Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom; Heart Department, University Hospital San Giovanni di Dio e Ruggi d'Aragona, Salerno, Italy.
Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom.
Int J Cardiol. 2019 Mar 1;278:76-83. doi: 10.1016/j.ijcard.2018.10.046. Epub 2018 Oct 14.
Accurate risk stratification of patients with Ebstein's anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients.
Fifty consecutive EA patients (1 day-18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ± 41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death). Clinical and instrumental features of EA patients with stable disease were compared with those of EA patients with progressive disease.
Twenty-four (48%) EA patients had progressive disease. A more severe grade of tricuspid valve (TV) displacement [59.7 mm/m (IQR 27.5-83) vs 28.4 mm/m (IQR 17.5-47); p = 0.002], a lower functional right ventricle (RV) fractional area change (FAC) (29.2 ± 7.7% vs 36.7 ± 9.6%; p = 0.004), a higher Celermajer index [0.8 (IQR 0.7-0.98) vs 0.55 (IQR 0.4-0.7); p = 0.000], a lower functional RV-longitudinal strain (-10.2 ± 6.2% vs -16.2 ± 7.3%; p = 0.003) and a lower right atrium peak systolic strain (RA-PALS) (25.2 ± 13.5% vs 36.3 ± 12.5%; p = 0.004) were detected in progressive disease group compared to stable one, respectively. Functional RV-FAC and RA-PALS were independent predictors of progressive disease at multivariate analysis.
Our study demonstrated for the first time the prognostic role of RV-FAC and RA-PALS in a long-term follow-up of EA young patients. A complete echocardiographic evaluation should be regular part in the evaluation and risk-stratification of EA children.
准确的风险分层对埃布斯坦畸形（EA）患者至关重要。本研究旨在评估超声心动图（包括二维斑点追踪（STE）衍生的心肌变形指数）的预后价值，以预测未修复的儿科和年轻成年 EA 患者的结局。
50 例连续的 EA 患者（1 天至 18 岁，52%为男性）接受了超声心动图检查，并进行了平均 60±41 个月的临床随访，以评估临床结局（室性心动过速、心力衰竭、需要手术和/或死亡）。比较稳定疾病和进展性疾病 EA 患者的临床和仪器特征。
24 例（48%）EA 患者有进展性疾病。三尖瓣（TV）位移的严重程度较高[59.7mm/m（IQR 27.5-83）比 28.4mm/m（IQR 17.5-47）；p=0.002]，功能右心室（RV）的分数面积变化（FAC）较低（29.2±7.7%比 36.7±9.6%；p=0.004），Celermajer 指数较高[0.8（IQR 0.7-0.98）比 0.55（IQR 0.4-0.7）；p=0.000]，功能 RV 纵向应变较低（-10.2±6.2%比-16.2±7.3%；p=0.003），右心房峰值收缩应变（RA-PALS）较低（25.2±13.5%比 36.3±12.5%；p=0.004），这些指标在进展性疾病组中较稳定组分别更低。在多变量分析中，功能 RV-FAC 和 RA-PALS 是进展性疾病的独立预测因素。
本研究首次证明了 RV-FAC 和 RA-PALS 在 EA 年轻患者长期随访中的预后作用。全面的超声心动图评估应成为 EA 患儿评估和风险分层的常规部分。
