HCMR - Novel Predictors of Outcome in Hypertrophic Cardiomyopathy.

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 140

Inclusion Criteria

• Male or Female, aged 18-65
• Established diagnosis of HCM defined as unexplained LVH defined as any segment >= 15mm thick
• Signed informed consent
• Able (in the investigator's opinion) and willing to comply with all study requirements

Exclusion Criteria

• Uncontrolled hypertension as judged by the investigator
• Uncontrolled atrial fibrillation at time of enrollment
• Angiographically documented >50% coronary stenosis
• Prior septal myectomy or alcohol septal ablation
• Prior myocardial infarction
• Incessant ventricular arrhythmias
• Diabetes with end organ damage
• Stage IV/V chronic kidney disease (eGFR <30ml/min)
• Inability to tolerate MRI scanning (severe claustrophobia, inability to lie flat)
• Contraindications to CMR imaging (implantable devices or other metal implants, cranial aneurysm clips, metallic ocular foreign bodies, hypersensitivity to gadolinium)
• Female participant who is pregnant or lactating
• Malignancy or other serious medical condition expected to limit lifespan <5 years
• Any other significant disease or disorder which, in the opinion of the investigator, might influence the participant*s ability to participate in the study.
• Involvement in other studies thought to compromise resulting study data or the health of the participant
• Prior inclusion in the study of 5 members of the same immediate family.
• Inability to give informed consent

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Primary outcomes<br /><br>Using exploratory data mining methods to identify demographic, clinical, and<br /><br>novel cardiac magnetic resonance imaging, genetic and biomarker variables<br /><br>associated with the outcomes.<br /><br><br /><br>Risk Markers<br /><br>1. CMR to measure cardiac volumes, mass, function and fibrosis<br /><br>2. Genotyping<br /><br>3. Serum biomarkers of fibrosis<br /><br>4. Clinical risk factors<br /><br><br /><br>Clinical outcome<br /><br>Primary<br /><br>1. The composite of cardiac death due to sudden cardiac death (SCD) and<br /><br>congestive heart failure (CHF)<br /><br>2. Aborted SCD including appropriate intracardiac defibrillator (ICD) firing<br /><br>3. Need for heart transplantation<br /><br>Secondary<br /><br>1. All-cause mortality<br /><br>2. Ventricular tachyarrhythmias<br /><br>3. Hospitalisation for heart failure<br /><br>4. Atrial fibrillation<br /><br>5. Stroke</p><br>

Secondary Outcome Measures

Name	Time	Method
<p>Secondary outcomes<br /><br>Using the demographic, clinical, imaging, biomarker and genetic measures, plus<br /><br>any interactions, identified in the tree analysis, Cox proportional hazards<br /><br>regression will be used to develop a predictive model.</p><br>