Gan Yi, Luo Li, Tian Jie, Liu Lingjuan, Lu Tiewei
Department of Cardiology; Ministry of Education Key Laboratory of Child Development and Disorders; National Clinical Research Center for Child Health and Disorders (Chongqing); China International Science and Technology Cooperation base of Child development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, People's Republic of China.
Pediatric Department, Maternal and Child Health Hospital of Hubei Province, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, People's Republic of China.
BMC Pediatr. 2020 Sep 9;20(1):430. doi: 10.1186/s12887-020-02312-5.
Ultrasonography is commonly used to diagnose left ventricular noncompaction (LVNC). A ratio of noncompacted to compacted myocardium (NC/C ratio) > >2 is often used to diagnose LVNC. However, a large proportion of patients with noncompact myocardium have NC/C < 2, and the prognosis of these patients have not been studied.
We included children diagnosed with LVNC between 0 and 15 years of age from January 2007 to December 2018. LVNC was diagnosed based on Stöllberger standard when over three trabeculae were found to be associated with the interventricular recesses. A maximal end systolic ratio of noncompacted to compacted layers was NC/C ratio. Outcomes for LVNC subjects with NC/C < 2 and NC/C > 2 were compared using Kaplan-Meier methods.
There were 124 newly diagnosed LVNC cases, classified as isolated (i-LVNC, n = 47) or non-isolated (ni-LVNC, n = 77) LVNC and NC/C > 2 (n = 43) or < 2 (n = 81). The median (interquartile range) follow-up duration was 12 (3-30) months for all patients and 16 (6-36) months for survivors. Sixteen patients with i-LVNC died during follow-up. Patients with i-LVNC and NC/C > 2 had worse survival than those with NC/C < 2 (p = 0.022).
In conclusion, during a 12-month follow-up, patients with i-LVNC with NC/C < 2 had a benign prognosis and better outcomes than those with NC/C > 2, suggesting that the former could have a more active and routine lifestyle.
超声心动图常用于诊断左心室心肌致密化不全（LVNC）。非致密心肌与致密心肌的比例（NC/C 比值）>2 常被用于诊断 LVNC。然而，很大一部分心肌致密化不全患者的 NC/C 比值<2，且这些患者的预后尚未得到研究。
我们纳入了 2007 年 1 月至 2018 年 12 月期间诊断为 LVNC 的 0 至 15 岁儿童。当发现三根以上小梁与室间隐窝相关时，根据施托尔贝格尔标准诊断为 LVNC。非致密层与致密层的最大收缩末期比值即为 NC/C 比值。使用 Kaplan-Meier 方法比较 NC/C<2 和 NC/C>2 的 LVNC 患者的预后。
共有 124 例新诊断的 LVNC 病例，分为孤立性（i-LVNC，n = 47）或非孤立性（ni-LVNC，n = 77）LVNC，以及 NC/C>2（n = 43）或<2（n = 81）。所有患者的中位（四分位间距）随访时间为 12（3 - 30）个月，幸存者为 16（6 - 36）个月。16 例 i-LVNC 患者在随访期间死亡。i-LVNC 且 NC/C>2 的患者生存率低于 NC/C<2 的患者（p = 0.022）。
总之，在 12 个月的随访期间，NC/C<2 的 i-LVNC 患者预后良好，结局优于 NC/C>2 的患者，这表明前者可以有更积极和正常的生活方式。
