NCCM Non-compaction cardiomyopathy
Conditions
Interventions
None listed
Sponsors
Erasmus MC, Universitair Medisch Centrum Rotterdam
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: • >=18 years old • Hypertrabeculation of the left ventricle fulfilling the echo-based Jenni criteria of NCCM • Cardiac MRI examination performed or planned for clinical purposes
Exclusion criteria
Exclusion criteria: Exclusion criteria to the study (Aim 1): • Complex congenital disease, neuromuscular disorders or isolated RV non-compaction • Inability to provide informed consent • Contra-indications to MRI Exclusion criteria to the cardiac CT exam (Aim 2): • Age 40 kg/m2 • Pregnancy (or cannot be ruled out) • Known iodine contrast medium allergy • Kidney dysfunction: eGFR
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| We hypothesize integrated analysis of clinical, genetic and imaging variables will improve outcome prediction in patients with NCCM. At 5 centers, 600 patients with suspected NCCM by joint adjudication will be comprehensively phenotyped and followed for up to 3 years. Independent core labs will perform comprehensive structural and functional evaluation of clinical echo and MRI exams. Adverse events are adjudicated by an independent event committee. Machine-learning techniques for complex multi-parameter predictions will be applied to develop predictive models for arrhythmic events, thromboembolic events and composite outcomes. Performance will be compared to currently used risk models and guideline-based criteria for ICD implantation and anticoagulation. | — |
Secondary
| Measure | Time frame |
|---|---|
| We hypothesize that high-resolution cardiac CT can discover structural differences between pathological non-compaction and benign hyper-trabeculation, independent of quantity. From the original cohort (Aim 1), 300 adult patients with suspected NCCM will undergo cardiac CT and advanced computational analytics including hypothesis-based features (thrombogenic cavities), measures of trabecular density and complexity, as well as hypothesis-free image interpretations of the non-compacted myocardium using artificial intelligence (radiomics), as demonstrated in our preliminary studies. Interactions between structure and function will be investigated using regional co-registration of CT and echo/MRI data. We will determine the incremental predictive value of the structural CT features to the models from the primary outcome. | — |
Countries
Netherlands
Outcome results
None listed