Department of Pediatrics, Graduate school of Medicine, University of Toyama.
Department of Pediatrics, International University of Health and Welfare.
Circ J. 2021 Dec 24;86(1):98-105. doi: 10.1253/circj.CJ-20-1148. Epub 2021 Aug 3.
Left ventricular noncompaction (LVNC) is morphologically characterized by numerous prominent trabeculations and a severely thickened, two-layered myocardium. The fetal onset of LVNC has rarely been described.
We conducted nationwide retrospective surveys on fetal cardiomyopathy (CM) in Japan from 2010 to 2016, from which 38 fetal patients with CM were enrolled, including 16 patients with LVNC. The rate of diagnostic concordance was 56.3% between fetal and postnatal visits in LVNC patients. The increase in the ratio of noncompacted to compacted (N/C) myocardium was time-dependent throughout the fetal period till birth (LV lateral: 1.6±0.1 to 2.8±0.2; LV apex: 2.0±0.1 to 3.2±0.2). Of all fetuses, 16 (42.1%) died or underwent heart transplantation (HT), with 3 intrauterine deaths. Lower fetal cardiovascular profile score (odds ratio, 26.9; P=0.0266) was a risk factor for death or HT. N/C ratio ≥1.6 at the apex at the first visit was a significant predictor of LVNC (odds ratio, 47.8; P=0.0113).
This is the first study to reveal the etiology of fetal CM based on results from a nationwide survey in Japan, highlighting the difficulty of diagnosing LVNC in fetal patients. To better understand and manage fetal CM, novel diagnostic criteria of LVNC in fetus should be established.
左心室心肌致密化不全（LVNC）在形态学上的特征是存在大量突出的小梁和严重增厚的双层心肌。LVNC 的胎儿起病很少被描述。
我们对 2010 年至 2016 年日本的胎儿心肌病（CM）进行了全国范围的回顾性调查，共纳入 38 例 CM 胎儿患者，其中 16 例为 LVNC 患者。LVNC 患者的胎儿期和出生后就诊的诊断一致性率为 56.3%。在整个胎儿期直至出生，非致密心肌与致密心肌（N/C）的比值呈时间依赖性增加（LV 侧壁：1.6±0.1 至 2.8±0.2；LV 心尖：2.0±0.1 至 3.2±0.2）。所有胎儿中，有 16 例（42.1%）死亡或接受心脏移植（HT），其中 3 例宫内死亡。较低的胎儿心血管状况评分（比值比，26.9；P=0.0266）是死亡或 HT 的危险因素。首次就诊时心尖部 N/C 比值≥1.6 是 LVNC 的显著预测因子（比值比，47.8；P=0.0113）。
这是第一项基于日本全国性调查结果揭示胎儿 CM 病因的研究，突出了在胎儿患者中诊断 LVNC 的困难。为了更好地理解和管理胎儿 CM，应建立胎儿 LVNC 的新诊断标准。
