Department of Pediatric Cardiology and General Pediatrics, Doctoral School, Medical University of Warsaw, 02-091, Warsaw, Poland.
Department of Pediatric Cardiology and General Pediatrics, Jozef Polikarp Brudzinski Public Pediatric Hospital, 02-091, Warsaw, Poland.
Sci Rep. 2024 Sep 10;14(1):21059. doi: 10.1038/s41598-024-72175-8.
Conventional diastolic dysfunction parameters seem to be imperfect when applied to the pediatric cardiomyopathy population. The aim of this pilot study was to search for novel echocardiographic parameters associated with adverse outcomes in children with the most common cardiomyopathies. Fifty-six patients with pediatric cardiomyopathies (28 with dilated, 21 with hypertrophic, 7 with left ventricular non-compaction cardiomyopathy) and 28 healthy subjects were included in the study. Left atrial reservoir (LASr), conduit (LAScd) and contraction (LASct) strain, left atrial stiffness index (LASI), as well as conventional diastolic dysfunction parameters were measured using echocardiography. Adverse outcomes were defined as heart failure (including heart transplant) and arrhythmic endpoints. Patients with adverse outcomes presented with significantly lower LASr (16.68% ± 8.64% vs. 33.97% ± 9.99%, p-value < 0.001), lower LAScd (- 10.37% ± 5.83% vs. - 25.50% ± 9.24%, p-value < 0.001) and higher values of LASI (0.69 [IQR 0.34; 1.11] vs. 0.21 [IQR 0.16; 0.31], p-value < 0.001). LASr < 20%, LAScd ≥ - 12%, and LASI ≥ 0.26 were all associated with reduced survival. LASr, LAScd and LASI seem to be promising parameters in predicting adverse outcomes in the most common pediatric cardiomyopathies. Left atrial strain parameters and LASI are helpful in differentiating healthy control subjects from children with hypertrophic and dilated cardiomyopathies.
当应用于儿科心肌病患者时，传统的舒张功能障碍参数似乎并不完善。本研究旨在寻找与常见儿科心肌病患者不良预后相关的新型超声心动图参数。
该研究纳入了 56 例儿科心肌病患者（扩张型心肌病 28 例，肥厚型心肌病 21 例，左室心肌致密化不全 7 例）和 28 例健康对照者。使用超声心动图测量左心房储备（LASr）、传导（LAScd）和收缩（LASct）应变、左心房僵硬度指数（LASI）以及传统舒张功能障碍参数。不良结局定义为心力衰竭（包括心脏移植）和心律失常终点。
与无不良结局患者相比，有不良结局患者的 LASr 明显更低（16.68%±8.64% vs. 33.97%±9.99%，p 值<0.001），LAScd 更低（-10.37%±5.83% vs. -25.50%±9.24%，p 值<0.001），LASI 更高（0.69[IQR 0.34; 1.11] vs. 0.21[IQR 0.16; 0.31]，p 值<0.001）。LASr<20%、LAScd≥-12%和 LASI≥0.26 均与生存率降低相关。
LASr、LAScd 和 LASI 似乎是预测常见儿科心肌病不良预后的有前途的参数。左心房应变参数和 LASI 有助于将健康对照者与肥厚型和扩张型心肌病患儿区分开来。
