Value of Cardiac Magnetic Resonance (CMR) Derived Parameters for Diagnosing Left Ventricular Non-compaction Cardiomyopathy
Condition: Left Ventricular Non-compaction Cardiomyopathy; Left Ventricular Failure; Left Ventricular Hypertrophy
MeSH terms: Cardiomyopathies; Hypertrophy, Left Ventricular; Hypertrophy
Study type: Observational

Summary: 
      Left ventricular non-compaction (LVNC) is a rare cardiomyopathy characterized by numerous
      excessively prominent left ventricular (LV) trabeculation and deep intertrabecular recesses
      communicating with the ventricular cavity and severely altering myocardial structure.
      Although most authors assume a developmental arrest in embryogenesis as the underlying
      pathology, the mechanisms of LVNC are not fully understood yet. Several gene mutations have
      been identified to be linked with LVNC and an autosomal dominant inheritance pattern is
      frequent To date the most commonly used imaging tool for diagnosing LVNC is echocardiography
      applying the criteria established by Jenni and coauthors However, qualitative parameters to
      differentiate normal compaction of the myocardium in healthy subjects from LVNC or from other
      cardiomyopathies like dilative cardiomyopathy (DCM) or hypertrophic cardiomyopathy (HCM) may
      fail due to highly variable LV trabeculation. Therefore, absolute quantification should be
      performed. Cardiac magnetic resonance (CMR) has been reported as a promising imaging modality
      to characterize patients with LVNC as it provides both a high spatial resolution and a good
      contrast between trabeculation and blood pool Jacquier et al. recently described a value of
      trabeculated LV myocardial mass above 20% of the global mass of the LV to be highly sensitive
      and specific for LVNC However, in their approach, a substantial degree of the LV cavity was
      included into calculated trabecular LV mass and led to systemic overestimation of the latter.
      Furthermore, the role and prognostic value of myocardial scarring as assessed by delayed
      enhancement (DE) CMR was not evaluated.

      The aim of the retrospective study was to establish revised and extended CMR criteria to
      distinguish LVNC from DCM, HCM and a group of healthy controls and to improve the assessment
      of trabeculated mass by excluding intertrabecular blood pool.
    


Observational model: Cohort
Time perspective: Retrospective
Sampling method: Probability Sample
Study population: 
        Twelve patients (3 male, 27%) with proven LVNC, two with familial LVNC, were included into
        the study. The patients initially presented with symptoms of heart failure and were
        referred to the department of cardiology for further evaluation. All patients underwent
        echocardiography performed by experienced specialists and fulfilled the LVNC criteria of
        Jenni. CMR imaging was performed within 3 days after echocardiography. Exclusion criteria
        were co-existing cardiac anomalies and usual CMR contraindications such as implanted
        defibrillators/pacemakers. The investigators furthermore included 10 consecutive patients
        (4 male, 36%) with HCM and 11 consecutive patients (3 male, 27%) with DCM. The diagnosis of
        HCM and DCM was established according to current guidelines Patient parameters were
        compared to a control group of 25 healthy age matched volunteers (12 male, 48%) without
        history of cardiovascular disease and without clinical symptoms.