Clinical Investigation
Congenital Heart Disease
Reduced Left Ventricular Compacta Thickness: A Novel Echocardiographic Criterion for Non-Compaction Cardiomyopathy

https://doi.org/10.1016/j.echo.2012.07.003Get rights and content

Background

Left ventricular noncompaction (LVNC) is characterized by a two-layered myocardium consisting of a noncompacted inner and a compacted outer layer. The ratio of the thicknesses of these two layers is a major diagnostic criterion, which is, however, often difficult to apply in clinical practice.

Methods

Transthoracic echocardiography was performed in 41 patients with LVNC, 41 patients with moderate or severe aortic valve stenosis (AVS), and 41 age-matched normal controls. The maximal systolic thicknesses of “noncompacta” and “compacta” were measured in standard short-axis views at the apical or midventricular level, in the segment with most prominent recesses (in patients with LVNC) or trabeculation (in patients with AVS and controls).

Results

The mean maximal systolic thickness of noncompacta was 1.8 ± 0.4 cm in patients with LVNC compared with 0.2 ± 0.1 cm in controls and 0.6 ± 0.02 cm in patients with AVS (P < .0001). The mean maximal systolic thickness of compacta was lower in patients with LVNC (0.5 ± 0.1 cm) compared to controls (1.2 ± 0.2 cm; P < .0001) and patients with AVS (1.6 ± 0.06 cm; P < .0001). The maximal systolic thickness of compacta was ≤8.1 mm in patients with LVNC compared with >8.1 mm (P < .0001) in controls and >8.1 mm in patients with AVS (P < .0001). The ratio of the maximal systolic thickness of the indexed basal septum to that of the compacta was ≥0.64/m2 in patients with LVNC compared to ≤0.62/m2 in controls and ≤0.96/m2 in patients with AVS.

Conclusions

Maximal systolic compacta thickness <8 mm is specific for LVNC and allows the differentiation of LVNC from normal hearts as well as those with myocardial thickening due to AVS. This observation may be particularly useful as an additional diagnostic criterion for preventing the overdiagnosis of LVNC.

Section snippets

Patients

Forty-one consecutive patients with definite diagnosis of LVNC were evaluated. The diagnosis of LVNC was established by echocardiography with strict adherence to previously published criteria comprising the following four conditions: (1) absence of coexisting cardiac abnormalities; (2) a two-layered myocardial wall with a compacted epicardial layer and a noncompacted endocardial layer, with a maximal end-systolic ratio of noncompacted to compacted layer > 2 in the parasternal short-axis view;

Patient Characteristics

A total of 41 patients (28 [68%] men) with LVNC, 41 age- and sex-matched controls, and 41 patients with hypertrophic myocardium due to moderate or severe AVS mean (left ventricular muscle mass index, 162 ± 7 g/m2) were analyzed. The mean age at presentation was 36 ± 16 years in patients with LVNC and 36 ± 16 years in controls; the mean age in patients with AVS was slightly higher (48 ± 12 years; P < .001 vs patients with LVNC and controls). In patients with LVNC, the most commonly involved

Discussion

Accurate diagnostic criteria for LVNC are important, because its clinical consequences include heart failure due to slowly progressive ventricular dysfunction, ventricular arrhythmias, and systemic embolism.13 However, the diagnosis of LVNC can be challenging despite the existence of echocardiographic criteria, because it is often difficult to apply the latter in clinical routine. The main reasons accounting for this are technical limitations, in particular poor echocardiographic windows and

Conclusions

Left ventricular compacta thickness represents a novel, simple, and robust criterion to diagnose LVNC and should be included in the list of diagnostic criteria for this cardiomyopathy. The criterion indicates that patients with LVNC, in contrast to those with normal or hypertrophic hearts, exhibit a maximal systolic compacta thickness <8 mm in the segment exhibiting the most prominent recesses. Hence, a compacta thickness >8.1 mm indicates either that the patient does not have LVNC or that an

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    The criteria of Jenni et al. [11] are the most widely accepted, but were developed based upon pathological confirmation in only seven patients with LVNC, and the ratio between non-compacted and compacted layers in the most trabeculated area by definition cannot give a global view of the trabecular entity in all of the LV. In another small study, the criterion of maximal systolic compacta thickness of < 8.1 mm was found to be very specific for myocardial thickening in LVNC compared with normal controls or patients with aortic stenosis [27]. Similarly, the criterion of Chin et al. [8] was based upon observations from only eight patients, and is defined as the presence of X/Y ≤ 0.5, where X is the distance from the epicardial surface to the trough of the trabecular recess and Y is the distance from the epicardial surface to the peak of trabeculation, at the LV apex on subxiphoid or apical 4 C views at end-diastole.

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Drs. Gebhard and Stähli contributed equally to this work.

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