Clinical Investigation
Left Ventricular Non-Compaction
Reproducibility of Echocardiographic Diagnosis of Left Ventricular Noncompaction

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Background

Left ventricular noncompaction (LVNC) cardiomyopathy is variably defined by numerous trabeculations, deep intertrabecular recesses, and noncompacted-to-compacted (NC/C) ratio >2. Limited studies exist on the reproducibility of diagnosing LVNC.

Methods

Clinical records of patients diagnosed with LVNC by echocardiography were reviewed. Blinded review of the index echocardiogram for all patients and a 1:1 match without LVNC was performed independently by two observers, measuring the number of trabeculations and the NC/C ratio.

Results

A total of 104 patients with LVNC were included in the study, 52 with no congenital heart disease (NCongHD) and 52 with congenital heart disease (CongHD). The duration of follow-up was 7.2 years (range, 0.5–23.1 years) for NCongHD and 8.2 years (range, 0–33.3 years) for CongHD. Agreement between observers in determining zero to three versus more than three trabeculations was 59% (NCongHD) and 73% (CongHD). Agreement in measuring an NC/C ratio ≤ 2 versus > 2 was 79% (NCongHD) and 74% (CongHD). Agreement with the original reader in diagnosing LVNC was 67%. There was no association between the number of trabeculations or the NC/C ratio and the likelihood of a major event. Patients with moderate or severe left ventricular dysfunction at the time of diagnosis were more likely to undergo cardiac transplantation or die compared with those with normal or mild dysfunction (NCongHD, 22% vs 0%, P = .01; CongHD, 39% vs 3%, P = .001).

Conclusions

The reproducibility of making measurements to diagnose LVNC by accepted criteria is poor. Heart transplantation and death are associated with significant ventricular dysfunction and not with increased trabeculations or NC/C ratios.

Section snippets

Identifying Cases

The records of all patients who received coded diagnoses of LVNC or excessive trabeculations by echocardiography from 1989 to 2006 at the Children’s Hospital of Boston were reviewed. Patients were included in the study if the index echocardiogram (on which the diagnosis of noncompaction was based) and at least one other echocardiogram were available for review. Patients with congenital heart disease (CongHD) and those with no congenital heart disease (NCongHD) were included in the study. The

Results

A total of 104 cases with LVNC were included in the study, 52 NCongHD and 52 CongHD. The median age of diagnosis of LVNC was 11.0 years (range, 0.0–19.1 years) and 1.8 years (range, 0.0–30.4 years) in the NCongHD and CongHD groups, respectively. The duration of follow-up was 7.2 years (range, 0.5–23.1 years) in the NCongHD group and 8.2 years (range, 0–33.3 years) in the CongHD group. The most frequent reasons for initial referral in the NCongHD group included arrhythmia, congestive heart

Discussion

LVNC was previously considered a rare cardiomyopathy, though over the past 5 years, there has been a surge in reporting of cases. Variable diagnostic criteria and individualized modifications by investigators allow the inclusion of a different population in each series, making comparison of data particularly difficult.2, 3, 5, 6, 7, 9, 10, 11, 12, 13, 14, 15, 16, 19, 20, 22 In our study, we considered a large cohort of patients initially assigned diagnostic codes for LVNC by an echocardiography

Conclusions

LVNC is considered a distinct, primary cardiomyopathy despite variable diagnostic criteria and limited validation of such criteria. Our study demonstrates that the qualitative diagnosis and quantitative measurements to fill diagnostic criteria are poorly reproducible between observers. Severe outcomes in our cohort of patients were associated with poor LV function and not morphologic findings. We call into question whether LV hypertrabeculation represents a primary cardiomyopathy or a secondary

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