Long term clinical outcomes associated with CMR quantified isolated left ventricular non-compaction in adults
Giuseppe Femia1, Danyi Zhu2, Preeti Choudhary3
1Sydney Medical School, Faculty of Medicine and Health, The University of Sydney, Australia.
Insights
Left ventricular non-compaction (LVNC) poses diagnostic challenges. Impaired LV function and late gadolinium enhancement (LGE) predict major adverse cardiovascular events (MACCE) in LVNC patients, not non-compacted mass alone.
Area of Science:
- Cardiology
- Medical Imaging
- Cardiovascular Disease
Background:
- Left ventricular non-compaction (LVNC) is a complex cardiomyopathy with varied diagnostic criteria and no definitive gold standard.
- The clinical significance of non-compacted myocardial mass and its association with adverse outcomes require further elucidation.
Purpose of the Study:
- To evaluate a thresholding technique for quantifying non-compacted myocardial mass in LVNC patients.
- To assess the risk of major adverse cardiovascular and cerebrovascular events (MACCE) in relation to myocardial characteristics.
Main Methods:
- Retrospective analysis of cardiac magnetic resonance (CMR) scans from 98 LVNC patients (Petersen criteria).
- Quantification of noncompacted myocardial mass, LV volumetric parameters, and presence of late gadolinium enhancement (LGE).
- Assessment of association between these parameters and MACCE, excluding patients with known genetic mutations or cardiovascular disease.
Main Results:
- 17.3% of patients had impaired LV function, and 5.1% had LGE.
- Impaired LV function was associated with higher end-systolic non-compacted mass (p < 0.001) and greater non-compacted to total myocardial mass ratio (p = 0.003).
- During 78 months follow-up, MACCE occurred in 11.3% of patients; impaired LV function (HR = 35.6, p < 0.001) and LGE (HR = 16.2, p < 0.001) were significant predictors, while non-compacted mass was not.
Conclusions:
- Non-compacted myocardial mass alone is not an independent predictor of MACCE in LVNC.
- Patients with impaired LV function and/or LGE exhibit a significantly higher risk of MACCE.
- Integrating LV volumetrics and scar assessment (LGE) is crucial for risk stratification in LVNC patients.
Background:
Left ventricular non-compaction (LVNC) is a complex clinical condition with several diagnostic criteria but no diagnostic gold standard. We aimed to evaluate our thresholding technique in a group of patients with LVNC and assess the risk of major adverse cardiovascular and cerebrovascular events (MACCE).
Methods:
We retrospectively analyzed cardiac magnetic resonance (CMR) scans of patients with Petersen criteria LVNC and quantified noncompacted myocardial mass. We assessed the association of noncompacted myocardial mass, CMR derived LV volumetric parameters and late gadolinium enhancement (LGE) to MACCE including cardiac death, cardiac transplantation, sustained ventricular tachycardia/ventricular fibrillation (VT/VF) and ischemic stroke. Patients with known genetic mutations and cardiovascular disease were excluded.
Results:
98 patients with LVNC were included (55 males,56.7%); 17(17.3%) patients had impaired LV function and five (5.1%) had LGE. Patients with impaired LV function had more end-systolic noncompacted mass (61.9 g±22.4 vs. 38.1 g±15.8, p < 0.001) and larger end-systolic noncompacted to total myocardial mass (44%±9 vs. 36%±12, p = 0.003). At 78 months follow-up [interquartile range(IQR) 66-90], MACCE occurred in 11(11.3%) patients; nine(81.8%) had impaired LV function and two(18.2%) had LGE. Impaired LV function and LV LGE were predictors of MACCE (HR = 35.6, 95% CI = 7.65-165.21, p < 0.001 and HR = 16.2, 95% CI = 4.54-57.84, p < 0.001) whereas noncompacted mass were not.
Conclusion:
Noncompacted mass was not an independent predictor of major adverse events but in patients with impaired LV function and/or LV LGE, the risk of MACCE was high. These results highlight the importance of including LV volumetrics and scar in the assessment of patients with LV noncompaction.
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