How Common Is LV Noncompaction in Adults?

Study Questions:

What is the true prevalence of noncompaction cardiomyopathy?

Methods:

A meta-analysis was performed using databases dating back to 1946. Original peer-reviewed articles that provided a prevalence estimate or gave the number of affected persons and the cohort size to allow calculation of prevalence of left ventricular noncompaction (LVNC) amongst adults or adolescents aged ≥12 years were included. Ultimately, 59 studies met eligibility criteria for qualitative analysis and 54 met criteria for quantitative analysis.

Results:

The majority of studies, 54%, were conducted in Europe; 36 cohorts in 32 studies used echocardiography (echo) (54%) as the diagnostic modality, while 26 cohorts in 22 studies used cardiac magnetic resonance (CMR) imaging (37%). The overall prevalence of LVNC was 1.28% (95% confidence interval [CI], 0.95-1.64) in the cohorts assessed with echo and 14.79% (95% CI, 8.85-21.85) in the cohorts assessed with CMR. There was a high degree of heterogeneity in prevalence estimates across the cohorts for both modalities, and prevalence varied depending on the cohort assessed. 18.63% of a primigravida pregnant cohort met echo criteria for LVNC and 36% of a noncardiac cohort met CMR criteria. Cohorts of athletes tended to have higher prevalence. There was also substantial variability in prevalence depending on criteria used. The pooled prevalence estimate of LVNC in the echo group was 2.76% when using the Jenni criteria compared to 0.56% when using noncompacted to compacted (NC/C) ratio ≥2. In the CMR group, when using the Petersen criteria, the pooled prevalence was 25.07% compared to 9.01% when using NC/C ratio ≥2.3.

Conclusions:

The true prevalence of LVNC remains unclear. The reported prevalence is higher with the advent of newer imaging modalities, specifically CMR, and varies based on criteria used and cohort studied.

Perspective:

LVNC remains a poorly defined entity whose diagnosis is increasing in prevalence, mostly due to the use of CMR. The clinical significance of these findings warrants further study, as currently, there exists the potential for overdiagnosis and overtreatment.

Clinical Topics: Cardiovascular Care Team, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Sports and Exercise Cardiology, Congenital Heart Disease, CHD and Pediatrics and Imaging, Acute Heart Failure, Echocardiography/Ultrasound, Magnetic Resonance Imaging, Sports and Exercise and Congenital Heart Disease and Pediatric Cardiology, Sports and Exercise and Imaging

Keywords: Adolescent, Athletes, Cardiomyopathies, Diagnostic Imaging, Echocardiography, Heart Failure, Isolated Noncompaction of the Ventricular Myocardium, Magnetic Resonance Imaging, Pregnancy, Prevalence


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