Sarnecki Jędrzej, Paszkowska Agata, Petryka-Mazurkiewicz Joanna, Kubik Agata, Feber Janusz, Jurkiewicz Elżbieta, Ziółkowska Lidia
Department of Diagnostic Imaging, The Children's Memorial Health Institute, 04-730 Warsaw, Poland.
Department of Cardiology, The Children's Memorial Health Institute, 04-730 Warsaw, Poland.
J Clin Med. 2022 Feb 19;11(4):1104. doi: 10.3390/jcm11041104.
: Left ventricular non-compaction (LVNC) is a rare cardiomyopathy typically involving the left ventricle (LV); however, the right ventricle (RV) can also be affected. This case-control study aimed to assess the morphology and function of LV and RV in children with LVNC. : Sixteen children (13 ± 3 years, six girls) with LVNC were compared with 16 sex- and age-matched controls. LV and RV morphology and function were evaluated in cardiovascular magnetic resonance (CMR) studies. Additionally, LV and RV global radial (GRS), circumferential (GCS), and longitudinal strain (GLS) were assessed using tissue-tracking analysis. : Patients with LVNC did not differ from the healthy controls in terms of age, height, weight, and body surface area (BSA). In total, 4/16 subjects with LVNC had mid-wall late gadolinium enhancement (LGE). Compared to the control group, patients with LVNC had higher end-diastolic volume (EDV) indexed for body surface area (BSA), lower ejection fraction (EF), and lower LV strain parameters (all < 0.05). Children with LVNC also presented with thicker RV apical trabeculation, whereas there were no differences in RV EF and EDV/BSA between the groups. Nevertheless, children with LVNC had impaired RV GRS and GCS (both < 0.05). : LVNC in pediatric patients is associated with LV enlargement and impaired LV systolic function. Additionally, children with LVNC have increased RV trabeculations and subclinical impairment of RV myocardial deformation.
左心室心肌致密化不全（LVNC）是一种罕见的心肌病，通常累及左心室（LV）；然而，右心室（RV）也可能受到影响。本病例对照研究旨在评估LVNC患儿左、右心室的形态和功能。：将16例LVNC患儿（13±3岁，6名女孩）与16名性别和年龄匹配的对照组进行比较。在心血管磁共振（CMR）研究中评估左、右心室的形态和功能。此外，使用组织追踪分析评估左、右心室的整体径向应变（GRS）、圆周应变（GCS）和纵向应变（GLS）。：LVNC患者在年龄、身高、体重和体表面积（BSA）方面与健康对照组无差异。总共，16例LVNC患者中有4例出现中层心肌延迟钆增强（LGE）。与对照组相比，LVNC患者的体表面积（BSA）指数化舒张末期容积（EDV）更高，射血分数（EF）更低，左心室应变参数更低（均<0.05）。LVNC患儿还表现为右心室心尖小梁增厚，而两组之间右心室EF和EDV/BSA无差异。然而，LVNC患儿的右心室GRS和GCS受损（均<0.05）。：小儿患者的LVNC与左心室扩大和左心室收缩功能受损有关。此外，LVNC患儿的右心室小梁增加，右心室心肌变形存在亚临床损害。
