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孤立性左心室致密化不全患者致密层变薄和收缩功能障碍:一项心脏磁共振研究。

Thinning of compact layer and systolic dysfunction in isolated left ventricular non-compaction: A cardiac magnetic resonance study.

机构信息

Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padua, Italy.

Department of Medicine, University of Padua, Italy.

出版信息

Int J Cardiol. 2024 Feb 15;397:131614. doi: 10.1016/j.ijcard.2023.131614. Epub 2023 Nov 26.

DOI:10.1016/j.ijcard.2023.131614
PMID:38016624
Abstract

BACKGROUND

The Petersen' index reflects an excess of myocardial trabeculation which is not a specific morpho-functional feature of left ventricular non-compaction (LVNC) cardiomyopathy, but a "phenotypic trait" even observed in association with other myocardial diseases and over-loading conditions. The present study was designed to evaluate the relation between a critical thinning of compact layer and the development of systolic dysfunction and LVNC cardiomyopathy.

METHODS

We compared CMR morpho-functional features and measurements of LV wall thickness using a 17 segment model of a cohort of patients fulfilling the Petersen criterion for LVNC with LV systolic dysfunction versus those of a cohort of age- and sex-matched controls with LVNC and preserved LV systolic function. All the study patients had an "isolated" LVNC defined as positive Petersen criterion in the absence of other diseases such as hypertrophic and dilated cardiomyopathy, valvular heart disease, or congenital heart disease and over-loading conditions.

RESULTS

he study population included 33 patients with "isolated" LVNC: 11 consecutive index patients with a reduced LV ejection fraction (LVNCrEF) and 22 controls with a preserved LVEF (LVNCpEF). The compact myocardial layer was thinner in patients with LVNCrEF than in those with LVNCpEF patients, both in mid-ventricular and apical LV segments. On linear regression analysis, there was a linear correlation between median thickness of mid-ventricular free wall segments and left ventricular ejection fraction (r = 0.51, p = 0.005). On the ROC curves analysis, ≥2 segments with a compact myocardial layer <5 mm in the free wall mid-ventricular segments showed the best accuracy for reduced LVEF (100% sensitivity and 60% specificity; AUC 0.81, p < 0.01). The negative predictive value for LV systolic dysfunction of <2 free wall mid ventricular segments <5 mm was 100%. On quantitative analysis, the mass of papillary muscles was lower in patients with LVNCrEF [1.2 (0.8-1.4) versus 1.6 (1.1-1.8) g/mq; p = 0.08].

CONCLUSIONS

A thinned compact layer of mid-ventricular segments of the LV free wall was associated with a reduced systolic function and "isolated" LVNC cardiomyopathy.

摘要

背景

彭氏指数反映了心肌小梁的过度增生,这不是左心室致密化不全(LVNC)心肌病的特定形态-功能特征,而是一种“表型特征”,甚至与其他心肌疾病和负荷过重情况有关。本研究旨在评估致密层临界变薄与收缩功能障碍和 LVNC 心肌病发展之间的关系。

方法

我们比较了满足 LVNC 彭氏标准的 LV 收缩功能障碍患者和年龄、性别匹配的 LVNC 且 LV 收缩功能正常患者的 CMR 形态-功能特征和 LV 壁厚度的 17 节段模型测量值。所有研究患者均存在“孤立性”LVNC,定义为在不存在肥厚型和扩张型心肌病、瓣膜性心脏病或先天性心脏病和负荷过重等其他疾病的情况下,彭氏标准为阳性。

结果

研究人群包括 33 名“孤立性”LVNC 患者:11 名连续指数患者的 LV 射血分数降低(LVNCrEF)和 22 名对照患者的 LV 射血分数保留(LVNCpEF)。LVNCrEF 患者的 LVNC 比 LVNCpEF 患者的 LVNC 患者的 LVNC 患者的中层更薄,无论是在中层心室还是心尖 LV 节段。在线性回归分析中,中层心室游离壁节段的中位数厚度与左心室射血分数呈线性相关(r=0.51,p=0.005)。在 ROC 曲线分析中,≥2 个中层心室游离壁节段的致密心肌层<5mm 对降低的 LVEF 具有最佳的准确性(100%敏感性和 60%特异性;AUC 0.81,p<0.01)。<2 个 LV 中层心室节段<5mm 的 LV 收缩功能障碍的阴性预测值为 100%。在定量分析中,LVNCrEF 患者的乳头肌质量较低[1.2(0.8-1.4)比 1.6(1.1-1.8)g/mq;p=0.08]。

结论

LV 游离壁中层节段的致密层变薄与收缩功能障碍和“孤立性”LVNC 心肌病有关。

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