Uthayakumaran Gajon, Asakai Hiroko, Gnanappa Ganesh
The Heart Centre for Children, The Children's Hospital at Westmead, Corner Hawkesbury Road and Hainsworth St, Westmead, NSW 2145, Australia.
Eur Heart J Case Rep. 2024 Sep 2;8(9):ytae442. doi: 10.1093/ehjcr/ytae442. eCollection 2024 Sep.
Conduction abnormalities are frequently encountered in patients with Ebstein anomaly. The following case describes the safe use of flecainide in an infant with accessory-pathway mediated left ventricular dysfunction in the setting of Ebstein anomaly.
An infant with an antenatal diagnosis of Ebstein anomaly developed progressive left ventricular dilatation and dysfunction over the first 2 months of life. ECG demonstrated persistent Wolff-Parkinson-White pattern with delta-wave polarity suggesting a right-sided septal accessory pathway. In the absence of SVT, accessory-pathway mediated dyssynchrony was suspected as the cause for left ventricular dilatation and dysfunction. He was commenced on flecainide which successfully blocked antegrade conduction via the accessory pathway resulting in a reduction in left ventricular volume and improvement in left ventricular systolic function. He remains asymptomatic at 12 months of age.
There is a known association between Ebstein anomaly and Wolff-Parkinson-White pattern. Right-sided septal accessory pathways can cause cardiomyopathy secondary to dyssynchronous left ventricular contraction. In patients who are unsuitable for accessory pathway ablation, flecainide can be used to block antegrade conduction via the accessory pathway resulting in improved left ventricular function, which was successful on this occasion.
埃布斯坦畸形患者常出现传导异常。以下病例描述了在一名患有埃布斯坦畸形且伴有旁路介导的左心室功能障碍的婴儿中安全使用氟卡尼的情况。
一名产前诊断为埃布斯坦畸形的婴儿在出生后的头2个月出现了进行性左心室扩张和功能障碍。心电图显示持续的预激综合征模式，δ波极性提示右侧间隔旁路。在无室上性心动过速的情况下，怀疑旁路介导的不同步是左心室扩张和功能障碍的原因。他开始使用氟卡尼，该药成功阻断了经旁路的前向传导，导致左心室容积减小，左心室收缩功能改善。他在12个月大时仍无症状。
已知埃布斯坦畸形与预激综合征模式之间存在关联。右侧间隔旁路可导致继发于左心室不同步收缩的心肌病。对于不适合进行旁路消融的患者，氟卡尼可用于阻断经旁路的前向传导，从而改善左心室功能，此次使用取得了成功。
