Myocardial Energetic Restoration in the Treatment of Obstructive Hypertrophic Cardiomyopathy

NCT07332767 | Not Yet Recruiting

• 1 other identifier: 367806
• Study Type: observational
• Target: 20
• Locations: 0 countries
• Sites: N/A

Brief Summary

Hypertrophic Cardiomyopathy (HCM) is the most common inherited heart condition, where the heart muscles can thicken to the point of obstructing blood flow out of the heart. This condition is associated with a chronic state of energy loss in the heart muscle. Till more recently, a new class of medication (cardiac myosin inhibitors) have been introduced to directly target the heart muscle proteins (sarcomeres) to reduce the strength of contraction and relieve obstruction of blood flow out of the heart. While clinical trials have shown this class of medication significantly improves physical capacity and patient symptoms, it is still unclear, based on small scale published studies, where this improvement is achieved by restoring the fundamental energy balance within the heart. Our research study aims to answer this question and prove mechanistic insights of the use of this class of medication in the HCM population with blood flow obstruction (otherwise known as obstructive HCM) by using a specialised non-invasive MRI technique which accurately measures the heart energy score (specifically known as the PCr/ATP ratio) in each participant. Our objective is to determine how a patient with obstructive HCM have their energy scores affected, and improve over time with this medication therapy. If positive, this finding could establish the use of PCr/ATP ratio as a crucial, objective biomarker for monitoring therapeutic response and informing personalised dosing strategies for patient in the future.

Conditions

Hypertrophic Cardiomyopathy (HCM)

Keywords

hypertrophic cardiomyopathy

Outcome Measures

Primary Outcomes (1)

• Mean change in PCr/ATP ratio

  The main goal is to determine if mavacamten therapy (cardiac myosin inhibitor) significantly improves the heart-muscle energy levels of patients with obstructive hypertrophic cardiomyopathy, as measured by the "energy score" (PCr/ATP) on a specialised MRI scan.

  24 weeks

Eligibility Criteria

• Age: 18 Years - 99 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

The cohort consists of adults diagnosed with symptomatic obstructive hypertrophic cardiomyopathy (oHCM) who are clinically eligible for and planning to initiate treatment with mavacamten.

You may qualify if:

• at least 18 years of age and
• Have a confirmed diagnosis of oHCM that is not solely explained by abnormal loading conditions such as significant hypertension or valvular disease
• Qualify for mavacamten therapy by exhibiting a peak Left Ventricular Outflow Tract (LVOT) gradient of ≥ 50mmHg at rest or with provocation, New York Heart Association (NYHA) functional class II or III symptoms, and a baseline Left Ventricular Ejection Fraction (LVEF) of ≥ 55%

You may not qualify if:

• HCM phenocopies such as cardiac amyloidosis or Fabry's disease
• Undergone a septal reduction therapy (myectomy or ablation) within the preceding 6 months
• Any contraindications to mavacamten (e.g., baseline LVEF \< 55%, pregnancy/breastfeeding)
• Inability to safely undergo a cardiac MRI, such as having non-compatible metal implants or severe claustrophobia

Sponsors & Collaborators

• University of Manchester: lead

MeSH Terms

Conditions

Cardiomyopathy, Hypertrophic

Condition Hierarchy (Ancestors)

Cardiomyopathies; Heart Diseases; Cardiovascular Diseases; Aortic Stenosis, Subvalvular; Aortic Valve Stenosis; Aortic Valve Disease; Heart Valve Diseases

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Cardiologist

Study Record Dates

• First Submitted: December 29, 2025
• First Posted: January 12, 2026
• Study Start: April 30, 2026
• Primary Completion (Estimated): April 1, 2028
• Study Completion (Estimated): April 1, 2028
• Last Updated: January 12, 2026

Record last verified: 2025-12