow-up monthly for first 3-4 months.

Quantitative perfusion CMRs at baseline, 2 and 6 months.

Recruitment Progress

2-month timepoint complete for 31 participants.

6-month timepoint for 24/31. Complete in March 2026.

But 28/31 before 2026.

Baseline Characteristics

Characteristic N = 231
Age 61 (12)
Sex
    female 9 (39%)
    male 14 (61%)
Ethnicity
    Asian or Asian British 5 (22%)
    Black, Black British, Caribbean or African 1 (4.3%)
    Mixed or multiple ethnic groups 1 (4.3%)
    White 16 (70%)
    Any other ethnic group 0 (0%)
Hypertension 4 (17%)
Diabetes Type 2 2 (8.7%)
Dyslipidaemia 1 (4.3%)
Smoking 0 (0%)
Atrial Fibrillation 5 (22%)
Stroke 1 (4.3%)
Family History of SCD 3 (14%)
Family History of Cardiomyopathy 0 (0%)
Gene Positive 8 (35%)
Gene Classification
    non pathogenic 0 (0%)
    VUS 2 (25%)
    pathogenic 5 (63%)
    likely pathogenic 1 (13%)
    likely non-pathogenic 0 (0%)
    not_applicable 0 (0%)
    missing 0 (0%)
Variant
     15 (65%)
    CSPR3 1 (4.3%)
    MYBPC3 3 (13%)
    MYH7 3 (13%)
    TNNI3 1 (4.3%)
NSVT 6 (30%)
CYP450 Metaboliser
    normal 8 (35%)
    rapid 7 (30%)
    intermediate 5 (22%)
    poor 1 (4.3%)
    ultrarapid 2 (8.7%)
Bisoprolol 15 (65%)
Verapamil 7 (30%)
CIED
    no 16 (70%)
    PPM 1 (4.3%)
    ICD_transvenous 3 (13%)
    ICD_subcut 2 (8.7%)
    CRTP 0 (0%)
    CRTD 0 (0%)
    ILR 1 (4.3%)
ICD indication
    prim_prevention 5 (100%)
    sec_prevention 0 (0%)
Haemoglobin 143 (17)
Haematocrit 0.42 (0.05)
White Cell Count 8.24 (2.54)
Creatinine 90 (21)
eGFR 69 (13)
Urea 6.34 (1.77)
NT-proBNP 1,077 (1,004)
hs Troponin T 15 (7)
LVEDV Indexed (trabecular) 71 (12)
LVEF (trabecular) 80 (7)
LV Mass Indexed (trabecular) 105 (32)
RVEDVi 61 (10)
RVEF 67 (9)
MAPSE 13.06 (3.48)
LA Area Indexed 17.4 (4.1)
RA Area Indexed 10.06 (2.42)
Global Native T1 1,032 (39)
Global T2 48.53 (2.12)
ECV 26.94 (3.35)
Global Stress MBF (mL/min/g) 1.73 (0.41)
Global Rest MBF (mL/min/g) 0.86 (0.21)
Global MPR 2.10 (0.64)
Max LVOT gradient (mmHg) 103 (39)
1 Mean (SD); n (%)

Stress

MBF (mL/min/g)
Timepoint Mean SE
Baseline 1.44 0.05
2 months 1.54 0.04
6 months 1.63 0.06

Myocardial Perfusion Reserve

MPR
Timepoint Mean SE
Baseline 1.69 0.05
2 months 1.98 0.05
6 months 2.04 0.06

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#2

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Dose response

Dose (mg) Mean SE
2.5 1.17 0.26
5.0 1.92 0.20
10.0 1.61 0.18