Abstract 17286: Reconciling Systolic and Diastolic Measures of Left Ventricular Noncompaction Cardiomyopathy in Cardiac Magnetic Resonance

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Richard B Stacey ◽  
Bharathi Upadhya ◽  
Michael M Milks ◽  
Christian Deutsch ◽  
Tiffany Lin ◽  
...  

Background: Previously, it was demonstrated that systolic measures identify higher levels of risk for events related to left ventricular noncompaction cardiomyopathy (LVNC) than diastolic measures. This analysis seeks to improve the ability of the diastolic measures to identify LVNC and predict associated events. Methods: Trabeculation/possible LVNC by cMRI was retrospectively observed among 122 consecutive cases. We assessed the extent of trabeculation, end-systolic noncompacted-to-compacted ratios (ESNCCR), end-diastolic noncompacted-to-compacted ratios (EDNCCR) along with myocardial thickening (MT) and ejection fraction (EF). Deaths, CHF readmissions, ventricular arrhythmias, and embolic events were pooled to increase statistical power and used to identify potential LVNC-associated events. Using logistic regression with an ESNCCR ≥ 2 to identify LVNC as the dependent variable and EDNCCR as the independent variable, analysis were performed with receiver operating curves with and without myocardial thickening as a covariate. Next, using pooled LVNC-associated events as the dependent variable, separate models were performed for ESNCCR ≥ 2 and EDNCCR ≥ 2.3, both adjusting for age, race, gender, body surface area, left ventricular ejection fraction, number of trabeculated segments, and myocardial thickening. Results: The area under the curve (AUC) for EDNCCR to identify potential LVNC was 0.84, but with adjustment for myocardial thickening, the AUC was 0.98, which was significantly increased (p-value = 0.005). Without adjusting for myocardial thickening, ESNCCR ≥ 2 was associated with LVNC-associated events, but EDNCCR ≥ 2.3 was not (p-value: 0.003 vs 0.2, respectively). With adjustment for myocardial thickening, both ESNCCR ≥ 2 and EDNCCR ≥ 2.3 were associated with LVNC-associated events (p-value: 0.03 vs 0.03, respectively). Conclusion: In using the diastolic measure to assess for possible left ventricular noncompaction cardiomyopathy, impaired function of the underlying compacted layer is important in identifying those with a higher risk of subsequent events. Further studies are needed to determine if individuals with increased trabeculations with preserved myocardial thickening are at increased risk of future events.

2021 ◽  
Vol 10 (2) ◽  
Author(s):  
Vaibhav R. Vaidya ◽  
Melissa Lyle ◽  
William R. Miranda ◽  
Medhat Farwati ◽  
Ameesh Isath ◽  
...  

Background The prognosis of left ventricular noncompaction (LVNC) remains elusive despite its recognition as a clinical entity for >30 years. We sought to identify clinical and imaging characteristics and risk factors for mortality in patients with LVNC. Methods and Results 339 adults with LVNC seen between 2000 and 2016 were identified. LVNC was defined as end‐systolic noncompacted to compacted myocardial ratio >2 (Jenni criteria) and end‐diastolic trough of trabeculation‐to‐epicardium (X):peak of trabeculation‐to‐epicardium (Y) ratio <0.5 (Chin criteria) by echocardiography; and end‐diastolic noncompacted:compacted ratio >2.3 (Petersen criteria) by magnetic resonance imaging. Median age was 47.4 years, and 46% of patients were female. Left ventricular ejection fraction <50% was present in 57% of patients and isolated apical noncompaction in 48%. During a median follow‐up of 6.3 years, 59 patients died. On multivariable Cox regression analysis, age (hazard ratio [HR] 1.04; 95% CI, 1.02–1.06), left ventricular ejection fraction <50% (HR, 2.37; 95% CI, 1.17–4.80), and noncompaction extending from the apex to the mid or basal segments (HR, 2.11; 95% CI, 1.21–3.68) were associated with all‐cause mortality. Compared with the expected survival for age‐ and sex‐matched US population, patients with LVNC had reduced overall survival ( P <0.001). However, patients with LVNC with preserved left ventricular ejection fraction and patients with isolated apical noncompaction had similar survival to the general population. Conclusions Overall survival is reduced in patients with LVNC compared with the expected survival of age‐ and sex‐matched US population. However, survival rate in those with preserved left ventricular ejection fraction and isolated apical noncompaction was comparable with that of the general population.


2020 ◽  
Author(s):  
Agnieszka Zienciuk-Krajka ◽  
Ludmiła Daniłowicz-Szymanowicz ◽  
Karolina Dorniak ◽  
Damian Kaufmann ◽  
Grzegorz Raczak

Circulation ◽  
2018 ◽  
Vol 138 (4) ◽  
pp. 367-376 ◽  
Author(s):  
William Y. Shi ◽  
Margarita Moreno-Betancur ◽  
Alan W. Nugent ◽  
Michael Cheung ◽  
Steven Colan ◽  
...  

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