Pediatric Cardiology Unit, Mansoura University Children's Hospital, Faculty of Medicine, Mansoura University, Mansoura, Egypt.
Pediatric Nephrology Unit, Mansoura University Children's Hospital, Faculty of Medicine, Mansoura University, Mansoura, Egypt.
Eur J Pediatr. 2023 Jun;182(6):2807-2819. doi: 10.1007/s00431-023-04936-y. Epub 2023 Apr 11.
Studies on the right ventricular dysfunction (RV) in systemic lupus erythematosus (SLE) patients are limited, particularly in the pediatric age group. The study aimed to identify subclinical RV alterations in childhood-onset SLE (c-SLE) using conventional and three-dimensional echocardiography (3DE). Forty SLE pediatric patients and 40 healthy controls were included. Disease activity and chronicity were evaluated by SLE disease activity index (SLEDAI) score and SLE damage index (SDI). Participants underwent detailed RV echocardiographic examination with conventional and 3DE assessment using 3D auto RV software. Patients included 35/40 (87.5%) females with mean age of 15.6 ± 1.7 years. Using conventional pulmonary artery systolic pressure echocardiography-derived measurement, none of the c-SLE patients had pulmonary hypertension. By 3DE, RV end-systolic and end-diastolic volumes (p = < 0.001, 0.02, respectively) were greater, whereas 3D-derived RV ejection fraction (p < 0.001), septal, and lateral longitudinal strain (both p < 0.001) were lower in SLE. SDI displayed a significant correlation with 3D auto RV ejection fraction (EF), tricuspid annular plane systolic excursion (TAPSE), fractional area change, and RV longitudinal strain (RVLS)-free wall (p = 0.01, 0.003, 0.007, and < 0.001, respectively). Cumulative SLEDAI score also showed a significant correlation with RV EF, TAPSE, FAC, and RVLS-free wall (p = 0.03, 0.007, 0.002, and < 0.001, respectively). By multivariate regression analysis, SDI remained an independent predictor of RVLS-free wall (ß coefficient - 0.4, p = 0.03) and TAPSE (ß - 0.5, p = 0.02). Conclusion: Subtle right ventricular myocardial dysfunction could be detected in childhood-onset SLE patients, especially via 3D-derived auto RV echocardiographic parameters, despite the absence of evident pulmonary hypertension. These parameters correlate with the SLE disease activity and chronicity scores. What is Known: •Diseases of the cardiovascular system are one of the most common causes of morbidity and mortality in SLE patients. •RV labeled the forgotten ventricle in many diseases, was also forgotten in SLE patients and has been rarely addressed in adults, with scarce research in pediatrics. What is New: •Right ventricular functions are affected in children with SLE in comparison to healthy controls, especially three-dimensional echocardiography-derived parameters, which is an aspect that has not been investigated in previous research in the pediatric age group. •Some of the detected myocardial dysfunctions of the right ventricle correlated with SLE disease activity and chronicity-related scores.
关于系统性红斑狼疮 (SLE) 患者右心室功能障碍 (RV) 的研究有限，尤其是在儿科年龄组。本研究旨在使用常规和三维超声心动图 (3DE) 来识别儿童发病的 SLE (c-SLE) 中的亚临床 RV 改变。纳入了 40 名 SLE 儿科患者和 40 名健康对照者。使用 SLE 疾病活动指数 (SLEDAI) 评分和 SLE 损伤指数 (SDI) 评估疾病活动度和慢性度。参与者接受了详细的 RV 超声心动图检查，使用 3D 自动 RV 软件进行常规和 3DE 评估。患者中 35/40 (87.5%) 为女性，平均年龄为 15.6±1.7 岁。使用常规肺动脉收缩压超声心动图测量，无 c-SLE 患者有肺动脉高压。通过 3DE，RV 收缩末期和舒张末期容积 (p<0.001，0.02) 增加，而 3D 衍生的 RV 射血分数 (p<0.001)、间隔和侧壁纵向应变 (均 p<0.001) 降低。SDI 与 3D 自动 RV 射血分数 (EF)、三尖瓣环平面收缩期位移 (TAPSE)、节段面积变化和 RV 纵向应变 (RVLS)-游离壁 (均 p=0.01、0.003、0.007 和 <0.001) 显著相关。累积 SLEDAI 评分也与 RV EF、TAPSE、FAC 和 RVLS-游离壁 (均 p=0.03、0.007、0.002 和 <0.001) 显著相关。多元回归分析显示，SDI 仍然是 RVLS-游离壁 (β系数-0.4，p=0.03) 和 TAPSE (β-0.5，p=0.02) 的独立预测因子。结论：尽管无明显肺动脉高压，但仍可在儿童发病的 SLE 患者中检测到微妙的右心室心肌功能障碍，特别是通过 3D 衍生的自动 RV 超声心动图参数。这些参数与 SLE 疾病活动度和慢性度评分相关。已知：·心血管系统疾病是 SLE 患者发病率和死亡率的最常见原因之一。·RV 在许多疾病中被称为被遗忘的心室，在 SLE 患者中也被遗忘，在成人中很少被提及，在儿科中研究甚少。新发现：·与健康对照组相比，SLE 患儿的右心室功能受到影响，尤其是三维超声心动图衍生参数，这是在儿科年龄组之前的研究中未被研究的方面。·一些检测到的右心室心肌功能障碍与 SLE 疾病活动度和与慢性度相关的评分相关。
