Value of Cardiac Magnetic Resonance (CMR) Derived Parameters for Diagnosing Left Ventricular Non-compaction Cardiomyopathy

Value of Different Myocardial Parameters to Differentiate Left Ventricular Noncompaction Cardiomyopathy From Other Cardiomyopathies and Healthy Controls by Cardiac Magnetic Resonance

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.

Study Overview

• Status: Completed
• Conditions: Left Ventricular Hypertrophy; Left Ventricular Failure; Left Ventricular Non-compaction Cardiomyopathy

• Study Type: Observational
• Enrollment (Actual): 57

Contacts and Locations

Study Locations

• Germany
  • Leipzig, Germany, 04289
    • University of Leipzig - Heart Center

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 14 years to 64 years (ADULT, CHILD)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• 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.

Description

Inclusion Criteria:

• left ventricular non-compaction cardiomyopathy
• dilatative cardiomyopathy
• hypertrophic cardiomyopathy or healty controls

Exclusion Criteria:

• contraindications for magnetic resonance imaging like pacemakers or other metallic implants

Study Plan

How is the study designed?

Number of groups / cohorts

4

Cohorts and Interventions

Group / Cohort
LVNC; 12 patients with left ventricular non-compaction cardiomyopathy
HCM; 10 patients with hypertrophic cardiomyopathy
DCM; 11 patients with dilatative cardiomyopathy
controls; 24 healthy controls

Collaborators and Investigators

• Sponsor: University of Leipzig

Study record dates

Study Major Dates

• Study Start: December 1, 2004
• Primary Completion (ACTUAL): July 1, 2008
• Study Completion (ACTUAL): October 1, 2008

Study Registration Dates

• First Submitted: November 25, 2011
• First Submitted That Met QC Criteria: November 28, 2011
• First Posted (ESTIMATE): November 29, 2011

Study Record Updates

• Last Update Posted (ESTIMATE): November 29, 2011
• Last Update Submitted That Met QC Criteria: November 28, 2011
• Last Verified: November 1, 2011

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Heart Diseases; Cardiovascular Diseases; Pathological Conditions, Anatomical; Cardiomegaly; Hypertrophy; Cardiomyopathies; Hypertrophy, Left Ventricular
• Other Study ID Numbers: LVNC 2011