Masso Anthony H, Uribe Carlo, Willerson James T, Cheong Benjamin Y, Davis Barry R
Department of Cardiology, The University of Texas School of Public Health, Houston, Texas 77030.
Department of Cardiovascular Radiology, Texas Heart Institute, The University of Texas School of Public Health, Houston, Texas 77030.
Tex Heart Inst J. 2020 Jun 1;47(3):183-193. doi: 10.14503/THIJ-19-7157.
In a previous cross-sectional screening study of 5,169 middle and high school students (mean age, 13.1 ± 1.78 yr) in which we estimated the prevalence of high-risk cardiovascular conditions associated with sudden cardiac death, we incidentally detected by cardiac magnetic resonance (CMR) 959 cases (18.6%) of left ventricular noncompaction (LVNC) that met the Petersen diagnostic criterion (noncompaction:compaction ratio >2.3). Short-axis CMR images were available for 511 of these cases (the Short-Axis Study Set). To determine how many of those cases were truly abnormal, we analyzed the short-axis images in terms of LV structural and functional variables and applied 3 published diagnostic criteria besides the Petersen criterion to our findings. The estimated prevalences were 17.5% based on trabeculated LV mass (Jacquier criterion), 7.4% based on trabeculated LV volume (Choi criterion), and 1.3% based on trabeculated LV mass and distribution (Grothoff criterion). Absent longitudinal clinical outcomes data or accepted diagnostic standards, our analysis of the screening data from the Short-Axis Study Set did not definitively differentiate normal from pathologic cases. However, it does suggest that many of the cases might be normal anatomic variants. It also suggests that cases marked by pathologically excessive LV trabeculation, even if asymptomatic, might involve unsustainable physiologic disadvantages that increase the risk of LV dysfunction, pathologic remodeling, arrhythmias, or mural thrombi. These disadvantages may escape detection, particularly in children developing from prepubescence through adolescence. Longitudinal follow-up of suspected LVNC cases to ascertain their natural history and clinical outcome is warranted.
在之前一项针对5169名中学生（平均年龄13.1±1.78岁）的横断面筛查研究中，我们估计了与心源性猝死相关的高危心血管疾病的患病率，通过心脏磁共振成像（CMR）偶然检测出959例（18.6%）符合彼得森诊断标准（非致密化：致密化比率>2.3）的左心室心肌致密化不全（LVNC）病例。其中511例（短轴研究组）有短轴CMR图像。为了确定这些病例中有多少是真正异常的，我们根据左心室结构和功能变量分析了短轴图像，并将除彼得森标准外的3种已发表的诊断标准应用于我们的研究结果。基于小梁化左心室质量（雅克奎尔标准）的估计患病率为17.5%，基于小梁化左心室容积（崔标准）的为7.4%，基于小梁化左心室质量和分布（格罗托夫标准）的为1.3%。由于缺乏纵向临床结局数据或公认的诊断标准，我们对短轴研究组筛查数据的分析未能明确区分正常病例和病理病例。然而，这确实表明许多病例可能是正常的解剖变异。这也表明，即使无症状，以病理性过度的左心室小梁化为特征的病例可能存在不可持续的生理劣势，会增加左心室功能障碍、病理性重塑、心律失常或壁血栓的风险。这些劣势可能难以被发现，尤其是在从青春期前到青春期发育的儿童中。对疑似LVNC病例进行纵向随访以确定其自然病史和临床结局是必要的。
