(obstructive) hypertrophic cardiomyopathy Thickened heart muscle
Conditions
Interventions
None listed
Sponsors
Cardiothoracale chirurgie
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: HOCM patients undergoing surgical myectomy o HOCM diagnosis according to the 2020 AHA/ACC Guideline for the Diagnosis and Treatment of patients With Hypertrophic Cardiomyopathy (2). Patients with resting or provoked gradients > 50 mm Hg generally considered to be the threshold for surgical myectomy in those patients with drug-refractory symptoms (2). o Patients undergoing surgical myectomy. Heart team (consisting of one cardiologist and one cardiothoracic surgeon) determine if surgical myectomy is required. o Basal and midventricular HCM. o No concomitant surgery. The following procedures are not considered as concomitant surgery: MAZE procedure, Left Atrial Appendage (LAA) resection, device implementation (Pacemaker or ICD). o Patients > 18 years at the moment of the myectomy procedure o Participation in UNRAVEL Biobank. o Willing to comply with the study procedures and written informed consent. Adults with HCM o HCM diagnosis according to the 2020 AHA/ACC Guideline for the Diagnosis and Treatment of patients With Hypertrophic Cardiomyopathy (2). Imaging (2D echocardiography or CMR) showing a maximal end-diastolic wall thickness of > 15 mm basal or midventricular, in the absence of another cause of hypertrophy. Limited hypertrophy (13-14 mm) can be diagnostic when present in family members of a patient with HCM or in conjunction with a positive genetic test. o Patients > 18 years, if possible aged matched HCM control subjects with respect to surgical myectomy patients. o Participation in UNRAVEL Biobank. o Willing to comply with the study procedures and written informed consent. Healthy adult controls o Patients > 18 years. o Willing to comply with the study procedures and written informed consent.
Exclusion criteria
Exclusion criteria: o General contra-indications to CMR. o Aortic valve stenosis grade > 2. o Intrinsic mitral valve (rheumatic, degenerative, infective, and mitral annulus calcification). o Apical HCM. o The coexistence of other forms of congenital heart disease. o Prior cardiac surgery, stroke, percutaneous coronary intervention or previous alcohol septal ablation therapy. o Concomitant surgery in group undergoing surgical myectomy. The following procedures are not considered as concomitant surgery: MAZE procedure, Left Atrial Appendage (LAA) resection, device implementation (Pacemaker or ICD).
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Primary outcome: wall shear stress in HOCM patients (preoperative and at 6 months follow-up), HCM patients and controls. | — |
Secondary
| Measure | Time frame |
|---|---|
| First secondary outcome: Omics in HOCM patients Omics (Tomo-seq and/or nanopore and/or other omics technique) will yield gene expression information for approximately 10.000 genes, for ±200 positions along ±2 cm tissue samples of patients with HCM. The raw output thus is a 10.000 x 200 gene expression table (genes x position) for each sample for each patient. Second secondary outcome: Hemodynamic parameters in HOCM patients, HCM patients and controls. Kinetics (kinetic energy, energy loss, LV myocardial deformation (by myocardial strain analysis)) and blood flow (helicity and vorticity of blood flow will be obtained to characterize LV flow). | — |
Countries
Netherlands
Outcome results
None listed