a thickened heart muscle based on genetic predisposition Hypertrophic cardiomyopathy
Conditions
Interventions
None listed
Sponsors
Universitair Medisch Centrum Sint Radboud
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: •Patients with an echocardiographically proven hypertrophic cardiomyopathy according to the ESC guidelines; •Individuals with a HCM associated mutation without the clinical characteristics of hypertrophic cardiomyopathy (pre-clinical HCM patients); •Age >= 18 years; •Able to comply with the protocol; •Written informed consent.
Exclusion criteria
Exclusion criteria: •Known significant epicardial coronary artery disease; •Patients with LVH in the clinical setting of other disorders that explain the myocardial hypertrophy (amyloidosis, MELAS, Anderson-Fabry, WPW etc.); •Heart failure NYHA class III-IV; •Patients with known hemodynamic instability or syncope during exercise due to left ventricular outflow gradient or occurrence of ventricular arrhythmia; •History of PTSMA (percutaneous transluminal septal myocardial ablation) or Morrow myectomy; •Patients not able to complete a bicycle test; •Any contraindication to MR imaging (MR imaging is not obligatory for assessment of the primary objective, therefore relative exclusion criterion); •Recent (within 30 days) admittance to the hospital for any cardiac reason (myocardial infarction, heart failure, cardiac arrhythmia, etc.); •Severe renal insufficiency (eGFR
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Proportion of patients (pre-clinical HCM mutation carriers and clinical HCM patients) with 1) a baseline troponin concentration above the upper reference limit (the 99th percentile) of the high-sensitivity troponin assay that will be used, and 2) a troponin rise after exercise testing (more than or equal to 20% of the baseline concentration) using the same high sensitivity-troponin assay. | — |
Secondary
| Measure | Time frame |
|---|---|
| Pre-clinical HCM-group (genotype positive, no hypertrophy) -The correlation between troponin (baseline concentration, rise after exercise) and MRI parameters (i.e. myocardial mass and function, number of segments with and volume (in mL) of LGE, presence of edema); -The correlation between troponin ( baseline, rise after exercise) and clinical follow-up (development of the hypertrophic phenotype, septum wall thickness >13mm). Clinical HCM group (genotype positive or negative, with hypertrophy) -The correlation between troponin ( baseline concentration, rise after exercise) and MRI parameters (i.e. myocardial mass and function, number of segments with and volume (in mL) of LGE, presence of edema); -The correlation between troponin (baseline concentration, rise after exercise) and: a) death due to all causes, cardiovascular death, and sudden cardiac death at two and 5 year follow-up; b) admission to the hospital for cardiac reasons (i.e. new episode of heart failure, arrhythmia, syncope) during two and five year follow-up; c) event free survival at two and 5 year, defined as alive at the time of telephone call and not admitted for any cardiac adverse event during follow-up. | — |
Countries
Netherlands
Outcome results
None listed