Tunis Med. 2021;99(6):614-622.
Ebstein's disease (ED) is a rare and heterogeneous congenital heart disease affecting the tricuspid valve and the right ventricle. Few studies have analyzed the electrocardiographic features of this disease.
To describe the electrocardiographic features observed in Ebstein's disease.
We conducted a retrospective descriptive study that enrolled 26 patients followed for ED.
The mean age of discovery of the ME was 103.5±99 months [0-31 years]. The diagnosis of ME is most often made between 5 and 10 years. We noted right atrial hypertrophy in 11 patients (42%), right ventricular hypertrophy in half of the patients. Right axial deviation was noted in 11 patients (42%). Eight patients (30%) had wide QRS≥ 120 ms. Seven of these 8 patients (27%) had a fragmented QRS appearance. A right bandle block was noted in 22 patients (84%), it was a complete block in 7 cases (27%). A preexcitation was found in 6 patients (23%). The localization of accessory pathway was right postero-septal in all cases. Rhythmic disorders were noted in 9 patients (34%). It was a junctional tachycardia in 3 patients (11%), atrial flutter in 4 patients (15%) and atrial fibrillation in 2 patients (7%). A second degree atriventricular block was observed in one patient, it was Mobitz I type. Two cases of postoperative rhythm disturbances were recorded: paroxysmal atrial fibrillation and junctional tachycardia related to Wolf Parkinson White (WPW) syndrome.
Surface ECG in the ED is often pathological with prevalence of rhythm disturbances related to WPW syndrome.
Ebstein 畸形(ED)是一种罕见的先天性心脏疾病,影响三尖瓣和右心室。很少有研究分析过这种疾病的心电图特征。
描述 Ebstein 畸形的心电图特征。
我们进行了一项回顾性描述性研究,共纳入 26 例接受 ED 治疗的患者。
平均首次发现 ME 的年龄为 103.5±99 个月[0-31 岁]。ME 的诊断最常发生在 5 至 10 岁之间。我们发现 11 例患者(42%)存在右心房肥大,一半的患者存在右心室肥大。11 例患者(42%)存在右轴偏。8 例患者(30%)存在 QRS 宽度≥120ms。其中 7 例(27%)存在碎裂 QRS 形态。22 例患者(84%)存在右束支阻滞,其中 7 例(27%)为完全阻滞。6 例患者(23%)存在预激。所有患者的旁路定位均在右侧后间隔。9 例患者(34%)存在节律紊乱。3 例患者(11%)为交界性心动过速,4 例患者(15%)为心房扑动,2 例患者(7%)为心房颤动。1 例患者出现二度房室传导阻滞,为 Mobitz I 型。记录到 2 例术后节律紊乱:与 Wolf Parkinson White(WPW)综合征相关的阵发性心房颤动和交界性心动过速。
ED 的体表心电图常存在病理性改变,与 WPW 综合征相关的节律紊乱较为常见。