Radiology Department, Pontificia Universidad Católica de Chile, Marcoleta 367, Hospital Clinico Universidad Catolica, Santiago 8330024, Santiago Chile.
J Cardiovasc Magn Reson. 2012 Jan 31;14(1):9. doi: 10.1186/1532-429X-14-9.
Isolated left ventricular non-compaction (LVNC) is an uncommon disorder characterized by the presence of increased trabeculations and deep intertrabecular recesses. In adults, it has been found that ejection fraction (EF) decreases significantly as non-compaction severity increases. In children however, there are a few data describing the relation between anatomical characteristics of LVNC and ventricular function. We aimed to find correlations between morphological features and ventricular performance in children and young adolescents with LVNC using cardiovascular magnetic resonance (CMR).
15 children with LVNC (10 males, mean age 9.7 y.o., range 0.6-17 y.o.), underwent a CMR scan. Different morphological measures such as the compacted myocardial mass (CMM), non-compaction (NC) to the compaction (C) distance ratio, compacted myocardial area (CMA) and non-compacted myocardial area (NCMA), distribution of NC, and the assessment of ventricular wall motion abnormalities were performed to investigate correlations with ventricular performance. EF was considered normal over 53%.
The distribution of non-compaction in children was similar to published adult data with a predilection for apical, mid-inferior and mid-lateral segments. Five patients had systolic dysfunction with decreased EF. The number of affected segments was the strongest predictor of systolic dysfunction, all five patients had greater than 9 affected segments. Basal segments were less commonly affected but they were affected only in these five severe cases.
The segmental pattern of involvement of non-compaction in children is similar to that seen in adults. Systolic dysfunction in children is closely related to the number of affected segments.
孤立性左心室致密化不全（LVNC）是一种罕见的疾病，其特征是存在增多的小梁和深小梁间隐窝。在成年人中，已发现随着非致密化程度的增加，射血分数（EF）显著降低。然而，在儿童中，只有少量数据描述 LVNC 的解剖特征与心室功能之间的关系。我们旨在使用心血管磁共振（CMR）在患有 LVNC 的儿童和青少年中寻找 LVNC 的形态特征与心室功能之间的相关性。
15 名 LVNC 患儿（男 10 名，平均年龄 9.7 岁，范围 0.6-17 岁）接受了 CMR 扫描。进行了不同的形态学测量，如致密心肌质量（CMM）、非致密（NC）与致密（C）距离比、致密心肌面积（CMA）和非致密心肌面积（NCMA）、NC 的分布以及心室壁运动异常的评估，以研究与心室功能的相关性。EF 被认为超过 53%是正常的。
儿童的非致密化分布与已发表的成人数据相似，优先累及心尖、中下部和中侧段。5 名患者存在收缩功能障碍，EF 降低。受影响节段的数量是收缩功能障碍的最强预测因子，所有 5 名患者的受影响节段均大于 9 个。基底段较少受累，但仅在这 5 例严重病例中受累。
儿童非致密化受累的节段模式与成人相似。儿童的收缩功能障碍与受影响节段的数量密切相关。
