PDF(Pubmed)
Abstract：
Due to the heterogeneity of anatomic anomalies in Ebstein\'s anomaly (EA), particularly in the subset of patients with atrial septal defect (ASD), hemodynamic changes, which ultimately cause left ventricular (LV) deterioration remain unclear. The current study aimed to investigate the effect of concomitant ASD on LV function using cardiovascular magnetic resonance (CMR) imaging in patients with EA.
This study included 31 EA patients with ASD, 76 EA patients without ASD, 35 patients with simple ASD and 40 healthy controls. Left/right ventricular (RV, the RV was defined as a summation of the functional RV and atrialized RV in EA patients) volumes and functional parameters, LV strain parameters, and echocardiogram indices were compared among the four groups. Associations between variables were evaluated via Spearman or Pearson correlation analyses. The association between risk factors and LV ejection fraction (EF) was determined via multivariate linear regression analysis.
Both EA patients and ASD patients had a higher RV/LV end-diastolic volume (RVEDV/LVEDV) as well as lower LV and RV ejection fractions (LVEF/RVEF) compared to healthy controls (all p < 0.05). Moreover, the EA patients with ASD had a significantly higher RVEDV/LVEDV and a lower LVEF and RVEF than those without ASD (all p < 0.05). Multivariate linear regression analysis revealed that the presence of ASD was independently associated with LVEF (β = - 0.337, p < 0.001). The RVEDV/LVEDV index was associated with LVEF (r = - 0.361, p < 0.001). Furthermore, the LV longitudinal peak diastolic strain rate (PDSR) was lower in EA patients with ASD than those without ASD, patients with simple ASD, and healthy controls (p < 0.05).
Concomitant ASD is an important risk factor of LV dysfunction in patients with EA, and diastolic dysfunction is likely the predominate mechanism related to LV dysfunction.
This study included 31 EA patients with ASD, 76 EA patients without ASD, 35 patients with simple ASD and 40 healthy controls. Left/right ventricular (RV, the RV was defined as a summation of the functional RV and atrialized RV in EA patients) volumes and functional parameters, LV strain parameters, and echocardiogram indices were compared among the four groups. Associations between variables were evaluated via Spearman or Pearson correlation analyses. The association between risk factors and LV ejection fraction (EF) was determined via multivariate linear regression analysis.
Both EA patients and ASD patients had a higher RV/LV end-diastolic volume (RVEDV/LVEDV) as well as lower LV and RV ejection fractions (LVEF/RVEF) compared to healthy controls (all p < 0.05). Moreover, the EA patients with ASD had a significantly higher RVEDV/LVEDV and a lower LVEF and RVEF than those without ASD (all p < 0.05). Multivariate linear regression analysis revealed that the presence of ASD was independently associated with LVEF (β = - 0.337, p < 0.001). The RVEDV/LVEDV index was associated with LVEF (r = - 0.361, p < 0.001). Furthermore, the LV longitudinal peak diastolic strain rate (PDSR) was lower in EA patients with ASD than those without ASD, patients with simple ASD, and healthy controls (p < 0.05).
Concomitant ASD is an important risk factor of LV dysfunction in patients with EA, and diastolic dysfunction is likely the predominate mechanism related to LV dysfunction.
摘要：
背景：由于Ebstein异常（EA）中解剖异常的异质性，特别是在患有房间隔缺损（ASD）的患者中，血液动力学变化,最终导致左心室（LV）恶化的原因尚不清楚。本研究旨在使用心血管磁共振（CMR）成像研究EA患者合并ASD对LV功能的影响。
方法：本研究包括31例ASDEA患者，76例无ASD的EA患者，35例单纯ASD患者和40例健康对照。左/右心室(右心室,RV定义为EA患者的功能性RV和心房化RV的总和）体积和功能参数，低压应变参数,比较四组的超声心动图指标。通过Spearman或Pearson相关分析评估变量之间的关联。通过多元线性回归分析确定危险因素与左心室射血分数（EF）之间的关联。
结果:与健康对照相比,EA患者和ASD患者均具有较高的RV/LV舒张末期容积(RVEDV/LVEDV)以及较低的LV和RV射血分数(LVEF/RVEF)(均p<0.05)。此外，与无ASD的EA患者相比，有ASD的EA患者的RVEDV/LVEDV明显较高，而LVEF和RVEF较低（均p<0.05）。多元线性回归分析显示，ASD的存在与LVEF独立相关（β=-0.337，p<0.001）。RVEDV/LVEDV指数与LVEF相关(r=-0.361,p<0.001)。此外，有ASD的EA患者的左心室纵向峰值舒张应变率（PDSR）低于无ASD的患者，单纯ASD患者，和健康对照组(p<0.05)。
结论：合并ASD是EA患者左心室功能障碍的重要危险因素，舒张功能障碍可能是与LV功能障碍相关的主要机制。
方法：本研究包括31例ASDEA患者，76例无ASD的EA患者，35例单纯ASD患者和40例健康对照。左/右心室(右心室,RV定义为EA患者的功能性RV和心房化RV的总和）体积和功能参数，低压应变参数,比较四组的超声心动图指标。通过Spearman或Pearson相关分析评估变量之间的关联。通过多元线性回归分析确定危险因素与左心室射血分数（EF）之间的关联。
结果:与健康对照相比,EA患者和ASD患者均具有较高的RV/LV舒张末期容积(RVEDV/LVEDV)以及较低的LV和RV射血分数(LVEF/RVEF)(均p<0.05)。此外，与无ASD的EA患者相比，有ASD的EA患者的RVEDV/LVEDV明显较高，而LVEF和RVEF较低（均p<0.05）。多元线性回归分析显示，ASD的存在与LVEF独立相关（β=-0.337，p<0.001）。RVEDV/LVEDV指数与LVEF相关(r=-0.361,p<0.001)。此外，有ASD的EA患者的左心室纵向峰值舒张应变率（PDSR）低于无ASD的患者，单纯ASD患者，和健康对照组(p<0.05)。
结论：合并ASD是EA患者左心室功能障碍的重要危险因素，舒张功能障碍可能是与LV功能障碍相关的主要机制。
