Cardiac Magnetic Resonance Imaging for Evaluation of Non-Compaction Cardiomyopathy in Patients with or without Left Ventricular Systolic Dysfunction

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Abstract

Objective
Non-compaction cardiomyopathy (NCC) is a genetic disorder characterized by the presence of an extensive layer of trabecular myocardium with intertrabecular recesses communicated with the ventricular cavity. The aim of this work is to evaluate the clinical and morphological characteristics of patients with NCC with and without systolic dysfunction assessed by cardiac magnetic resonance imaging (Cardiac MRI).

Material and Methods
Twenty patients with NCC diagnosis were retrospectively included. End- diastolic volume (LVEDV) and end-systolic volume, end-diastolic diameter (LVEDD) and end- systolic diameter, ejection fraction (LVEF), cardiac mass and LV trabeculations were determined. The distribution of NC myocardium was carried out with the model of 17 myocardial segments.

Results
The average thickness of NC myocardium and normal myocardium was 13.1 ± 3.3 mm and 3.6 ± 0.6 mm respectively. LVEDD, LVEDV, global, compacted and trabecular left ventricular mass were significantly increased in patients with ventricular dysfunction. LVEF had a negative linear correlation with the trabecular myocardial mass (MM)/m2 (R = 0.67, p = 0.001), LVEDV/m2 (R = .77, p <0.001) and LVEDD/ m2 (R = 0.7, p <0.001). There was also a negative linear correlation between MM/m2 and LVEDV/m2 (R = 0.76, p <0.001).

Conclusions
There are two ways of presenting the disorder, a subtle or mild with preserved systolic function and another associated to a ventricular dysfunction that behaves as dilated cardiomyopathy.