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Regional and global ventricular systolic function in isolated ventricular non-compaction: pathophysiological insights
Santo Dellegrottaglie1, Patrizia Pedrotti, Alberto Roghi
1Department of Clinical Medicine, Cardiovascular and Immunological Sciences, Federico II University, Naples, Italy. Santo.Dellegrottaglie@mssm.edu
Insights
The extent of myocardial non-compaction predicts left ventricular dysfunction in patients with isolated ventricular non-compaction (IVNC). Disease severity correlates with regional left ventricular dysfunction.
Area of Science:
- Cardiology
- Medical Imaging
- Cardiac MRI
Background:
- Isolated ventricular non-compaction (IVNC) is often linked to left ventricular (LV) systolic dysfunction.
- Factors influencing regional and global LV function in IVNC are not fully understood.
Purpose of the Study:
- To use cardiac MRI to assess how the extent and severity of ventricular non-compaction affect LV systolic function in IVNC patients.
Main Methods:
- MRI was used to evaluate 16 adult IVNC patients based on non-compaction to compacted myocardium ratio (NC/C > 2.3).
- Regional LV function was assessed using wall motion score (WMS) and fractional wall thickening (FWT) in a 16-segment LV model.
Main Results:
- The NC/C ratio significantly correlated with WMS and FWT, indicating disease severity impacts regional function.
- The number of non-compacted segments (NoNC) independently predicted LV ejection fraction and global LV dysfunction.
Conclusions:
- In IVNC patients, disease severity is linked to regional LV dysfunction.
- The extent of myocardial non-compaction is a key independent predictor of global LV dysfunction.
Background:
Isolated ventricular non-compaction (IVNC) is frequently, but not invariably, associated with left ventricular (LV) systolic dysfunction. Factors impacting on regional and global LV function are unknown. The aim of the study was to apply magnetic resonance imaging (MRI) to evaluate the impact of extent and severity of ventricular non-compaction on LV systolic function in patients with IVNC.
Methods:
Sixteen adult patients with IVNC as defined by previously validated MRI criteria [ratio between end-diastolic thickness of non-compacted and compacted myocardium (NC/C ratio)> 2.3 in ≥ 1 LV segment] were enrolled. Short-axis cine images were employed for analysis. Applying a 16-segment LV model, regional systolic performance was assessed qualitatively (wall motion score, WMS; 1 = normal, 2 = mild hypokinesia, 3 = moderate-to-severe hypokinesia, and 4 = a/dyskinesia) as well as quantitatively [fractional wall thickening, FWT (%)=100 × (end-diastolic wall thickness-end-systolic wall thickness)/end-diastolic wall thickness)].
Results:
Mean LV ejection fraction was 43.8 ± 15.4% (range, 17-68%). Regional disease severity, as expressed by the NC/C ratio, revealed a significant correlation with WMS (r=0.26; p=0.018) and FWT (r=-0.30; p=0.006). The total number of non-compacted segments/patient (NoNC) as an index of disease extent was a significant independent correlate of LV ejection fraction by multivariate regression analysis (β=-5.24; p=0.038) and an excellent predictor of global LV dysfunction (ROC analysis, AUC=0.98; p<0.0001).
Conclusions:
In patients with IVNC, disease severity correlates with the degree of LV dysfunction at a regional level. The extent of myocardial non-compaction is an independent predictor of global LV dysfunction.
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