Paszkowska Agata, Sarnecki Jędrzej, Mirecka-Rola Alicja, Kowalczyk-Domagała Monika, Mazurkiewicz Łukasz, Ziółkowska Lidia
Department of Cardiology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw, Poland.
Department of Diagnostic Imaging, The Children's Memorial Health Institute, 04-730 Warsaw, Poland.
J Cardiovasc Dev Dis. 2022 Mar 5;9(3):77. doi: 10.3390/jcdd9030077.
Left ventricular noncompaction (LVNC) is a distinct cardiomyopathy characterized by the presence of a two-layer myocardium with prominent trabeculation and deep intertrabecular recesses. The diagnosis of LVNC can be challenging because the diagnostic criteria are not uniform. The aim of our study was to evaluate echocardiographic and CMR findings in a group of children with isolated LVNC. From February 2008 to July 2021, pediatric patients under 18 years of age at the time of diagnosis with echocardiographic evidence of isolated LVNC were prospectively enrolled. The patients underwent echocardiography and contrast-enhanced cardiovascular magnetic resonance (CMR) with late gadolinium enhancement to assess myocardial noncompaction, ventricular size, and function. A total of 34 patients, with a median age of 11.9 years, were recruited. The patients were followed prospectively for a median of 5.1 years. Of the 31 patients who met Jenni's criteria in echocardiography, CMR was performed on 27 (79%). Further comprehensive analysis was performed in the group of 25 patients who met the echocardiographic and CMR criteria for LVNC. In echocardiography, the median NC/C ratio in systole was 2.60 and in diastole 3.40. In 25 out of 27 children (93%), LVNC was confirmed by CMR, according to Petersen's criteria, with a median NC/C ratio of 3.27. (1) Echocardiography precisely identifies patients with LVNC. (2) Echocardiography is a good method for monitoring LV systolic function, but CMR is indicated for the precise assessment of LV remodeling and RV size and function, as well as for the detection of myocardial fibrosis.
左心室心肌致密化不全(LVNC)是一种独特的心肌病,其特征是存在两层心肌,伴有明显的肌小梁和深陷的小梁间隐窝。LVNC的诊断可能具有挑战性,因为诊断标准并不统一。我们研究的目的是评估一组孤立性LVNC患儿的超声心动图和心脏磁共振成像(CMR)结果。从2008年2月至2021年7月,前瞻性纳入了诊断时年龄小于18岁、超声心动图有孤立性LVNC证据的儿科患者。患者接受了超声心动图和对比增强心血管磁共振成像(CMR)检查,并进行延迟钆增强以评估心肌致密化不全、心室大小和功能。共招募了34例患者,中位年龄为11.9岁。对患者进行了中位时间为5.1年的前瞻性随访。在31例超声心动图符合詹尼标准的患者中,27例(79%)进行了CMR检查。对25例超声心动图和CMR均符合LVNC标准的患者进行了进一步的综合分析。在超声心动图检查中,收缩期NC/C比值的中位数为2.60,舒张期为3.40。根据彼得森标准,27例儿童中有25例(93%)通过CMR确诊为LVNC,NC/C比值的中位数为3.27。(1)超声心动图能准确识别LVNC患者。(2)超声心动图是监测左心室收缩功能的良好方法,但CMR适用于精确评估左心室重构、右心室大小和功能,以及检测心肌纤维化。