Why the study?
LVNC is associated with adverse outcomes, but discerning pathologic trabeculations from normal variation is difficult, leaving uncertain whether a diastolic CMR NC:C ratio >2.3 predicts adverse clinical outcomes and imaging findings in children and young adults.
Does a ratio of noncompacted to compacted myocardium >2.3 by CMR predict adverse clinical outcomes and imaging findings in children and young adults with suspected LVNC?
Cohort (n=51)
No
Does a ratio of noncompacted to compacted myocardium >2.3 by CMR predict adverse clinical outcomes and imaging findings in children and young adults with suspected LVNC?
An NC:C ratio >2.3 by CMR alone may be an inadequate marker of pathologic left ventricular trabeculations and does not predict adverse outcomes in children and young adults.
Answers are grounded in this paper and cite their sources.
Questions reliance on NC:C >2.3 by CMR for pediatric LVNC risk stratification; leaves open validation of alternative diagnostic criteria.
Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent trabeculations in the left ventricle and has been associated with adverse outcomes ; yet, there is difficulty in discerning pathologic trabeculations from normal variation. This study sought to test the hypothesis that a ratio of noncompacted to compacted (NC:C) myocardium of >2.3 in diastole by cardiac magnetic resonance imaging (CMR) is associated with adverse clinical outcome and imaging findings in children and young adults referred for CMR to assess for presence of LVNC cardiomyopathy. A retrospective cohort study was performed of patients who were referred for CMR at our center for suspected LVNC between 1/1/11 and 12/31/21. An NC:C ratio was calculated for each CMR, and patients were classified as CMR+ or CMR− using a NC:C cutoff of >2.3. Demographic, electrocardiographic, echocardiographic, CMR, and clinical outcome data were compared. Fifty-one patients were identified, with 30 (59 %) being CMR+. Patients were 12.9 ± 4.7 years old at the time of CMR and followed for a median of 1.7 (IQR 0.1–4.4) years. Baseline characteristics were similar between the CMR+ and CMR− groups. Of the cohort, 1/51 (2 %) who was CMR−, had a stroke, and 5/51 (10 %) experienced significant arrhythmia or ectopy with no significant difference between the groups. There was no mortality, transplant, or mechanical support. LV systolic dysfunction was noted in 8/51 (16 %) of patients by CMR, with no significant difference between the CMR+ and CMR− groups. There was no correlation between the NC:C ratio and left or right ventricular systolic function by CMR. NC:C ratio, as measured by CMR, alone may be an inadequate marker of pathologic left ventricular trabeculations in children and young adults. • Intermediate outcomes good in children referred for evaluation of LV noncompaction • No differences in outcomes or cardiac testing using a NC:C ratio of 2.3 by CMR • Degree of LV trabeculation may be limited in ability to predict adverse outcomes.
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Milligan et al. (2024) conducted a cohort in suspected left ventricular noncompaction cardiomyopathy (LVNC) (n=51). CMR+ (NC:C ratio >2.3) vs. CMR- (NC:C ratio ≤2.3) was evaluated on adverse clinical outcome and imaging findings (stroke, significant arrhythmia, mortality, transplant, mechanical support, LV systolic dysfunction).
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